Pemfigus vulgaris
Charakterystyka, pielęgnacja i opieka
Pemfigus vulgaris to autoimmunologiczna choroba pęcherzowa, charakteryzująca się obecnością pęcherzy i nadżerek na skórze oraz błonach śluzowych, najczęściej w jamie ustnej, z możliwością zajęcia nosa, gardła, oczu i narządów płciowych. W patogenezie dochodzi do produkcji przeciwciał przeciwko białkom desmosomalnym, co prowadzi do akantolizy i powstawania pęcherzy śródnaskórkowych. Nieleczona choroba cechuje się wysoką śmiertelnością (dawniej do 99%), jednak dzięki wczesnej diagnostyce i terapii immunosupresyjnej, w tym stosowaniu kortykosteroidów systemowych w dawkach 1-2 mg/kg/dobę prednizonu lub pulsacyjnej terapii metyloprednizolonem, śmiertelność spadła do 5-15%. Leczenie obejmuje fazy kontroli, konsolidacji i podtrzymania, z zastosowaniem leków oszczędzających sterydy, takich jak azatiopryna, mykofenolan mofetylu czy rytuksymab. W ciężkich przypadkach rozważa się dożylne immunoglobuliny (IVIg) oraz plazmaferezę. Kluczowa jest także specjalistyczna pielęgnacja ran, zapobieganie infekcjom oraz wielodyscyplinarne podejście terapeutyczne.
- Definicja i charakterystyka pemfigus vulgaris
- Cele leczenia i opieki w pemfigus vulgaris
- Farmakoterapia w pemfigus vulgaris
- Pielęgnacja skóry i nadżerek w pemfigus vulgaris
- Pielęgnacja jamy ustnej w pemfigus vulgaris
- Opieka pielęgnacyjna nad pacjentem z pemfigus vulgaris
- Zalecenia dla pacjenta z pemfigus vulgaris
- Monitorowanie i kontrola leczenia
- Współpraca interdyscyplinarna w opiece nad pacjentem
- Praktyczne zalecenia dla personelu medycznego
Definicja i charakterystyka pemfigus vulgaris
Pemfigus vulgaris to rzadka, autoimmunologiczna choroba, która powoduje powstawanie pęcherzy na skórze i błonach śluzowych. W tej chorobie przeciwciała atakują białka skórne, co prowadzi do tworzenia się pęcherzy i nadżerek, które wymagają specjalistycznej opieki pielęgniarskiej.1 Najczęściej zmiany pojawiają się w jamie ustnej, a następnie mogą rozprzestrzeniać się na skórę i inne błony śluzowe. Choroba może dotyczyć jamy ustnej, nosa, gardła, oczu i narządów płciowych.2 Pemfigus vulgaris jest najczęstszym typem pemfigusu, dotyka osoby w każdym wieku, choć rzadko dzieci, a szczyt zachorowań przypada między 50. a 60. rokiem życia.3
Nieleczony pemfigus vulgaris może być stanem zagrażającym życiu, ze śmiertelnością sięgającą dawniej nawet 99%. Dzięki nowoczesnym metodom leczenia wskaźnik ten spadł do około 5-15%.4 Wczesne rozpoznanie i rozpoczęcie leczenia są kluczowe dla pomyślnego rokowania, a wielodyscyplinarne podejście jest zalecane w celu optymalizacji opieki nad pacjentem.5
Cele leczenia i opieki w pemfigus vulgaris
Główne cele terapeutyczne w leczeniu pemfigus vulgaris obejmują:67
- Kontrolę choroby i zmniejszenie tworzenia się nowych pęcherzy
- Wspieranie gojenia istniejących pęcherzy i nadżerek
- Zapobieganie powikłaniom, szczególnie infekcjom
- Minimalizację dawek leków potrzebnych do kontroli choroby
- Poprawę jakości życia pacjenta
- Zapobieganie nawrotom choroby
Leczenie pemfigus vulgaris przebiega zazwyczaj w trzech etapach:89
- Kontrola: wysokie dawki leków kontrolują rozprzestrzenianie się pęcherzy i rozpoczynają gojenie istniejących
- Konsolidacja: po osiągnięciu kontroli choroby następuje stopniowe zmniejszanie dawek leków
- Podtrzymanie: minimalne dawki leków zapobiegają nawrotom
Farmakoterapia w pemfigus vulgaris
Leczenie farmakologiczne jest podstawą terapii pemfigus vulgaris i powinno być rozpoczęte jak najwcześniej.10 Główne grupy leków stosowane w terapii to:
Kortykosteroidy
Kortykosteroidy systemowe są podstawą leczenia pemfigus vulgaris. Zazwyczaj stosuje się wysokie dawki prednizonu lub prednisolonu doustnie (1-2 mg/kg/dobę) lub jako pulsacyjną terapię dożylną metyloprednizolonem.1112 Celem jest szybkie uzyskanie kontroli nad chorobą, a następnie stopniowe zmniejszanie dawki o około 25% po osiągnięciu remisji i wygojeniu większości zmian.13
Długotrwałe stosowanie kortykosteroidów może powodować istotne działania niepożądane, takie jak cukrzyca, nadciśnienie, osteoporoza, choroba wrzodowa, zaćma czy jałowa martwica kości.1415 Pacjenci otrzymujący długotrwałą terapię kortykosteroidami powinni być oceniani przez reumatologa w ciągu pierwszych 30 dni leczenia w celu oceny ryzyka osteoporozy i rozważenia profilaktycznego stosowania bisfosfonianów.16
Leki immunosupresyjne
Leki immunosupresyjne są często przepisywane razem z kortykosteroidami jako leki oszczędzające steroidy. Do najczęściej stosowanych należą:1718
- Azatiopryna
- Mykofenolan mofetylu
- Cyklofosfamid
- Rytuksymab (zatwierdzony przez FDA)
- Dapson (lek przeciwzapalny)
- Metotreksat
- Cyklosporyna
Immunosupresanty powinny być rozważone wcześnie w przebiegu choroby jako środki oszczędzające sterydy.19 Azatiopryna i mykofenolan mofetylu są uznawane za pierwszorzędowe adiuwantowe terapie immunosupresyjne w pemfigusie, uważane za bezpieczne i skuteczne.20
Inne metody terapeutyczne
W przypadkach ciężkich lub opornych na standardowe leczenie można zastosować:2122
- Dożylne immunoglobuliny (IVIg)
- Plazmaferezę (usuwanie przeciwciał z krwi pacjenta)
- Leki zawierające złoto
W przypadku ciężkiego przebiegu choroby pacjenci mogą wymagać hospitalizacji, podobnie jak przy ciężkich oparzeniach, z koniecznością podawania płynów i elektrolitów dożylnie, szczególnie gdy owrzodzenia w jamie ustnej utrudniają przyjmowanie pokarmów i płynów.2324
Pielęgnacja skóry i nadżerek w pemfigus vulgaris
Prawidłowa pielęgnacja skóry i ran jest kluczowa w leczeniu pemfigus vulgaris. Cztery główne cele pielęgnacji ran to:25
- Zapobieganie infekcjom
- Utrzymanie wilgotnego środowiska
- Ochrona rany
- Minimalizacja tworzenia się blizn
Postępowanie z pęcherzami
Małe pęcherze powinny pozostać nienaruszone, aby zapobiec wtórnym infekcjom. Duże pęcherze należy nakłuć i odessać sterylną igłą (najlepiej 18G), zachowując dach pęcherza jako barierę ochronną.26
Ostrożne oczyszczanie jest priorytetem i można je wykonać za pomocą soli fizjologicznej lub antybakteryjnego mydła/antyseptyku dwa razy dziennie. Po dokładnym osuszeniu rany należy nałożyć obojętną maść nawilżającą, na przykład mieszaninę 50% białej miękkiej parafiny i 50% parafiny ciekłej, bezpośrednio na łożysko rany lub na opatrunek.2728
Wybór odpowiedniego opatrunku
Przy wyborze opatrunku należy uwzględnić zarówno charakterystykę rany, jak i specyficzne cechy opatrunku, takie jak zdolność absorpcyjna, jakość nawilżająca, właściwości przylepne i komfort.29 Wszystkie produkty opatrunkowe stosowane u pacjentów z pemfigusem muszą być nieprzylepne.
W zależności od stanu rany można stosować:3031
- Nieprzylepną gazę
- Przezroczystą folię
- Opatrunki hydrokoloidowe
- Opatrunki hydrożelowe
- Opatrunki z siatką silikonową
- Opatrunki zawierające antyseptyk
Jeśli rana jest mokra, opatrunek powinien absorbować nadmiar wysięku. Jeśli rana jest sucha, opatrunek powinien dostarczać wilgoci do łożyska rany.32 W przypadku ran sączących się, na opatrunek pierwotny można nałożyć opatrunek absorbujący.33
Zapobieganie infekcjom
Infekcja jest główną przyczyną zgonów w pemfigus vulgaris, dlatego należy zachować czujność w identyfikowaniu i leczeniu wszelkich infekcji.3435 Zalecenia obejmują:3637
- Stosowanie antybiotyków i leków przeciwgrzybiczych do kontrolowania lub zapobiegania infekcjom
- Kąpiele w roztworze nadmanganianu potasu (1:10000) lub kąpiele wybielaczowe
- Częste zmienianie i pranie ręczników, prześcieradeł i bielizny osobistej w gorącej wodzie
- Stosowanie talku, aby zapobiec przywieraniu skóry do pościeli
Pielęgnacja jamy ustnej w pemfigus vulgaris
Owrzodzenia jamy ustnej są często pierwszym objawem pemfigus vulgaris i mogą utrzymywać się przez tygodnie lub miesiące.38 Dobra higiena jamy ustnej jest kluczowa dla pozytywnych wyników leczenia.39
Zalecenia dotyczące higieny jamy ustnej
Pacjentom z pemfigus vulgaris zaleca się:404142
- Używanie bardzo miękkiej szczoteczki do zębów
- Stosowanie pasty do zębów bez dodatku mięty
- Delikatne i dokładne szczotkowanie zębów dwa razy dziennie
- Płukanie jamy ustnej płynem antyseptycznym, takim jak glukonian chlorheksydyny 0,2% lub roztwór nadtlenku wodoru 1:4
- Unikanie płynów do płukania ust zawierających alkohol
- Regularne wizyty u dentysty
Instrukcje dotyczące higieny jamy ustnej do stosowania w domu mogą wymagać dostosowania w zależności od stopnia zajęcia błony śluzowej. Gdy obecna jest znaczna choroba jamy ustnej, łagodna pielęgnacja domowa może być wszystkim, co pacjent może tolerować. Niektórzy pacjenci mogą nie być w stanie używać nici dentystycznej z powodu krwawienia i bólu. W tym czasie ważniejsze stają się częste czyszczenia dentystyczne.43
Leczenie owrzodzeń jamy ustnej
W przypadku owrzodzeń w jamie ustnej można złagodzić ból poprzez:444546
- Spożywanie łagodnych, miękkich pokarmów
- Stosowanie miejscowych środków znieczulających (pastylki do ssania)
- Przyjmowanie leków przeciwbólowych, szczególnie przed jedzeniem lub szczotkowaniem zębów
- Stosowanie płynów do płukania ust z działaniem przeciwzapalnym
- Leczenie kandydozy jamy ustnej, jeśli występuje
Pacjenci powinni unikać pokarmów, które mogą podrażniać lub uszkadzać wnętrze jamy ustnej (pikantne, kwaśne, twarde i chrupiące pokarmy).4748 W przypadku ciężkich owrzodzeń jamy ustnej, które uniemożliwiają jedzenie, pacjenci mogą wymagać karmienia dożylnego lub suplementów odżywczych, aby uniknąć niedożywienia.4950
Opieka pielęgnacyjna nad pacjentem z pemfigus vulgaris
Opieka pielęgniarska nad pacjentem z pemfigus vulgaris obejmuje szereg działań mających na celu poprawę wyników klinicznych i jakości życia pacjenta.51
Ocena pielęgniacyjna
Ocena pielęgniarska zmian w pemfigus vulgaris jest kluczowa dla wczesnego leczenia i monitorowania postępu choroby. Pielęgniarki muszą rozpoznawać zmiany skórne związane z pemfigus vulgaris i właściwie je dokumentować.52
W celu standaryzacji rejestrowania owrzodzeń w pemfigus vulgaris zaleca się używanie terminów opisujących zmiany podstawowe: pęcherzyki, pęcherze, nadżerki, owrzodzenia i uogólnione strupy.53 Standaryzacja nomenklatury używanej do opisywania zmian skórnych prowadzi do bardziej jednolitej komunikacji między specjalistami, unikając nieporozumień i skutkując bezpieczniejszą opieką nad pacjentem.54
Interwencje pielęgnacyjne
Interwencje pielęgniarskie obejmują:5556
- Staranną higienę jamy ustnej, aby utrzymać błonę śluzową jamy ustnej w czystości i umożliwić regenerację nabłonka
- Częste płukanie jamy ustnej w celu usunięcia resztek i złagodzenia owrzodzonych obszarów
- Premedykację lekami przeciwbólowymi przed rozpoczęciem pielęgnacji skóry u pacjentów z bolesnymi i rozległymi zmianami
- Oczyszczanie skóry w celu usunięcia resztek i martwej skóry oraz zapobiegania infekcjom
- Podawanie leków przeciwdrobnoustrojowych zgodnie z zaleceniami i ocenę odpowiedzi na leczenie
- Zachęcanie pacjenta do utrzymania odpowiedniego doustnego przyjmowania płynów
W przypadku ciężkiego przebiegu choroby z rozległym zajęciem skóry, pacjenci są zazwyczaj przyjmowani na oddział oparzeń. Dlatego opieka wspomagająca przypomina tę, którą wykonuje się przy ciężkich oparzeniach termicznych. Ma na celu zminimalizowanie potencjalnych powikłań, które mogą ostatecznie doprowadzić do śmierci pacjenta, takich jak hipowolemii, zaburzeń elektrolitowych, niewydolności nerek i sepsy.57
Zalecenia dla pacjenta z pemfigus vulgaris
Pacjenci z pemfigus vulgaris mogą podjąć kroki w celu zarządzania swoimi objawami i poprawy jakości życia.58
Ogólne zalecenia
- Delikatne traktowanie skóry, aby uniknąć tworzenia nowych pęcherzy i nadżerek
- Unikanie aktywności, które mogą traumatyzować skórę i błony śluzowe podczas aktywnych faz choroby (np. sporty kontaktowe)
- Dbanie o pęcherze i rany zgodnie z zaleceniami lekarza
- Przestrzeganie zaleceń dotyczących higieny jamy ustnej
- Zgłaszanie lekarzowi problemów z jedzeniem i piciem
- Unikanie słońca i wysokiej temperatury w przypadku pemfigus foliaceus lub pemfigusu wywołanego lekami
Istotne jest również, aby pacjenci byli poinformowani o swoich lekach, dawkach, działaniach niepożądanych i objawach toksyczności, co pozwoli im lepiej zgłaszać wszelkie działania niepożądane lekarzowi.62
Zalecenia dietetyczne
Odżywianie jest kluczowym aspektem opieki ze względu na utratę białka i innych niezbędnych składników w surowiczo-krwistym drenażu.63 Zalecenia dietetyczne obejmują:6465
- Spożywanie łagodnych, miękkich pokarmów w przypadku owrzodzeń jamy ustnej
- Unikanie pikantnych, kwaśnych lub cytrusowych pokarmów, które mogą nasilać objawy pemfigus vulgaris
- Zwracanie uwagi na to, co pomaga poczuć się lepiej i co pogarsza objawy
- Rozważenie suplementów odżywczych w przypadku trudności z jedzeniem, aby uniknąć niedożywienia
Wsparcie psychologiczne
Pemfigus vulgaris może być poważną chorobą, która zmienia życie. Pacjenci powinni być zachęcani do swobodnego wyrażania niepokojów, dyskomfortu i uczuć beznadziejności.66 Pomocne mogą być techniki relaksacyjne i ćwiczenia jako sposoby na poprawę mechanizmów radzenia sobie z chorobą.67
Monitorowanie i kontrola leczenia
Ciągłe monitorowanie aktywności choroby i działań niepożądanych leczenia jest kluczowe w zarządzaniu pemfigus vulgaris. Regularne wizyty kontrolne są konieczne do dostosowywania dawek leków i zapewnienia optymalnej opieki.68
Lekarz prowadzący będzie regularnie monitorował stan pacjenta i zlecał badania krwi i/lub moczu, aby sprawdzić, czy leczenie działa. Testy sprawdzają również negatywne reakcje na leki stosowane w leczeniu pemfigusu.69
Długoterminowe zarządzanie koncentruje się na utrzymaniu remisji. Po opanowaniu choroby zaleca się stopniowe zmniejszanie dawek kortykosteroidów i terapii immunosupresyjnej. W przypadku nawrotu należy zintensyfikować leczenie, aby odzyskać kontrolę nad chorobą.70
Pemfigus vulgaris jest chorobą przewlekłą i może wymagać leczenia przez miesiące lub lata, a nawet całe życie. Większość osób z pemfigus vulgaris ma jednak normalną długość życia przy odpowiednim leczeniu.71 Leczenie pomaga zarządzać objawami, ale może być kontynuowane przez całe życie.72
Współpraca interdyscyplinarna w opiece nad pacjentem
Zarządzanie pacjentami z pemfigus vulgaris wymaga koordynacji opieki między dermatologiem a lekarzem podstawowej opieki zdrowotnej.73 W zależności od nasilenia choroby i obecności powikłań, pacjent może wymagać konsultacji różnych specjalistów:
- Dermatolog – do diagnostyki i leczenia choroby skóry74
- Stomatolog – dla regularnych kontroli stomatologicznych i opieki nad jamą ustną75
- Reumatolog – do oceny ryzyka osteoporozy u pacjentów otrzymujących długotrwałą terapię kortykosteroidami76
- Dietetyk – w przypadku problemów z odżywianiem77
- Pielęgniarka – do specjalistycznej opieki nad ranami i prowadzenia edukacji pacjenta78
Zarówno zespół medyczny, jak i stomatologiczny są potrzebni do leczenia pacjenta z pemfigus vulgaris jamy ustnej.79 Wczesne rozpoznanie i szybkie rozpoczęcie leczenia są niezbędne dla obiecującego rokowania, a podejście wielodyscyplinarne jest zalecane w celu optymalizacji opieki nad pacjentem.80
W przypadku ciężkiego przebiegu choroby pacjenci mogą wymagać hospitalizacji na oddziale oparzeń lub oddziale intensywnej terapii, gdzie otrzymają specjalistyczną opiekę, w tym leczenie ran, nawodnienie dożylne i monitorowanie pod kątem infekcji.8182
Praktyczne zalecenia dla personelu medycznego
Dla personelu medycznego opiekującego się pacjentami z pemfigus vulgaris, kluczowe jest:
- Wczesne rozpoznanie i rozpoczęcie leczenia, co jest niezbędne dla pomyślnego rokowania83
- Indywidualizacja terapii w zależności od nasilenia choroby i współistniejących schorzeń84
- Odpowiednia pielęgnacja ran z delikatnym oczyszczaniem, stosowaniem odpowiednich opatrunków i zapobieganiem infekcjom85
- Edukacja pacjenta na temat choroby, leków i pielęgnacji skóry oraz jamy ustnej86
- Regularne monitorowanie aktywności choroby i działań niepożądanych leczenia87
- Współpraca z innymi specjalistami w celu zapewnienia kompleksowej opieki88
Lekarze pierwszego kontaktu, z odpowiednim przeszkoleniem w zakresie dermatologii ogólnej, mogą odegrać kluczową rolę we wczesnym rozpoznawaniu, diagnostyce i szybkim leczeniu pemfigusu, poprawiając tym samym jakość życia swoich pacjentów.89
Szczególną uwagę należy zwrócić na pacjentów z ciemną karnacją skóry, u których pemfigus vulgaris może manifestować się inaczej, z większą skłonnością do pozapalnej hiperpigmentacji, co zmienia klasyczny wygląd choroby. Brak obrazów i dowodów w literaturze dotyczącej pemfigus vulgaris u pacjentów z ciemnym odcieniem skóry prowadzi do opóźnienia diagnozy, co ma negatywny wpływ na zarządzanie pacjentem i ogólne rokowanie.9091
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Materiały źródłowe
- #1 Effect of Implementing a Protocol of Nursing Care on Pemphigus Vulgaris Patients’ Clinical Health Outcomes.https://ejhc.journals.ekb.eg/article_380830.html
Pemphigus Vulgaris is a skin disease recognized for its characteristic autoantibody production, which assail skin proteins, leading to the formation of blisters and erosions that require special nursing care to maximize patients health outcomes. […] Nursings care protocol was effective in improving patients clinical health outcomes. […] Creation of an educational unit for Nursing Care on Pemphigus of Vulgaris Patients in the dermatology hospital departments. Conducting similar studies on a larger probability sample is recommended to achieve generalization of the findings.
- #2 Pemphigus Vulgarishttps://healthlibrary.wjmc.org/Library/DiseasesConditions/Adult/Spine/134,110
Pemphigus vulgaris is a rare group of autoimmune diseases. It causes blisters on the skin and mucous membranes in the body. It can affect the mouth, nose, throat, eyes, and genitals. Pemphigus vulgaris is the most common type of pemphigus. It isn’t contagious. […] Good oral healthcare. Blistering may affect the health of your mouth. So working with your dentist to take the best care of your teeth and gums is important. […] Pain management. Pain medicine or other pain management strategies may be used to help with painful blisters. […] You may be advised to use baths and special wound dressings to help sores heal. If you get an infection, your healthcare provider may prescribe antibiotic, antifungal, or antiviral medicines. People with severe cases of pemphigus vulgaris may need to be hospitalized to get wound care and intravenous (IV) fluids or electrolytes if mouth sores make it difficult to eat and drink.
- #3 Pemphigus vulgarishttps://www.pcds.org.uk/clinical-guidance/pemphigus-vulgaris
Pemphigus vulgaris is a rare immunobullous condition that is characterised by blisters and erosions on the skin and mucous membranes, most commonly the mouth. […] Pemphigus vulgaris affects all races and both sexes. […] It most commonly presents between the ages of 50-60 years, but can affect any age, although rarely children. […] The vast majority of patients have mucosal lesions, and the mouth is the most common site of presentation. […] Mucosal lesions may precede cutaneous lesions by months, or may be the only manifestation of the condition. […] The mucosa returns to normal when the condition is in remission. […] Patients will need referring urgently to dermatology. […] Supportive measures include wound care and reducing the risk of secondary infection by using antiseptic regimes, eg Dermol 500 lotion as a wash and / or topical emollient.
- #4 Pemphigus Vulgaris: A Complete Overview â DermNethttps://dermnetnz.org/topics/pemphigus-vulgaris
The primary aim of treatment of pemphigus vulgaris is to decrease blister formation, prevent infections and promote healing of blisters and erosions. Systemic corticosteroids are the mainstay of medical treatment for controlling the disease, usually in the form of moderate to high doses of oral prednisone or prednisolone, or as pulsed intravenous methylprednisolone. Since their use, many deaths from pemphigus vulgaris have been prevented (the mortality rate dropped from 99% to 515%). Corticosteroids are not a cure for the disease but improve the patient’s quality of life by reducing disease activity. The doses of corticosteroids needed to control pemphigus vulgaris and the length of time on treatment may result in serious side effects and risks. […] Other immunosuppressive drugs (mostly off-label) used to reduce the dose of steroids and may be required by patients with pemphigus vulgaris for years. These are most often: Azathioprine, Mycophenolate mofetil, Cyclophosphamide, Rituximab, which is now approved by the Food and Drug Administration (FDA) in the US.
- #5 Pemphigus vulgaris – approach and managementhttps://pmc.ncbi.nlm.nih.gov/articles/PMC6895778/
The gold standard for the diagnosis of PV is the detection of autoantibodies or complement component 3 by direct immunofluorescence microscopy of a perilesional biopsy. […] Early diagnosis and initiation of treatment are compulsory for a promising prognosis, and a multidisciplinary approach is recommended to optimize patient care.
- #6 Management of pemphigus vulgaris: challenges and solutionshttps://pmc.ncbi.nlm.nih.gov/articles/PMC4622091/
The main objective in the treatment of pemphigus vulgaris is to control the disease, prevent relapses, and avoid adverse events associated with the prolonged use of steroids and immunosuppressive agents. […] The primary objective of the therapeutic management of PV is initially to control the disease, heal the bullous skin and mucous lesions, and minimize the associated functional impairment. […] The challenge is to minimize hospitalization and improve patients quality of life. […] The aim of pharmacologic therapy for PV is to reduce inflammatory response and autoantibody production. […] Systemic CSs are still the first-line treatment for PV. […] A second debate often concerns whether to start with a low or high dose of CSs. […] Once remission is induced and maintained with healing of the majority of lesions, the dose can be tapered by 25%.
- #7 Pemphigus Vulgaris Treatment & Management: Medical Care, Complications, Diethttps://emedicine.medscape.com/article/1064187-treatment
The aims of treatment are the same in pemphigus vulgaris as in other autoimmune bullous diseasesnamely, to decrease blister formation, to promote healing of blisters and erosions, and to determine the minimal dose of medication necessary to control the disease process. Therapy must be tailored for each patient, taking into account preexisting and coexisting conditions. Patients may continue to experience mild disease activity while under optimal treatment. […] Wound care for erosions includes daily gentle cleaning, application of topical agents to promote wound healing, and use of nonadhesive dressings. The goal of wound care is to promote healing, minimize trauma to the surrounding skin, and diminish scarring. Epidermal growth factor (EGF) may speed healing of localized lesions. […] Management of patients with pemphigus vulgaris requires coordination of care between the dermatologist and the patient’s primary care physician. […] Patients receiving long-term systemic corticosteroid therapy should be evaluated by a rheumatologist within the first 30 days of treatment for osteoporosis risk assessment and consideration of a bisphosphonate for prophylaxis against osteoporosis.
- #8 Pemphigus: Types, Symptoms, Causes & Treatmenthttps://my.clevelandclinic.org/health/diseases/21130-pemphigus
Pemphigus vulgaris causes red and white fluid-filled blisters or open sores to form inside of your mouth. […] Pemphigus vulgaris causes blisters to form on your skin. A common location for blisters is near your groin and on the skin on your legs. […] Treatment is unique to each person diagnosed with pemphigus and could include: Taking medicine to prevent infections and help your skin heal. […] Your healthcare provider will treat your condition in stages. Most people go through all three stages of treatment, which include: Control: High doses of medications control the spread of blisters and begin healing existing ones. […] You can take steps at home to manage your symptoms of pemphigus by: Caring for your blisters as directed by your healthcare provider. […] Treatment for pemphigus takes time before you see results. With treatment, you’ll notice new blisters stop forming after several weeks and your skin will begin to heal.
- #9 Pemphigus vulgaris – approach and managementhttps://pmc.ncbi.nlm.nih.gov/articles/PMC6895778/
The role of treatment in patients with PV is to suppress the immune system and to prevent the production of pathogens. […] The British guidelines recommend the consideration of two stages in the management of PV: induction of remission and maintenance of remission. […] The first-line treatment is represented by corticosteroids, owing to their rapid effect (within days). […] When corticosteroids are administered over long periods of time, side effects such as diabetes, hypertension or osteoporosis may occur. […] Azathioprine and mycophenolate mofetil represent the first-line adjunctive immunosuppressive therapies in pemphigus, considered safe and efficient. […] The treatment for the severe and nonresponsive cases may be represented by corticosteroids in the form of intravenous pulse therapy, plasmapheresis and IVIG.
- #10 Pemphigus vulgaris: MedlinePlus Medical EncyclopediaLockhttps://medlineplus.gov/ency/article/000882.htm
Severe cases of PV may need wound management, similar to the treatment for severe burns. People with PV may need to stay in a hospital and receive care in a burn unit or intensive care unit. […] Treatment is aimed at reducing symptoms, including pain. It also aims to prevent complications, especially infections. […] Treatment may involve: Antibiotics and antifungal medicines to control or prevent infections, Fluids and electrolytes given through a vein (IV) if there are severe mouth ulcers, IV feedings if there are severe mouth ulcers, Numbing (anesthetic) mouth lozenges to reduce mouth ulcer pain, Pain medicines if local pain relief is not enough. […] Body-wide (systemic) therapy is needed to control PV and should be started as early as possible. Systemic treatment includes: An anti-inflammatory medicine called dapsone, Corticosteroids, Medicines containing gold, Medicines that suppress the immune system (such as azathioprine, methotrexate, cyclosporine, cyclophosphamide, mycophenolate mofetil, or rituximab).
- #11 Pemphigus Vulgaris: A Complete Overview â DermNethttps://dermnetnz.org/topics/pemphigus-vulgaris
The primary aim of treatment of pemphigus vulgaris is to decrease blister formation, prevent infections and promote healing of blisters and erosions. Systemic corticosteroids are the mainstay of medical treatment for controlling the disease, usually in the form of moderate to high doses of oral prednisone or prednisolone, or as pulsed intravenous methylprednisolone. Since their use, many deaths from pemphigus vulgaris have been prevented (the mortality rate dropped from 99% to 515%). Corticosteroids are not a cure for the disease but improve the patient’s quality of life by reducing disease activity. The doses of corticosteroids needed to control pemphigus vulgaris and the length of time on treatment may result in serious side effects and risks. […] Other immunosuppressive drugs (mostly off-label) used to reduce the dose of steroids and may be required by patients with pemphigus vulgaris for years. These are most often: Azathioprine, Mycophenolate mofetil, Cyclophosphamide, Rituximab, which is now approved by the Food and Drug Administration (FDA) in the US.
- #12 Managing pemphigus in primary carehttps://www.healthcert.com/blog/how-to-manage-pemphigus
Pemphigus is a complex condition that requires careful management. Primary care doctors, with adequate training in general dermatology, can play a vital role in early recognition, diagnosis, and prompt treatment of pemphigus, thereby improving the quality of life of their patients. […] Pemphigus vulgaris is a more severe form. The condition can lead to significant morbidity, so effective management is vital. […] Rapid initiation of treatment is essential to control the disease and prevent complications, such as secondary infections or fluid loss. The primary goals are to: Control blister formation, Reduce inflammation, and Minimize the need for long-term immunosuppression. […] High-dose systemic corticosteroids (prednisone) are the first-line treatment for pemphigus. Dosing typically starts at 1-2 mg/kg/day, adjusted based on the patients response and side effects.
- #13 Management of pemphigus vulgaris: challenges and solutionshttps://pmc.ncbi.nlm.nih.gov/articles/PMC4622091/
The main objective in the treatment of pemphigus vulgaris is to control the disease, prevent relapses, and avoid adverse events associated with the prolonged use of steroids and immunosuppressive agents. […] The primary objective of the therapeutic management of PV is initially to control the disease, heal the bullous skin and mucous lesions, and minimize the associated functional impairment. […] The challenge is to minimize hospitalization and improve patients quality of life. […] The aim of pharmacologic therapy for PV is to reduce inflammatory response and autoantibody production. […] Systemic CSs are still the first-line treatment for PV. […] A second debate often concerns whether to start with a low or high dose of CSs. […] Once remission is induced and maintained with healing of the majority of lesions, the dose can be tapered by 25%.
- #14 Pemphigus vulgaris – approach and managementhttps://pmc.ncbi.nlm.nih.gov/articles/PMC6895778/
The role of treatment in patients with PV is to suppress the immune system and to prevent the production of pathogens. […] The British guidelines recommend the consideration of two stages in the management of PV: induction of remission and maintenance of remission. […] The first-line treatment is represented by corticosteroids, owing to their rapid effect (within days). […] When corticosteroids are administered over long periods of time, side effects such as diabetes, hypertension or osteoporosis may occur. […] Azathioprine and mycophenolate mofetil represent the first-line adjunctive immunosuppressive therapies in pemphigus, considered safe and efficient. […] The treatment for the severe and nonresponsive cases may be represented by corticosteroids in the form of intravenous pulse therapy, plasmapheresis and IVIG.
- #15 Management of pemphigus vulgaris during acute phase – Indian Journal of Dermatology, Venereology and Leprologyhttps://ijdvl.com/management-of-pemphigus-vulgaris-during-acute-phase/
We present our experience with 21 patients of pemphigus vulgaris seen over a period of 10 years managed in service hospitals during acute phase of the disease. All the cases were hospitalized in ICU, till the acute phase of the disease subsided. The treatment comprised of potassium permanganate lotion bath (1:10000) and 1 framycetin gauze dressing of the denuded areas, maintenance of fluid and electrolyte balance. All suspected infections and septicemia were treated with appropriate antibiotics. The corticosteroids were usually administered as a single dose of prednisolone 1 mg/kg/day. Cyclophosphamide was given at an initial dose of 50mg/day and the dose was escalated to 100mg/day. Once the bulk of the lesions were healed, the dose of corticosteroids was gradually lowered by approximately 50% every two weeks and cyclophosphamide was continued till patients were symptom-free. Out of 21 patients receiving corticosteroids, cyclophosphamide and other supportive therapy, 20(95%) had undergone clinical resolution of the disease. During follow up study 15(71.4%) patients remained symptom-free and undergone clinical remission. The management of pemphigus vulgaris during active phase of the disease necessitates intensive care preferably in ICU till the main disease responsible for acute skin failure is under control. All the water and electrolyte needs of patients with pemphigus (PV) are maintained by a nasogastric silicone tube and venous access is used only a few hours a day for a discontinuous supply of macromolecules. Aggressive nutritional support is needed as soon as possible to minimize the protein losses and to promote tissue synthesis during the healing of cutaneous lesions. Infection is the main cause of death in all widespread skin involvement in PV. Corticosteroids are the mainstay of therapy for patients with pemphigus vulgaris. The aim of therapy is to find the lowest possible dosage of prednisolone, which will control the majority of the patients symptoms. Long term side effects associated with corticosteroid used include osteoporosis, peptic ulcer disease, aseptic vascular necrosis, cataract formation and unmasking of diabetes mellitus. Adjuvant therapy is meant to complement but not substitute for treatment with corticosteroids. Cyclophosphamide has been reported to be effective both as a first line adjuvant in patients with pemphigus and in the treatment of those whose disease has previously failed to respond to azathioprine.
- #16 Pemphigus Vulgaris Treatment & Management: Medical Care, Complications, Diethttps://emedicine.medscape.com/article/1064187-treatment
The aims of treatment are the same in pemphigus vulgaris as in other autoimmune bullous diseasesnamely, to decrease blister formation, to promote healing of blisters and erosions, and to determine the minimal dose of medication necessary to control the disease process. Therapy must be tailored for each patient, taking into account preexisting and coexisting conditions. Patients may continue to experience mild disease activity while under optimal treatment. […] Wound care for erosions includes daily gentle cleaning, application of topical agents to promote wound healing, and use of nonadhesive dressings. The goal of wound care is to promote healing, minimize trauma to the surrounding skin, and diminish scarring. Epidermal growth factor (EGF) may speed healing of localized lesions. […] Management of patients with pemphigus vulgaris requires coordination of care between the dermatologist and the patient’s primary care physician. […] Patients receiving long-term systemic corticosteroid therapy should be evaluated by a rheumatologist within the first 30 days of treatment for osteoporosis risk assessment and consideration of a bisphosphonate for prophylaxis against osteoporosis.
- #17 Pemphigus Vulgaris: A Complete Overview â DermNethttps://dermnetnz.org/topics/pemphigus-vulgaris
The primary aim of treatment of pemphigus vulgaris is to decrease blister formation, prevent infections and promote healing of blisters and erosions. Systemic corticosteroids are the mainstay of medical treatment for controlling the disease, usually in the form of moderate to high doses of oral prednisone or prednisolone, or as pulsed intravenous methylprednisolone. Since their use, many deaths from pemphigus vulgaris have been prevented (the mortality rate dropped from 99% to 515%). Corticosteroids are not a cure for the disease but improve the patient’s quality of life by reducing disease activity. The doses of corticosteroids needed to control pemphigus vulgaris and the length of time on treatment may result in serious side effects and risks. […] Other immunosuppressive drugs (mostly off-label) used to reduce the dose of steroids and may be required by patients with pemphigus vulgaris for years. These are most often: Azathioprine, Mycophenolate mofetil, Cyclophosphamide, Rituximab, which is now approved by the Food and Drug Administration (FDA) in the US.
- #18 Pemphigus vulgaris: MedlinePlus Medical EncyclopediaLockhttps://medlineplus.gov/ency/article/000882.htm
Severe cases of PV may need wound management, similar to the treatment for severe burns. People with PV may need to stay in a hospital and receive care in a burn unit or intensive care unit. […] Treatment is aimed at reducing symptoms, including pain. It also aims to prevent complications, especially infections. […] Treatment may involve: Antibiotics and antifungal medicines to control or prevent infections, Fluids and electrolytes given through a vein (IV) if there are severe mouth ulcers, IV feedings if there are severe mouth ulcers, Numbing (anesthetic) mouth lozenges to reduce mouth ulcer pain, Pain medicines if local pain relief is not enough. […] Body-wide (systemic) therapy is needed to control PV and should be started as early as possible. Systemic treatment includes: An anti-inflammatory medicine called dapsone, Corticosteroids, Medicines containing gold, Medicines that suppress the immune system (such as azathioprine, methotrexate, cyclosporine, cyclophosphamide, mycophenolate mofetil, or rituximab).
- #19 Pemphigus Vulgaris: Practice Essentials, Pathophysiology, Etiologyhttps://emedicine.medscape.com/article/1064187-overview
The aim of pharmacologic therapy for pemphigus vulgaris is to reduce inflammatory response and autoantibody production. Medications used in the diseases treatment include the following: […] Corticosteroids – Discourage the inflammatory process by inhibiting specific cytokine production […] Immunosuppressants – Should be considered early in the course of disease as steroid-sparing agents […] Rituximab […] Intravenous immune globulin (IVIG) […] Patients should be advised to minimize skin trauma because their skin will be more fragile than usual as a result of both the disease itself and the use of topical and systemic steroids to treat it. […] They should also be educated regarding their medications. If patients are informed about dosages, adverse effects, and symptoms of toxicity, they will be better able to report any adverse effects to the physician. […] Finally, instructions regarding appropriate wound care should be provided.
- #20 Pemphigus vulgaris – approach and managementhttps://pmc.ncbi.nlm.nih.gov/articles/PMC6895778/
The role of treatment in patients with PV is to suppress the immune system and to prevent the production of pathogens. […] The British guidelines recommend the consideration of two stages in the management of PV: induction of remission and maintenance of remission. […] The first-line treatment is represented by corticosteroids, owing to their rapid effect (within days). […] When corticosteroids are administered over long periods of time, side effects such as diabetes, hypertension or osteoporosis may occur. […] Azathioprine and mycophenolate mofetil represent the first-line adjunctive immunosuppressive therapies in pemphigus, considered safe and efficient. […] The treatment for the severe and nonresponsive cases may be represented by corticosteroids in the form of intravenous pulse therapy, plasmapheresis and IVIG.
- #21 Pemphigus vulgaris: MedlinePlus Medical EncyclopediaLockhttps://medlineplus.gov/ency/article/000882.htm
Severe cases of PV may need wound management, similar to the treatment for severe burns. People with PV may need to stay in a hospital and receive care in a burn unit or intensive care unit. […] Treatment is aimed at reducing symptoms, including pain. It also aims to prevent complications, especially infections. […] Treatment may involve: Antibiotics and antifungal medicines to control or prevent infections, Fluids and electrolytes given through a vein (IV) if there are severe mouth ulcers, IV feedings if there are severe mouth ulcers, Numbing (anesthetic) mouth lozenges to reduce mouth ulcer pain, Pain medicines if local pain relief is not enough. […] Body-wide (systemic) therapy is needed to control PV and should be started as early as possible. Systemic treatment includes: An anti-inflammatory medicine called dapsone, Corticosteroids, Medicines containing gold, Medicines that suppress the immune system (such as azathioprine, methotrexate, cyclosporine, cyclophosphamide, mycophenolate mofetil, or rituximab).
- #22 Pemphigus vulgaris: MedlinePlus Medical EncyclopediaLockhttps://medlineplus.gov/ency/article/000882.htm
Antibiotics may be used to treat or prevent infection. Intravenous immunoglobulin (IVIg) is occasionally used. […] Plasmapheresis may be used along with systemic medicines to reduce the amount of antibodies in the blood. Plasmapheresis is a process in which antibody-containing plasma is removed from the blood and replaced with intravenous fluids or donated plasma. […] Ulcer and blister treatments include soothing or drying lotions, wet dressings, or similar measures.
- #23 Pemphigus vulgaris: MedlinePlus Medical EncyclopediaLockhttps://medlineplus.gov/ency/article/000882.htm
Severe cases of PV may need wound management, similar to the treatment for severe burns. People with PV may need to stay in a hospital and receive care in a burn unit or intensive care unit. […] Treatment is aimed at reducing symptoms, including pain. It also aims to prevent complications, especially infections. […] Treatment may involve: Antibiotics and antifungal medicines to control or prevent infections, Fluids and electrolytes given through a vein (IV) if there are severe mouth ulcers, IV feedings if there are severe mouth ulcers, Numbing (anesthetic) mouth lozenges to reduce mouth ulcer pain, Pain medicines if local pain relief is not enough. […] Body-wide (systemic) therapy is needed to control PV and should be started as early as possible. Systemic treatment includes: An anti-inflammatory medicine called dapsone, Corticosteroids, Medicines containing gold, Medicines that suppress the immune system (such as azathioprine, methotrexate, cyclosporine, cyclophosphamide, mycophenolate mofetil, or rituximab).
- #24 Pemphigus Vulgaris – UF Healthhttps://ufhealth.org/conditions-and-treatments/pemphigus-vulgaris
Severe cases of pemphigus may need wound management, similar to the treatment for severe burns. People with PV may need to stay in a hospital and receive care in a burn unit or intensive care unit. […] Treatment is aimed at reducing symptoms, including pain. It also aims to prevent complications, especially infections. […] Treatment may involve: Antibiotics and antifungal medicines to control or prevent infections, Fluids and electrolytes given through a vein (IV) if there are severe mouth ulcers, IV feedings if there are severe mouth ulcers, Numbing (anesthetic) mouth lozenges to reduce mouth ulcer pain, Pain medicines if local pain relief is not enough. […] Body-wide (systemic) therapy is needed to control pemphigus and should be started as early as possible. Systemic treatment includes: An anti-inflammatory medicine called dapsone, Corticosteroids, Medicines containing gold, Medicines that suppress the immune system (such as azathioprine, methotrexate, cyclosporine, cyclophosphamide, mycophenolate mofetil, or rituximab).
- #25 Management of chronic wounds in patients with pemphigus | CWCMRhttps://www.dovepress.com/management-of-chronic-wounds-in-patients-with-pemphigus-peer-reviewed-fulltext-article-CWCMR
The four main goals of wound care are (1) prevention of infection, (2) maintenance of a moist environment, (3) protection of the wound, and (4) minimizing scar formation. small blisters should be left intact, to prevent secondary infection. However, large blisters should be punctured and aspirated with a large (18-gauge) sterile needle, to keep the blister roof in place for wound protection. Gentle cleansing is an early priority and can be accomplished by normal saline or antibacterial soap/antiseptic twice a day. Allow it to dry well. Next, a bland emollient ointment, such as 50% white soft paraffin +50% liquid paraffin, is applied directly to the wound bed, or apply the ointment to a dressing. If a wound is wet, the dressing should absorb excess exudate. If a wound is dry, the dressing should provide moisture to the wound bed. Pemphigus is characterized by superficial wounds, where the damage is generally limited to the epidermis and dressing is comparable to dressing surgical wounds except that extra attention should be paid to early closure and prevention of fluid losses through evaporation. This can be accomplished by using moisture retentive dressings, either occlusive or semiocclusive, help promote reepithelialization and help restore the skin barrier. Non-adherent gauze, transparent film, hydrocolloid, and hydrogel dressings may be used directly on the wounds. When selecting a dressing, one should take into account not only wound characteristics, but also specific dressing features such as absorptive capacity, hydrating quality, adhesive quality, and comfortability. Various types of wound dressing are highlighted in (Table 3). All dressing products used for pemphigus patients must be non-adherent.
- #26 Management of chronic wounds in patients with pemphigus | CWCMRhttps://www.dovepress.com/management-of-chronic-wounds-in-patients-with-pemphigus-peer-reviewed-fulltext-article-CWCMR
The four main goals of wound care are (1) prevention of infection, (2) maintenance of a moist environment, (3) protection of the wound, and (4) minimizing scar formation. small blisters should be left intact, to prevent secondary infection. However, large blisters should be punctured and aspirated with a large (18-gauge) sterile needle, to keep the blister roof in place for wound protection. Gentle cleansing is an early priority and can be accomplished by normal saline or antibacterial soap/antiseptic twice a day. Allow it to dry well. Next, a bland emollient ointment, such as 50% white soft paraffin +50% liquid paraffin, is applied directly to the wound bed, or apply the ointment to a dressing. If a wound is wet, the dressing should absorb excess exudate. If a wound is dry, the dressing should provide moisture to the wound bed. Pemphigus is characterized by superficial wounds, where the damage is generally limited to the epidermis and dressing is comparable to dressing surgical wounds except that extra attention should be paid to early closure and prevention of fluid losses through evaporation. This can be accomplished by using moisture retentive dressings, either occlusive or semiocclusive, help promote reepithelialization and help restore the skin barrier. Non-adherent gauze, transparent film, hydrocolloid, and hydrogel dressings may be used directly on the wounds. When selecting a dressing, one should take into account not only wound characteristics, but also specific dressing features such as absorptive capacity, hydrating quality, adhesive quality, and comfortability. Various types of wound dressing are highlighted in (Table 3). All dressing products used for pemphigus patients must be non-adherent.
- #27 Pemphigus Vulgaris: A Complete Overview â DermNethttps://dermnetnz.org/topics/pemphigus-vulgaris
Appropriate wound care is particularly important, as this should promote healing of blisters and erosions. Handle skin very gently to avoid causing new blisters and erosions. Wear surgical gloves and use aseptic technique when changing dressings. Analgesics may be needed especially for dressing changes. Gently cleanse with an antiseptic solution or take a bleach bath. Drain intact blisters, but leave the blister roof in place. Apply a bland emollient ointment, such as 50% white soft paraffin + 50% liquid paraffin, directly to the skin, or apply the ointment to a dressing. Use non-adherent dressings (such as petrolatum-soaked gauze or silicone mesh). These may incorporate an antiseptic. An absorbent dressing may be applied over the primary dressing if the erosions are oozing. Be vigilant in identifying and treating any infection.
- #28 Management of chronic wounds in patients with pemphigus | CWCMRhttps://www.dovepress.com/management-of-chronic-wounds-in-patients-with-pemphigus-peer-reviewed-fulltext-article-CWCMR
The four main goals of wound care are (1) prevention of infection, (2) maintenance of a moist environment, (3) protection of the wound, and (4) minimizing scar formation. small blisters should be left intact, to prevent secondary infection. However, large blisters should be punctured and aspirated with a large (18-gauge) sterile needle, to keep the blister roof in place for wound protection. Gentle cleansing is an early priority and can be accomplished by normal saline or antibacterial soap/antiseptic twice a day. Allow it to dry well. Next, a bland emollient ointment, such as 50% white soft paraffin +50% liquid paraffin, is applied directly to the wound bed, or apply the ointment to a dressing. If a wound is wet, the dressing should absorb excess exudate. If a wound is dry, the dressing should provide moisture to the wound bed. Pemphigus is characterized by superficial wounds, where the damage is generally limited to the epidermis and dressing is comparable to dressing surgical wounds except that extra attention should be paid to early closure and prevention of fluid losses through evaporation. This can be accomplished by using moisture retentive dressings, either occlusive or semiocclusive, help promote reepithelialization and help restore the skin barrier. Non-adherent gauze, transparent film, hydrocolloid, and hydrogel dressings may be used directly on the wounds. When selecting a dressing, one should take into account not only wound characteristics, but also specific dressing features such as absorptive capacity, hydrating quality, adhesive quality, and comfortability. Various types of wound dressing are highlighted in (Table 3). All dressing products used for pemphigus patients must be non-adherent.
- #29 Management of chronic wounds in patients with pemphigus | CWCMRhttps://www.dovepress.com/management-of-chronic-wounds-in-patients-with-pemphigus-peer-reviewed-fulltext-article-CWCMR
The four main goals of wound care are (1) prevention of infection, (2) maintenance of a moist environment, (3) protection of the wound, and (4) minimizing scar formation. small blisters should be left intact, to prevent secondary infection. However, large blisters should be punctured and aspirated with a large (18-gauge) sterile needle, to keep the blister roof in place for wound protection. Gentle cleansing is an early priority and can be accomplished by normal saline or antibacterial soap/antiseptic twice a day. Allow it to dry well. Next, a bland emollient ointment, such as 50% white soft paraffin +50% liquid paraffin, is applied directly to the wound bed, or apply the ointment to a dressing. If a wound is wet, the dressing should absorb excess exudate. If a wound is dry, the dressing should provide moisture to the wound bed. Pemphigus is characterized by superficial wounds, where the damage is generally limited to the epidermis and dressing is comparable to dressing surgical wounds except that extra attention should be paid to early closure and prevention of fluid losses through evaporation. This can be accomplished by using moisture retentive dressings, either occlusive or semiocclusive, help promote reepithelialization and help restore the skin barrier. Non-adherent gauze, transparent film, hydrocolloid, and hydrogel dressings may be used directly on the wounds. When selecting a dressing, one should take into account not only wound characteristics, but also specific dressing features such as absorptive capacity, hydrating quality, adhesive quality, and comfortability. Various types of wound dressing are highlighted in (Table 3). All dressing products used for pemphigus patients must be non-adherent.
- #30 Management of chronic wounds in patients with pemphigus | CWCMRhttps://www.dovepress.com/management-of-chronic-wounds-in-patients-with-pemphigus-peer-reviewed-fulltext-article-CWCMR
The four main goals of wound care are (1) prevention of infection, (2) maintenance of a moist environment, (3) protection of the wound, and (4) minimizing scar formation. small blisters should be left intact, to prevent secondary infection. However, large blisters should be punctured and aspirated with a large (18-gauge) sterile needle, to keep the blister roof in place for wound protection. Gentle cleansing is an early priority and can be accomplished by normal saline or antibacterial soap/antiseptic twice a day. Allow it to dry well. Next, a bland emollient ointment, such as 50% white soft paraffin +50% liquid paraffin, is applied directly to the wound bed, or apply the ointment to a dressing. If a wound is wet, the dressing should absorb excess exudate. If a wound is dry, the dressing should provide moisture to the wound bed. Pemphigus is characterized by superficial wounds, where the damage is generally limited to the epidermis and dressing is comparable to dressing surgical wounds except that extra attention should be paid to early closure and prevention of fluid losses through evaporation. This can be accomplished by using moisture retentive dressings, either occlusive or semiocclusive, help promote reepithelialization and help restore the skin barrier. Non-adherent gauze, transparent film, hydrocolloid, and hydrogel dressings may be used directly on the wounds. When selecting a dressing, one should take into account not only wound characteristics, but also specific dressing features such as absorptive capacity, hydrating quality, adhesive quality, and comfortability. Various types of wound dressing are highlighted in (Table 3). All dressing products used for pemphigus patients must be non-adherent.
- #31 Pemphigus Vulgaris: A Complete Overview â DermNethttps://dermnetnz.org/topics/pemphigus-vulgaris
Appropriate wound care is particularly important, as this should promote healing of blisters and erosions. Handle skin very gently to avoid causing new blisters and erosions. Wear surgical gloves and use aseptic technique when changing dressings. Analgesics may be needed especially for dressing changes. Gently cleanse with an antiseptic solution or take a bleach bath. Drain intact blisters, but leave the blister roof in place. Apply a bland emollient ointment, such as 50% white soft paraffin + 50% liquid paraffin, directly to the skin, or apply the ointment to a dressing. Use non-adherent dressings (such as petrolatum-soaked gauze or silicone mesh). These may incorporate an antiseptic. An absorbent dressing may be applied over the primary dressing if the erosions are oozing. Be vigilant in identifying and treating any infection.
- #32 Management of chronic wounds in patients with pemphigus | CWCMRhttps://www.dovepress.com/management-of-chronic-wounds-in-patients-with-pemphigus-peer-reviewed-fulltext-article-CWCMR
The four main goals of wound care are (1) prevention of infection, (2) maintenance of a moist environment, (3) protection of the wound, and (4) minimizing scar formation. small blisters should be left intact, to prevent secondary infection. However, large blisters should be punctured and aspirated with a large (18-gauge) sterile needle, to keep the blister roof in place for wound protection. Gentle cleansing is an early priority and can be accomplished by normal saline or antibacterial soap/antiseptic twice a day. Allow it to dry well. Next, a bland emollient ointment, such as 50% white soft paraffin +50% liquid paraffin, is applied directly to the wound bed, or apply the ointment to a dressing. If a wound is wet, the dressing should absorb excess exudate. If a wound is dry, the dressing should provide moisture to the wound bed. Pemphigus is characterized by superficial wounds, where the damage is generally limited to the epidermis and dressing is comparable to dressing surgical wounds except that extra attention should be paid to early closure and prevention of fluid losses through evaporation. This can be accomplished by using moisture retentive dressings, either occlusive or semiocclusive, help promote reepithelialization and help restore the skin barrier. Non-adherent gauze, transparent film, hydrocolloid, and hydrogel dressings may be used directly on the wounds. When selecting a dressing, one should take into account not only wound characteristics, but also specific dressing features such as absorptive capacity, hydrating quality, adhesive quality, and comfortability. Various types of wound dressing are highlighted in (Table 3). All dressing products used for pemphigus patients must be non-adherent.
- #33 Pemphigus Vulgaris: A Complete Overview â DermNethttps://dermnetnz.org/topics/pemphigus-vulgaris
Appropriate wound care is particularly important, as this should promote healing of blisters and erosions. Handle skin very gently to avoid causing new blisters and erosions. Wear surgical gloves and use aseptic technique when changing dressings. Analgesics may be needed especially for dressing changes. Gently cleanse with an antiseptic solution or take a bleach bath. Drain intact blisters, but leave the blister roof in place. Apply a bland emollient ointment, such as 50% white soft paraffin + 50% liquid paraffin, directly to the skin, or apply the ointment to a dressing. Use non-adherent dressings (such as petrolatum-soaked gauze or silicone mesh). These may incorporate an antiseptic. An absorbent dressing may be applied over the primary dressing if the erosions are oozing. Be vigilant in identifying and treating any infection.
- #34 Pemphigus Vulgaris: A Complete Overview â DermNethttps://dermnetnz.org/topics/pemphigus-vulgaris
Appropriate wound care is particularly important, as this should promote healing of blisters and erosions. Handle skin very gently to avoid causing new blisters and erosions. Wear surgical gloves and use aseptic technique when changing dressings. Analgesics may be needed especially for dressing changes. Gently cleanse with an antiseptic solution or take a bleach bath. Drain intact blisters, but leave the blister roof in place. Apply a bland emollient ointment, such as 50% white soft paraffin + 50% liquid paraffin, directly to the skin, or apply the ointment to a dressing. Use non-adherent dressings (such as petrolatum-soaked gauze or silicone mesh). These may incorporate an antiseptic. An absorbent dressing may be applied over the primary dressing if the erosions are oozing. Be vigilant in identifying and treating any infection.
- #35 Pemphigus Vulgaris: Cause, Types, and Symptomshttps://www.healthline.com/health/pemphigus-vulgaris
If the blisters in your mouth keep you from brushing or flossing your teeth, you may need special oral health treatment to prevent gum disease and tooth decay. See your dentist to ask them about oral care. […] The complications of pemphigus vulgaris can be fatal and severe. […] If left untreated, pemphigus vulgaris can be life-threatening. The most common cause of death is a severe secondary infection. […] Pemphigus vulgaris is a lifelong condition. It cannot be cured. However, most people go into remission after receiving corticosteroids. Improvement is usually noticed within days of starting the corticosteroids.
- #36 Pemphigus: 10 tips for managinghttps://www.aad.org/public/diseases/a-z/pemphigus-self-care
If you are unsure how to take care of your blisters and sores, ask your dermatologist. […] Pemphigus can be a serious disease that changes your life. The following can help you manage life with pemphigus. […] Take precautions to avoid infections. Blisters and sores leave you more likely to develop an infection. […] Take care of mouth sores. If pemphigus causes sores in your mouth, you can ease the pain by: Eating bland, soft foods. […] Tell your dermatologist about eye problems. Blisters can form on the tissue lining an eyelid. […] Avoid sun and heat if you have pemphigus foliaceus or drug-induced pemphigus. […] Partner with your dermatologist. Your dermatologist can be your strongest ally. Dermatologists help patients with pemphigus find treatment that works.
- #37 Pemphigus Vulgaris – UF Healthhttps://ufhealth.org/conditions-and-treatments/pemphigus-vulgaris
Severe cases of pemphigus may need wound management, similar to the treatment for severe burns. People with PV may need to stay in a hospital and receive care in a burn unit or intensive care unit. […] Treatment is aimed at reducing symptoms, including pain. It also aims to prevent complications, especially infections. […] Treatment may involve: Antibiotics and antifungal medicines to control or prevent infections, Fluids and electrolytes given through a vein (IV) if there are severe mouth ulcers, IV feedings if there are severe mouth ulcers, Numbing (anesthetic) mouth lozenges to reduce mouth ulcer pain, Pain medicines if local pain relief is not enough. […] Body-wide (systemic) therapy is needed to control pemphigus and should be started as early as possible. Systemic treatment includes: An anti-inflammatory medicine called dapsone, Corticosteroids, Medicines containing gold, Medicines that suppress the immune system (such as azathioprine, methotrexate, cyclosporine, cyclophosphamide, mycophenolate mofetil, or rituximab).
- #38 Pemphigus: Symptoms, Causes, Diagnosis, Treatment, and Copinghttps://www.verywellhealth.com/pemphigus-4691395
Pemphigus vulgaris will generally affect mucosal membranes of the mouth first, causing multiple ulcers that can persist for weeks and months. […] If not treated immediately, pemphigus can be fatal, usually as the result of overwhelming opportunistic infections. Because of this, pemphigus may require hospitalization and involve many of the same interventions used in burn centers. […] The mainstay of pemphigus treatment is oral corticosteroids, usually prednisone. This typically requires extremely high doses, which can be dangerous for some, causing intestinal perforation and sepsis. […] If oral corticosteroids cannot be used, other options may be considered, including local steroid injections, immunosuppressant drugs, intravenous gamma globulin (IVIG), usually reserved for severe paraneoplastic pemphigus, and biologic drugs, like Rituxan (rituximab) if other drug treatments fail. […] Many people get better with treatment, although it can sometimes take years to fully recover. Others will need to take medications permanently to prevent a recurrence.
- #39 Disease Management – IPPFhttps://www.pemphigus.org/disease-management/
Oral hygiene is crucial. Otherwise PV may be complicated by dental decay; toothbrushing should be encouraged and antiseptic mouthwashes may be used, such as chlorhexidine gluconate 0.2% or 1:4 hydrogen peroxide solutions. […] Good oral hygiene is very important to positive treatment outcomes. Poor oral hygiene with dental plaque and gingival inflammation may reduce and delay treatment outcome. […] Oral hygiene instructions for home care may need to be tailored based on the level of mucosal involvement. When significant oral disease is present, gentle home care including extra-soft toothbrushes, mildly flavored toothpastes, and mild mouth rinses may be all that the patient can tolerate. Some patients may not be able to floss due to bleeding and pain. During this time, frequent dental cleanings become more important. Once the oral ulcerations and associated pain decrease, the standard home care routine, including regular brushing and flossing, can be recommended.
- #40 Disease Management – IPPFhttps://www.pemphigus.org/disease-management/
Oral hygiene is crucial. Otherwise PV may be complicated by dental decay; toothbrushing should be encouraged and antiseptic mouthwashes may be used, such as chlorhexidine gluconate 0.2% or 1:4 hydrogen peroxide solutions. […] Good oral hygiene is very important to positive treatment outcomes. Poor oral hygiene with dental plaque and gingival inflammation may reduce and delay treatment outcome. […] Oral hygiene instructions for home care may need to be tailored based on the level of mucosal involvement. When significant oral disease is present, gentle home care including extra-soft toothbrushes, mildly flavored toothpastes, and mild mouth rinses may be all that the patient can tolerate. Some patients may not be able to floss due to bleeding and pain. During this time, frequent dental cleanings become more important. Once the oral ulcerations and associated pain decrease, the standard home care routine, including regular brushing and flossing, can be recommended.
- #41 Pemphigus Vulgaris: A Complete Overview â DermNethttps://dermnetnz.org/topics/pemphigus-vulgaris
Patients should minimise activities that may traumatise the skin and mucous membranes during active phases of the disease. These include activities such as contact sports and eating or drinking food that may irritate or damage the inside of the mouth (spicy, acidic, hard and crunchy foods). Oral hygiene and proper dental care are essential. Use a soft toothbrush and mint-free toothpaste to gently and thoroughly brush teeth twice daily. Rinse using an antiseptic or anti-inflammatory mouthwash. Treat oral candidiasis, if present.
- #42 Pemphigus vulgarishttps://www.nhs.uk/conditions/pemphigus-vulgaris/
If you have pemphigus vulgaris, there are some things you can do to help ease the symptoms and reduce the risk of further problems. […] clean your teeth using a soft toothbrush and mint-free toothpaste […] use antiseptic or painkilling (anaesthetic) mouthwash (try to avoid mouthwashes that contain alcohol) […] keep any sore patches clean to reduce the risk of infection […] have regular dental check-ups. […] do not eat spicy, acidic or hard foods if you have blisters in your mouth […] do not do activities that could damage your skin (such as contact sports) if you have blisters on your skin. […] You’ll have regular check-ups to look for these problems and may be referred to other specialists (such as a dietitian) if needed.
- #43 Disease Management – IPPFhttps://www.pemphigus.org/disease-management/
Oral hygiene is crucial. Otherwise PV may be complicated by dental decay; toothbrushing should be encouraged and antiseptic mouthwashes may be used, such as chlorhexidine gluconate 0.2% or 1:4 hydrogen peroxide solutions. […] Good oral hygiene is very important to positive treatment outcomes. Poor oral hygiene with dental plaque and gingival inflammation may reduce and delay treatment outcome. […] Oral hygiene instructions for home care may need to be tailored based on the level of mucosal involvement. When significant oral disease is present, gentle home care including extra-soft toothbrushes, mildly flavored toothpastes, and mild mouth rinses may be all that the patient can tolerate. Some patients may not be able to floss due to bleeding and pain. During this time, frequent dental cleanings become more important. Once the oral ulcerations and associated pain decrease, the standard home care routine, including regular brushing and flossing, can be recommended.
- #44 Pemphigus: 10 tips for managinghttps://www.aad.org/public/diseases/a-z/pemphigus-self-care
If you are unsure how to take care of your blisters and sores, ask your dermatologist. […] Pemphigus can be a serious disease that changes your life. The following can help you manage life with pemphigus. […] Take precautions to avoid infections. Blisters and sores leave you more likely to develop an infection. […] Take care of mouth sores. If pemphigus causes sores in your mouth, you can ease the pain by: Eating bland, soft foods. […] Tell your dermatologist about eye problems. Blisters can form on the tissue lining an eyelid. […] Avoid sun and heat if you have pemphigus foliaceus or drug-induced pemphigus. […] Partner with your dermatologist. Your dermatologist can be your strongest ally. Dermatologists help patients with pemphigus find treatment that works.
- #45 Pemphigus Vulgaris – UF Healthhttps://ufhealth.org/conditions-and-treatments/pemphigus-vulgaris
Severe cases of pemphigus may need wound management, similar to the treatment for severe burns. People with PV may need to stay in a hospital and receive care in a burn unit or intensive care unit. […] Treatment is aimed at reducing symptoms, including pain. It also aims to prevent complications, especially infections. […] Treatment may involve: Antibiotics and antifungal medicines to control or prevent infections, Fluids and electrolytes given through a vein (IV) if there are severe mouth ulcers, IV feedings if there are severe mouth ulcers, Numbing (anesthetic) mouth lozenges to reduce mouth ulcer pain, Pain medicines if local pain relief is not enough. […] Body-wide (systemic) therapy is needed to control pemphigus and should be started as early as possible. Systemic treatment includes: An anti-inflammatory medicine called dapsone, Corticosteroids, Medicines containing gold, Medicines that suppress the immune system (such as azathioprine, methotrexate, cyclosporine, cyclophosphamide, mycophenolate mofetil, or rituximab).
- #46 Pemphigus vulgaris: MedlinePlus Medical EncyclopediaLockhttps://medlineplus.gov/ency/article/000882.htm
Severe cases of PV may need wound management, similar to the treatment for severe burns. People with PV may need to stay in a hospital and receive care in a burn unit or intensive care unit. […] Treatment is aimed at reducing symptoms, including pain. It also aims to prevent complications, especially infections. […] Treatment may involve: Antibiotics and antifungal medicines to control or prevent infections, Fluids and electrolytes given through a vein (IV) if there are severe mouth ulcers, IV feedings if there are severe mouth ulcers, Numbing (anesthetic) mouth lozenges to reduce mouth ulcer pain, Pain medicines if local pain relief is not enough. […] Body-wide (systemic) therapy is needed to control PV and should be started as early as possible. Systemic treatment includes: An anti-inflammatory medicine called dapsone, Corticosteroids, Medicines containing gold, Medicines that suppress the immune system (such as azathioprine, methotrexate, cyclosporine, cyclophosphamide, mycophenolate mofetil, or rituximab).
- #47 Pemphigus Vulgaris: A Complete Overview â DermNethttps://dermnetnz.org/topics/pemphigus-vulgaris
Patients should minimise activities that may traumatise the skin and mucous membranes during active phases of the disease. These include activities such as contact sports and eating or drinking food that may irritate or damage the inside of the mouth (spicy, acidic, hard and crunchy foods). Oral hygiene and proper dental care are essential. Use a soft toothbrush and mint-free toothpaste to gently and thoroughly brush teeth twice daily. Rinse using an antiseptic or anti-inflammatory mouthwash. Treat oral candidiasis, if present.
- #48 Pemphigus vulgarishttps://www.nhs.uk/conditions/pemphigus-vulgaris/
If you have pemphigus vulgaris, there are some things you can do to help ease the symptoms and reduce the risk of further problems. […] clean your teeth using a soft toothbrush and mint-free toothpaste […] use antiseptic or painkilling (anaesthetic) mouthwash (try to avoid mouthwashes that contain alcohol) […] keep any sore patches clean to reduce the risk of infection […] have regular dental check-ups. […] do not eat spicy, acidic or hard foods if you have blisters in your mouth […] do not do activities that could damage your skin (such as contact sports) if you have blisters on your skin. […] You’ll have regular check-ups to look for these problems and may be referred to other specialists (such as a dietitian) if needed.
- #49 Pemphigus Vulgaris: Symptoms, Causes & Treatmenthttps://my.clevelandclinic.org/health/diseases/23033-pemphigus-vulgaris
Pemphigus vulgaris is an autoimmune condition that causes blisters to form on your skin and mucous membranes. […] Treatment for pemphigus vulgaris focuses on managing symptoms and preventing complications like infections. Treatment is unique to each person and could include: […] Caring for sores and broken blisters as you would a burn or wound. […] Eating a bland diet or receiving nutrients through an IV to avoid malnutrition if sores in your mouth prevent you from eating. […] You can take care of yourself and manage your symptoms of pemphigus vulgaris at home by: […] Cleaning and caring for your blisters and sores like wounds or burns. […] Visit your healthcare provider if you have: […] Trouble eating and drinking. […] Talk to your healthcare provider about treatment options unique to your symptoms.
- #50 Pemphigus: Types, Symptoms, Causes & Treatmenthttps://my.clevelandclinic.org/health/diseases/21130-pemphigus
Your healthcare provider will monitor your condition regularly and order blood and/or urine tests to verify that your treatment is working. Tests also check for negative reactions to medications that treat pemphigus. […] If you have trouble eating, contact your healthcare provider. They may recommend taking nutritional supplements to avoid malnutrition. […] The majority of people diagnosed with pemphigus have a normal lifespan. Treatment helps manage symptoms and could be ongoing throughout your life.
- #51 Effect of Implementing a Protocol of Nursing Care on Pemphigus Vulgaris Patients’ Clinical Health Outcomes.https://ejhc.journals.ekb.eg/article_380830.html
Pemphigus Vulgaris is a skin disease recognized for its characteristic autoantibody production, which assail skin proteins, leading to the formation of blisters and erosions that require special nursing care to maximize patients health outcomes. […] Nursings care protocol was effective in improving patients clinical health outcomes. […] Creation of an educational unit for Nursing Care on Pemphigus of Vulgaris Patients in the dermatology hospital departments. Conducting similar studies on a larger probability sample is recommended to achieve generalization of the findings.
- #52 The nursing assessment of pemphigus vulgaris ulcers :: Cambridge Media Journalshttps://journals.cambridgemedia.com.au/wcetfr/volume-41-number-3/nursing-assessment-pemphigus-vulgaris-ulcers
The nursing assessment of pemphigus vulgaris ulcers is crucial for early treatment and monitoring disease progression. […] Nurses need to recognize skin lesions associated with pemphigus vulgaris and communicate appropriate findings. […] The purpose of this integrative review was to describe the taxonomy for the description and assessment of skin changes related to pemphigus vulgaris by nurses. […] Studies with better levels of evidence are needed on this issue in order to determine the best way to describe the lesions using the dermatological glossary for nursing assessment. […] The analysis of the nursing diagnosis impaired skin integrity and impaired oral mucous membrane made it possible to perceive that the nurse assessment to arrive at this diagnosis does not require a specialised evaluation since the defining characteristics for such diagnoses involve only the rupture of the skin or mucosa.
- #53 The nursing assessment of pemphigus vulgaris ulcers :: Cambridge Media Journalshttps://journals.cambridgemedia.com.au/wcetfr/volume-41-number-3/nursing-assessment-pemphigus-vulgaris-ulcers
The proposed NIC intervention wound care involves the decision about the intervention to be adopted, presupposing specific knowledge, skills and preparation in this area. […] As the nomenclature used to describe skin lesions becomes standard, whether secondary to pemphigus vulgaris or not, the interlocution between professionals tends to become more uniform, avoiding misunderstanding and resulting in safer patient care. […] In order to standardise the register of pemphigus vulgaris ulcers, we suggest the use of the elementary lesions terms vesicles, blisters, erosions, ulcers and generalised crusts.
- #54 The nursing assessment of pemphigus vulgaris ulcers :: Cambridge Media Journalshttps://journals.cambridgemedia.com.au/wcetfr/volume-41-number-3/nursing-assessment-pemphigus-vulgaris-ulcers
The proposed NIC intervention wound care involves the decision about the intervention to be adopted, presupposing specific knowledge, skills and preparation in this area. […] As the nomenclature used to describe skin lesions becomes standard, whether secondary to pemphigus vulgaris or not, the interlocution between professionals tends to become more uniform, avoiding misunderstanding and resulting in safer patient care. […] In order to standardise the register of pemphigus vulgaris ulcers, we suggest the use of the elementary lesions terms vesicles, blisters, erosions, ulcers and generalised crusts.
- #55 Phemphigus | PPThttps://www.slideshare.net/sivanandareddy52/phemphigus
Pemphigus vulgaris is a blistering autoimmune disease that affects the skin and mucous membranes. […] The goals of therapy are to bring the disease under control as rapidly as possible, to prevent loss of serum and the development of secondary infection, and to promote re-epithelization. […] Corticosteroids are administered in high doses to control the disease and keep the skin free of blisters. In some cases, corticosteroid therapy must be maintained for life. […] Immunosuppressive agents (eg, Azathioprine, Cyclophosphamide) may be prescribed to help control the disease and reduce the corticosteroid dose. […] Nursing interventions include meticulous oral hygiene to keep the oral mucosa clean and allow the epithelium to regenerate. […] Frequent rinsing of the mouth is prescribed to rid the mouth of debris and to soothe ulcerated areas.
- #56 Phemphigus | PPThttps://www.slideshare.net/sivanandareddy52/phemphigus
The patient with painful and extensive lesions should be pre-medicated with analgesics before skin care is initiated. […] The patient is encouraged to express freely anxieties, discomfort, and feelings of hopelessness. […] The skin is cleaned to remove debris and dead skin and to prevent infection. […] Antimicrobial agents are administered as prescribed, and response to treatment is assessed. […] The patient is encouraged to maintain adequate oral fluid intake.
- #57 Management of chronic wounds in patients with pemphigus | CWCMRhttps://www.dovepress.com/management-of-chronic-wounds-in-patients-with-pemphigus-peer-reviewed-fulltext-article-CWCMR
When the disease becomes severe with extensive skin involvement, patients are typically admitted to the burn unit. Therefore, supportive care resembles that performed for severe thermal burns TEN. It aims at minimizing potential complications, which may ultimately lead to patient mortality. For instance, it aims to avoid hypovolemia, electrolyte imbalance, renal insufficiency, and sepsis. […] Nutrition is a crucial aspect of care, due to the loss of protein and other essential components in the serous sanguineous drainage. Patients should avoid excessive skin manipulation and minimize activities that may traumatize the skin and mucous membranes during active phases of the disease. These include contact sports and eating or drinking food that may irritate the oral cavity (spicy, acidic, hard and crunchy foods).
- #58 Pemphigus: 10 tips for managinghttps://www.aad.org/public/diseases/a-z/pemphigus-self-care
If you are unsure how to take care of your blisters and sores, ask your dermatologist. […] Pemphigus can be a serious disease that changes your life. The following can help you manage life with pemphigus. […] Take precautions to avoid infections. Blisters and sores leave you more likely to develop an infection. […] Take care of mouth sores. If pemphigus causes sores in your mouth, you can ease the pain by: Eating bland, soft foods. […] Tell your dermatologist about eye problems. Blisters can form on the tissue lining an eyelid. […] Avoid sun and heat if you have pemphigus foliaceus or drug-induced pemphigus. […] Partner with your dermatologist. Your dermatologist can be your strongest ally. Dermatologists help patients with pemphigus find treatment that works.
- #59 Pemphigus Vulgaris: A Complete Overview â DermNethttps://dermnetnz.org/topics/pemphigus-vulgaris
Patients should minimise activities that may traumatise the skin and mucous membranes during active phases of the disease. These include activities such as contact sports and eating or drinking food that may irritate or damage the inside of the mouth (spicy, acidic, hard and crunchy foods). Oral hygiene and proper dental care are essential. Use a soft toothbrush and mint-free toothpaste to gently and thoroughly brush teeth twice daily. Rinse using an antiseptic or anti-inflammatory mouthwash. Treat oral candidiasis, if present.
- #60 Pemphigus Vulgaris: Symptoms, Causes & Treatmenthttps://my.clevelandclinic.org/health/diseases/23033-pemphigus-vulgaris
Pemphigus vulgaris is an autoimmune condition that causes blisters to form on your skin and mucous membranes. […] Treatment for pemphigus vulgaris focuses on managing symptoms and preventing complications like infections. Treatment is unique to each person and could include: […] Caring for sores and broken blisters as you would a burn or wound. […] Eating a bland diet or receiving nutrients through an IV to avoid malnutrition if sores in your mouth prevent you from eating. […] You can take care of yourself and manage your symptoms of pemphigus vulgaris at home by: […] Cleaning and caring for your blisters and sores like wounds or burns. […] Visit your healthcare provider if you have: […] Trouble eating and drinking. […] Talk to your healthcare provider about treatment options unique to your symptoms.
- #61 Pemphigus: 10 tips for managinghttps://www.aad.org/public/diseases/a-z/pemphigus-self-care
If you are unsure how to take care of your blisters and sores, ask your dermatologist. […] Pemphigus can be a serious disease that changes your life. The following can help you manage life with pemphigus. […] Take precautions to avoid infections. Blisters and sores leave you more likely to develop an infection. […] Take care of mouth sores. If pemphigus causes sores in your mouth, you can ease the pain by: Eating bland, soft foods. […] Tell your dermatologist about eye problems. Blisters can form on the tissue lining an eyelid. […] Avoid sun and heat if you have pemphigus foliaceus or drug-induced pemphigus. […] Partner with your dermatologist. Your dermatologist can be your strongest ally. Dermatologists help patients with pemphigus find treatment that works.
- #62 Pemphigus Vulgaris: Practice Essentials, Pathophysiology, Etiologyhttps://emedicine.medscape.com/article/1064187-overview
The aim of pharmacologic therapy for pemphigus vulgaris is to reduce inflammatory response and autoantibody production. Medications used in the diseases treatment include the following: […] Corticosteroids – Discourage the inflammatory process by inhibiting specific cytokine production […] Immunosuppressants – Should be considered early in the course of disease as steroid-sparing agents […] Rituximab […] Intravenous immune globulin (IVIG) […] Patients should be advised to minimize skin trauma because their skin will be more fragile than usual as a result of both the disease itself and the use of topical and systemic steroids to treat it. […] They should also be educated regarding their medications. If patients are informed about dosages, adverse effects, and symptoms of toxicity, they will be better able to report any adverse effects to the physician. […] Finally, instructions regarding appropriate wound care should be provided.
- #63 Management of chronic wounds in patients with pemphigus | CWCMRhttps://www.dovepress.com/management-of-chronic-wounds-in-patients-with-pemphigus-peer-reviewed-fulltext-article-CWCMR
When the disease becomes severe with extensive skin involvement, patients are typically admitted to the burn unit. Therefore, supportive care resembles that performed for severe thermal burns TEN. It aims at minimizing potential complications, which may ultimately lead to patient mortality. For instance, it aims to avoid hypovolemia, electrolyte imbalance, renal insufficiency, and sepsis. […] Nutrition is a crucial aspect of care, due to the loss of protein and other essential components in the serous sanguineous drainage. Patients should avoid excessive skin manipulation and minimize activities that may traumatize the skin and mucous membranes during active phases of the disease. These include contact sports and eating or drinking food that may irritate the oral cavity (spicy, acidic, hard and crunchy foods).
- #64 Pemphigus Vulgarishttps://encyclopedia.nm.org/Library/DiseasesConditions/Pediatric/PedArthritis/134,110
Call your healthcare provider if you notice painful, soft blistering on your skin or mucous membranes. Treatment will prevent the blisters from spreading, becoming infected, and getting worse. […] Some people find that stress and eating certain spicy, acidic, or citrus foods make pemphigus vulgaris worse. This may be true even during treatment. Pay attention to what helps you feel better and what makes symptoms worse.
- #65 Pemphigus Vulgaris | Cedars-Sinaihttps://www.cedars-sinai.org/health-library/diseases-and-conditions/p/pemphigus-vulgaris.html
Pemphigus vulgaris is a rare group of autoimmune diseases. It causes blisters on the skin and mucous membranes in the body. […] Good oral health care. Blistering may affect the health of your mouth. So working with your dentist to take the best care of your teeth and gums is important. […] Pain management. Pain medicine or other pain management strategies may be used to help with painful blisters. […] You may be advised to use baths and special wound dressings to help sores heal. If you get an infection, your provider may prescribe antibiotic, antifungal, or antiviral medicines. […] Contact your provider if you notice painful, soft blistering on your skin or mucous membranes. Treatment will prevent the blisters from spreading, becoming infected, and getting worse. […] Some people find that stress and eating certain spicy, acidic, or citrus foods make pemphigus vulgaris worse. This may be true even during treatment. Pay attention to what helps you feel better and what makes symptoms worse. […] You may need to see a dermatologist to diagnose and treat this condition.
- #66 Phemphigus | PPThttps://www.slideshare.net/sivanandareddy52/phemphigus
The patient with painful and extensive lesions should be pre-medicated with analgesics before skin care is initiated. […] The patient is encouraged to express freely anxieties, discomfort, and feelings of hopelessness. […] The skin is cleaned to remove debris and dead skin and to prevent infection. […] Antimicrobial agents are administered as prescribed, and response to treatment is assessed. […] The patient is encouraged to maintain adequate oral fluid intake.
- #67 Azthena logo with the word Azthenahttps://www.news-medical.net/health/Treatment-and-Self-Help-Tips-for-Pemphigus-Vulgaris.aspx
Pemphigus vulgaris is a rare autoimmune disease that causes blistering of the skin and mucous membranes of the mouth, throat and genital area. […] There is no cure for pemphigus vulgaris, but symptoms can be kept under control with the use of corticosteroids or immunosuppressants. […] In most cases, pemphigus vulgaris is initially treated with corticosteroids for a few weeks or months to suppress the autoimmune reaction. […] Once symptoms are under control, another immunosuppressant may be used in combination with the low-dose corticosteroid. […] It may be very distressing for people to find out that they have a rare and serious disease. […] Other recommendations that may be helpful to manage the condition include the following: Avoiding activities that could cause the skin to become damaged, such as contact sports, using a soft toothbrush, avoiding crisp, hard, hot or spicy foods, taking painkillers, particularly prior to eating or brushing the teeth, maintaining good oral and skin hygiene, talcum powder used liberally to prevent the skin sticking to the bedsheets, frequently changing and laundering towels, sheets and body linen in hot water to prevent secondary infection, seeking medical attention as soon as any symptoms of infection develop, using exercise and relaxation techniques as ways of improving coping mechanisms, attending a pain management course.
- #68 Managing pemphigus in primary carehttps://www.healthcert.com/blog/how-to-manage-pemphigus
Where there is a need to reduce the need for long-term corticosteroid application, steroid-sparing agents such as azathioprine, mycophenolate mofetil, or methotrexate are commonly used. […] Continuous monitoring of disease activity and treatment side effects is critical in pemphigus management. Regular follow-up visits are necessary to adjust medication dosages and ensure optimal care. […] Long-term management focuses on maintaining remission. Once the disease is under control, a gradual tapering of corticosteroids and immunosuppressive therapy is advised. Regular follow-up is necessary to detect early signs of relapse. If relapse occurs, treatment should be escalated to regain control of the disease.
- #69 Pemphigus: Types, Symptoms, Causes & Treatmenthttps://my.clevelandclinic.org/health/diseases/21130-pemphigus
Your healthcare provider will monitor your condition regularly and order blood and/or urine tests to verify that your treatment is working. Tests also check for negative reactions to medications that treat pemphigus. […] If you have trouble eating, contact your healthcare provider. They may recommend taking nutritional supplements to avoid malnutrition. […] The majority of people diagnosed with pemphigus have a normal lifespan. Treatment helps manage symptoms and could be ongoing throughout your life.
- #70 Managing pemphigus in primary carehttps://www.healthcert.com/blog/how-to-manage-pemphigus
Where there is a need to reduce the need for long-term corticosteroid application, steroid-sparing agents such as azathioprine, mycophenolate mofetil, or methotrexate are commonly used. […] Continuous monitoring of disease activity and treatment side effects is critical in pemphigus management. Regular follow-up visits are necessary to adjust medication dosages and ensure optimal care. […] Long-term management focuses on maintaining remission. Once the disease is under control, a gradual tapering of corticosteroids and immunosuppressive therapy is advised. Regular follow-up is necessary to detect early signs of relapse. If relapse occurs, treatment should be escalated to regain control of the disease.
- #71 Pemphigus: Types, Symptoms, Causes & Treatmenthttps://my.clevelandclinic.org/health/diseases/21130-pemphigus
Your healthcare provider will monitor your condition regularly and order blood and/or urine tests to verify that your treatment is working. Tests also check for negative reactions to medications that treat pemphigus. […] If you have trouble eating, contact your healthcare provider. They may recommend taking nutritional supplements to avoid malnutrition. […] The majority of people diagnosed with pemphigus have a normal lifespan. Treatment helps manage symptoms and could be ongoing throughout your life.
- #72 Pemphigus: Types, Symptoms, Causes & Treatmenthttps://my.clevelandclinic.org/health/diseases/21130-pemphigus
Your healthcare provider will monitor your condition regularly and order blood and/or urine tests to verify that your treatment is working. Tests also check for negative reactions to medications that treat pemphigus. […] If you have trouble eating, contact your healthcare provider. They may recommend taking nutritional supplements to avoid malnutrition. […] The majority of people diagnosed with pemphigus have a normal lifespan. Treatment helps manage symptoms and could be ongoing throughout your life.
- #73 Pemphigus Vulgaris Treatment & Management: Medical Care, Complications, Diethttps://emedicine.medscape.com/article/1064187-treatment
The aims of treatment are the same in pemphigus vulgaris as in other autoimmune bullous diseasesnamely, to decrease blister formation, to promote healing of blisters and erosions, and to determine the minimal dose of medication necessary to control the disease process. Therapy must be tailored for each patient, taking into account preexisting and coexisting conditions. Patients may continue to experience mild disease activity while under optimal treatment. […] Wound care for erosions includes daily gentle cleaning, application of topical agents to promote wound healing, and use of nonadhesive dressings. The goal of wound care is to promote healing, minimize trauma to the surrounding skin, and diminish scarring. Epidermal growth factor (EGF) may speed healing of localized lesions. […] Management of patients with pemphigus vulgaris requires coordination of care between the dermatologist and the patient’s primary care physician. […] Patients receiving long-term systemic corticosteroid therapy should be evaluated by a rheumatologist within the first 30 days of treatment for osteoporosis risk assessment and consideration of a bisphosphonate for prophylaxis against osteoporosis.
- #74 Pemphigus Vulgaris | Cedars-Sinaihttps://www.cedars-sinai.org/health-library/diseases-and-conditions/p/pemphigus-vulgaris.html
Pemphigus vulgaris is a rare group of autoimmune diseases. It causes blisters on the skin and mucous membranes in the body. […] Good oral health care. Blistering may affect the health of your mouth. So working with your dentist to take the best care of your teeth and gums is important. […] Pain management. Pain medicine or other pain management strategies may be used to help with painful blisters. […] You may be advised to use baths and special wound dressings to help sores heal. If you get an infection, your provider may prescribe antibiotic, antifungal, or antiviral medicines. […] Contact your provider if you notice painful, soft blistering on your skin or mucous membranes. Treatment will prevent the blisters from spreading, becoming infected, and getting worse. […] Some people find that stress and eating certain spicy, acidic, or citrus foods make pemphigus vulgaris worse. This may be true even during treatment. Pay attention to what helps you feel better and what makes symptoms worse. […] You may need to see a dermatologist to diagnose and treat this condition.
- #75 Oral pemphigus vulgaris | Registered Dental Hygienistshttps://www.rdhmag.com/patient-care/rinses-pastes/article/16407116/oral-pemphigus-vulgaris
The patient that you are treating in your practice today is a male, age 47, whose name is Mr. Wallace. He has been diagnosed with pemphigus vulgaris during the past six months. […] Early treatment is crucial in controlling pemphigus vulgaris. […] The dental team may be the first health-care members to notice the oral lesions that occur in pemphigus vulgaris, and they are a crucial link, assisting the patient in obtaining the necessary medical care that is needed. […] Maintenance appointments with the hygienist need to be conducted in a gentle fashion with multiple appointments for debridement in severe cases. […] As with other mucosal disease states such as MMP and OLP, gentle tissue manipulation is the best course of treatment for the patient. […] Additionally, more frequent maintenance appointments for debridement are recommended with meticulous plaque control.
- #76 Pemphigus Vulgaris Treatment & Management: Medical Care, Complications, Diethttps://emedicine.medscape.com/article/1064187-treatment
The aims of treatment are the same in pemphigus vulgaris as in other autoimmune bullous diseasesnamely, to decrease blister formation, to promote healing of blisters and erosions, and to determine the minimal dose of medication necessary to control the disease process. Therapy must be tailored for each patient, taking into account preexisting and coexisting conditions. Patients may continue to experience mild disease activity while under optimal treatment. […] Wound care for erosions includes daily gentle cleaning, application of topical agents to promote wound healing, and use of nonadhesive dressings. The goal of wound care is to promote healing, minimize trauma to the surrounding skin, and diminish scarring. Epidermal growth factor (EGF) may speed healing of localized lesions. […] Management of patients with pemphigus vulgaris requires coordination of care between the dermatologist and the patient’s primary care physician. […] Patients receiving long-term systemic corticosteroid therapy should be evaluated by a rheumatologist within the first 30 days of treatment for osteoporosis risk assessment and consideration of a bisphosphonate for prophylaxis against osteoporosis.
- #77 Pemphigus vulgarishttps://www.nhs.uk/conditions/pemphigus-vulgaris/
If you have pemphigus vulgaris, there are some things you can do to help ease the symptoms and reduce the risk of further problems. […] clean your teeth using a soft toothbrush and mint-free toothpaste […] use antiseptic or painkilling (anaesthetic) mouthwash (try to avoid mouthwashes that contain alcohol) […] keep any sore patches clean to reduce the risk of infection […] have regular dental check-ups. […] do not eat spicy, acidic or hard foods if you have blisters in your mouth […] do not do activities that could damage your skin (such as contact sports) if you have blisters on your skin. […] You’ll have regular check-ups to look for these problems and may be referred to other specialists (such as a dietitian) if needed.
- #78 Pemphigus and Pemphigoid > Fact Sheets > Yale Medicinehttps://www.yalemedicine.org/conditions/pemphigus-and-pemphigoid
The standard initial treatment is corticosteroids, which are administered topically and orally. […] You dont want to be on high doses of corticosteroids in the pill form for a long time because of the side effects, says Dr. Tomayko. So we also use other medications, injections and infusions that suppress the bodys immune response and deplete the antibodies. Mycophenolate mofetil and rituximab are two common ones. […] Wound care is highly specialized when it comes to these disorders. Our nurses and attending physicians are familiar with the most comfortable and effective dressings and procedures. […] Patients are not cured, per se, but the symptoms can be controlled, skin will heal if cared for properly, and, over the long term, there is a good likelihood that the disorders will go away.
- #79 Oral pemphigus vulgaris | Registered Dental Hygienistshttps://www.rdhmag.com/patient-care/rinses-pastes/article/16407116/oral-pemphigus-vulgaris
The individual patient and the extent of the ulceration are always considerations. […] Both medical and dental teams are needed for treatment of the patient with oral pemphigus vulgaris. […] The hygienist should be alerted to the typical oral implications and identification of candida in patients with pemphigus vulgaris.
- #80 Pemphigus vulgaris – approach and managementhttps://pmc.ncbi.nlm.nih.gov/articles/PMC6895778/
The gold standard for the diagnosis of PV is the detection of autoantibodies or complement component 3 by direct immunofluorescence microscopy of a perilesional biopsy. […] Early diagnosis and initiation of treatment are compulsory for a promising prognosis, and a multidisciplinary approach is recommended to optimize patient care.
- #81 Pemphigus vulgaris: MedlinePlus Medical EncyclopediaLockhttps://medlineplus.gov/ency/article/000882.htm
Severe cases of PV may need wound management, similar to the treatment for severe burns. People with PV may need to stay in a hospital and receive care in a burn unit or intensive care unit. […] Treatment is aimed at reducing symptoms, including pain. It also aims to prevent complications, especially infections. […] Treatment may involve: Antibiotics and antifungal medicines to control or prevent infections, Fluids and electrolytes given through a vein (IV) if there are severe mouth ulcers, IV feedings if there are severe mouth ulcers, Numbing (anesthetic) mouth lozenges to reduce mouth ulcer pain, Pain medicines if local pain relief is not enough. […] Body-wide (systemic) therapy is needed to control PV and should be started as early as possible. Systemic treatment includes: An anti-inflammatory medicine called dapsone, Corticosteroids, Medicines containing gold, Medicines that suppress the immune system (such as azathioprine, methotrexate, cyclosporine, cyclophosphamide, mycophenolate mofetil, or rituximab).
- #82 Pemphigus vulgaris Information | Mount Sinai – New Yorkhttps://www.mountsinai.org/health-library/diseases-conditions/pemphigus-vulgaris
Severe cases of PV may need wound management, similar to the treatment for severe burns. People with PV may need to stay in a hospital and receive care in a burn unit or intensive care unit. […] Treatment is aimed at reducing symptoms, including pain. It also aims to prevent complications, especially infections. […] Treatment may involve: Antibiotics and antifungal medicines to control or prevent infections, Fluids and electrolytes given through a vein (IV) if there are severe mouth ulcers, IV feedings if there are severe mouth ulcers, Numbing (anesthetic) mouth lozenges to reduce mouth ulcer pain, Pain medicines if local pain relief is not enough. […] Body-wide (systemic) therapy is needed to control PV and should be started as early as possible. […] Antibiotics may be used to treat or prevent infection. Intravenous immunoglobulin (IVIg) is occasionally used. […] Ulcer and blister treatments include soothing or drying lotions, wet dressings, or similar measures.
- #83 Oral pemphigus vulgaris | Registered Dental Hygienistshttps://www.rdhmag.com/patient-care/rinses-pastes/article/16407116/oral-pemphigus-vulgaris
The patient that you are treating in your practice today is a male, age 47, whose name is Mr. Wallace. He has been diagnosed with pemphigus vulgaris during the past six months. […] Early treatment is crucial in controlling pemphigus vulgaris. […] The dental team may be the first health-care members to notice the oral lesions that occur in pemphigus vulgaris, and they are a crucial link, assisting the patient in obtaining the necessary medical care that is needed. […] Maintenance appointments with the hygienist need to be conducted in a gentle fashion with multiple appointments for debridement in severe cases. […] As with other mucosal disease states such as MMP and OLP, gentle tissue manipulation is the best course of treatment for the patient. […] Additionally, more frequent maintenance appointments for debridement are recommended with meticulous plaque control.
- #84 Pemphigus Vulgaris Treatment & Management: Medical Care, Complications, Diethttps://emedicine.medscape.com/article/1064187-treatment
The aims of treatment are the same in pemphigus vulgaris as in other autoimmune bullous diseasesnamely, to decrease blister formation, to promote healing of blisters and erosions, and to determine the minimal dose of medication necessary to control the disease process. Therapy must be tailored for each patient, taking into account preexisting and coexisting conditions. Patients may continue to experience mild disease activity while under optimal treatment. […] Wound care for erosions includes daily gentle cleaning, application of topical agents to promote wound healing, and use of nonadhesive dressings. The goal of wound care is to promote healing, minimize trauma to the surrounding skin, and diminish scarring. Epidermal growth factor (EGF) may speed healing of localized lesions. […] Management of patients with pemphigus vulgaris requires coordination of care between the dermatologist and the patient’s primary care physician. […] Patients receiving long-term systemic corticosteroid therapy should be evaluated by a rheumatologist within the first 30 days of treatment for osteoporosis risk assessment and consideration of a bisphosphonate for prophylaxis against osteoporosis.
- #85 Pemphigus Vulgaris: A Complete Overview â DermNethttps://dermnetnz.org/topics/pemphigus-vulgaris
Appropriate wound care is particularly important, as this should promote healing of blisters and erosions. Handle skin very gently to avoid causing new blisters and erosions. Wear surgical gloves and use aseptic technique when changing dressings. Analgesics may be needed especially for dressing changes. Gently cleanse with an antiseptic solution or take a bleach bath. Drain intact blisters, but leave the blister roof in place. Apply a bland emollient ointment, such as 50% white soft paraffin + 50% liquid paraffin, directly to the skin, or apply the ointment to a dressing. Use non-adherent dressings (such as petrolatum-soaked gauze or silicone mesh). These may incorporate an antiseptic. An absorbent dressing may be applied over the primary dressing if the erosions are oozing. Be vigilant in identifying and treating any infection.
- #86 Pemphigus Vulgaris: Practice Essentials, Pathophysiology, Etiologyhttps://emedicine.medscape.com/article/1064187-overview
The aim of pharmacologic therapy for pemphigus vulgaris is to reduce inflammatory response and autoantibody production. Medications used in the diseases treatment include the following: […] Corticosteroids – Discourage the inflammatory process by inhibiting specific cytokine production […] Immunosuppressants – Should be considered early in the course of disease as steroid-sparing agents […] Rituximab […] Intravenous immune globulin (IVIG) […] Patients should be advised to minimize skin trauma because their skin will be more fragile than usual as a result of both the disease itself and the use of topical and systemic steroids to treat it. […] They should also be educated regarding their medications. If patients are informed about dosages, adverse effects, and symptoms of toxicity, they will be better able to report any adverse effects to the physician. […] Finally, instructions regarding appropriate wound care should be provided.
- #87 Managing pemphigus in primary carehttps://www.healthcert.com/blog/how-to-manage-pemphigus
Where there is a need to reduce the need for long-term corticosteroid application, steroid-sparing agents such as azathioprine, mycophenolate mofetil, or methotrexate are commonly used. […] Continuous monitoring of disease activity and treatment side effects is critical in pemphigus management. Regular follow-up visits are necessary to adjust medication dosages and ensure optimal care. […] Long-term management focuses on maintaining remission. Once the disease is under control, a gradual tapering of corticosteroids and immunosuppressive therapy is advised. Regular follow-up is necessary to detect early signs of relapse. If relapse occurs, treatment should be escalated to regain control of the disease.
- #88 Pemphigus Vulgaris Treatment & Management: Medical Care, Complications, Diethttps://emedicine.medscape.com/article/1064187-treatment
The aims of treatment are the same in pemphigus vulgaris as in other autoimmune bullous diseasesnamely, to decrease blister formation, to promote healing of blisters and erosions, and to determine the minimal dose of medication necessary to control the disease process. Therapy must be tailored for each patient, taking into account preexisting and coexisting conditions. Patients may continue to experience mild disease activity while under optimal treatment. […] Wound care for erosions includes daily gentle cleaning, application of topical agents to promote wound healing, and use of nonadhesive dressings. The goal of wound care is to promote healing, minimize trauma to the surrounding skin, and diminish scarring. Epidermal growth factor (EGF) may speed healing of localized lesions. […] Management of patients with pemphigus vulgaris requires coordination of care between the dermatologist and the patient’s primary care physician. […] Patients receiving long-term systemic corticosteroid therapy should be evaluated by a rheumatologist within the first 30 days of treatment for osteoporosis risk assessment and consideration of a bisphosphonate for prophylaxis against osteoporosis.
- #89 Managing pemphigus in primary carehttps://www.healthcert.com/blog/how-to-manage-pemphigus
Pemphigus is a complex condition that requires careful management. Primary care doctors, with adequate training in general dermatology, can play a vital role in early recognition, diagnosis, and prompt treatment of pemphigus, thereby improving the quality of life of their patients. […] Pemphigus vulgaris is a more severe form. The condition can lead to significant morbidity, so effective management is vital. […] Rapid initiation of treatment is essential to control the disease and prevent complications, such as secondary infections or fluid loss. The primary goals are to: Control blister formation, Reduce inflammation, and Minimize the need for long-term immunosuppression. […] High-dose systemic corticosteroids (prednisone) are the first-line treatment for pemphigus. Dosing typically starts at 1-2 mg/kg/day, adjusted based on the patients response and side effects.
- #90 Pemphigus Vulgaris in a Black Patient: Early Recognition of Disease Saves Liveshttps://www.dermatoljournal.com/articles/pemphigus-vulgaris-in-a-black-patient-early-recognition-of-disease-saves-lives.html
Pemphigus vulgaris (PV) is a rare autoimmune disease (IgG and C3) that attacks transmembrane glycoprotein of the Cadherin family called Desmogleins 1 and 3. […] PV has a high morbidity and mortality, and if left untreated disease will progress steadily with an average time of death of 14 months. Therefore, early diagnosis is imperative. […] The lack of images and evidence within literature concerning Pemphigus Vulgaris (PV) in patients with dark skin tone leads to a delay in diagnosis, thus having a negative impact on the patients management and overall prognosis. […] This case aims to create awareness among Internists, Hospitalists and Primary care physicians (PCP), helping them recognize PV in dark skin patients, which is essential to improve outcomes. […] As for Pemphigus vulgaris, suspect it when a patient complains of a non-pruritic poor healing ulcer that started as a flaccid blister that may or may not be over an erythematous base. During the physical exam, it is imperative to verify and assess for palatine or buccal mucosa involvement. Be aware that black skin tone patients are more prone to Postinflammatory Hyperpigmentation (PIH), hence changing the classic appearance of PV.
- #91 Pemphigus Vulgaris in a Black Patient: Early Recognition of Disease Saves Liveshttps://www.dermatoljournal.com/articles/pemphigus-vulgaris-in-a-black-patient-early-recognition-of-disease-saves-lives.html
This case report serves as an educational resource by providing images of a rare skin disease in people with dark skin with the purpose of filing major gaps in medical literature, highlighting the importance of timely recognizing PV in patients with dark skin, and creating awareness among physicians.