Pemfigus
Charakterystyka, pielęgnacja i opieka

Pemfigus to grupa rzadkich autoimmunologicznych chorób pęcherzowych, charakteryzujących się produkcją autoprzeciwciał przeciwko białkom skóry, co prowadzi do powstawania pęcherzy i nadżerek na skórze oraz błonach śluzowych, zwłaszcza w jamie ustnej, gdzie u 80% pacjentów pojawiają się pierwsze objawy. Pemfigus vulgaris stanowi około 70% przypadków i wymaga wczesnego rozpoznania oraz długoterminowego leczenia systemowego, obejmującego wysokie dawki kortykosteroidów (prednizon, prednisolon), leki immunosupresyjne (azatiopryna, mykofenolan mofetylu, cyklofosfamid) oraz rytuksymab jako lek pierwszego rzutu. Leczenie przebiega w trzech fazach: kontroli, konsolidacji i podtrzymania, a terapia miejscowa obejmuje stosowanie steroidów, środków antyseptycznych i nieprzylepnych opatrunków, które zapewniają wilgotne środowisko rany i chronią przed infekcjami bakteryjnymi (np. MRSA, Pseudomonas) i wirusowymi (HSV). Kluczowa jest także pielęgnacja jamy ustnej z użyciem bardzo miękkich szczoteczek, łagodnych past i płukanek bez alkoholu, a także odpowiednie postępowanie przeciwbólowe, w tym miejscowe znieczulenia lidokainą i systemowe leki przeciwbólowe.

Pemfigus – wprowadzenie

Pemfigus (pemphigus) to grupa rzadkich autoimmunologicznych chorób pęcherzowych, które powodują powstawanie pęcherzy i nadżerek na skórze oraz błonach śluzowych, najczęściej w jamie ustnej. Charakteryzuje się produkcją autoprzeciwciał, które atakują białka skóry, prowadząc do powstawania pęcherzy i nadżerek wymagających specjalistycznej opieki pielęgniarskiej.12 Pemfigus zwykły (pemphigus vulgaris) jest najczęstszym podtypem pemfigusu, stanowiącym około 70% wszystkich przypadków na świecie.3

Wczesne rozpoznanie pemfigusu jest kluczowe, ponieważ wcześnie rozpoczęte leczenie jest bardziej skuteczne. Choroba ta jest przewlekła i wymaga długoterminowego leczenia, jednak przy odpowiednim postępowaniu medycznym i pielęgnacyjnym można skutecznie kontrolować jej objawy.45

Cele opieki pielęgniarskiej w pemfigusie

Główne cele opieki pielęgniarskiej w pemfigusie obejmują:67

  • Zmniejszenie lub zapobieganie powstawaniu nowych pęcherzy
  • Zapobieganie infekcjom
  • Wspieranie gojenia się pęcherzy i nadżerek
  • Zapewnienie właściwego środowiska dla gojących się ran
  • Ochrona ran przed urazami mechanicznymi
  • Minimalizowanie powstawania blizn

89

Pielęgnacja ran w pemfigusie

Pielęgnacja ran w pemfigusie jest szczególnie istotna ze względu na charakter zmian skórnych. Pacjenci z pemfigusem mają otwarte rany z tendencją do powstawania nowych pęcherzy, co stanowi unikalny problem w kontekście opieki nad ranami. Ponadto mają delikatną skórę, co ogranicza stosowanie materiałów przylepnych.10

Zalecenia dotyczące pielęgnacji ran w pemfigusie obejmują:1112

  • Codzienne delikatne oczyszczanie ran
  • Stosowanie środków miejscowych wspierających gojenie ran
  • Używanie nieprzylepnych opatrunków
  • Minimalizowanie urazów okolicznej skóry
  • Ograniczanie powstawania blizn

13

W przypadku dużych pęcherzy należy je nakłuć sterylną igłą, zachowując dach pęcherza na miejscu. Obszary pozbawione naskórka wymagają oczyszczenia za pomocą antyseptyków lub soli fizjologicznej, a następnie pokrycia nieprzylepnym opatrunkiem. Należy unikać nadmiernej manipulacji skórą i urazów w aktywnym pemfigusie.14

Idealny materiał opatrunkowy powinien zapewniać wilgotne środowisko dla rany, jednocześnie chroniąc ją przed inwazją bakteryjną. W przypadku pacjentów z pemfigusem, gdzie rany są powierzchowne, odpowiednim wyborem są opatrunki w postaci filmów, hydrożeli i materiałów nieprzylepnych.15

Pielęgnacja zmian w jamie ustnej

U 80% pacjentów z pemfigus vulgaris pierwsze objawy choroby pojawiają się w jamie ustnej.16 Pielęgnacja jamy ustnej jest niezwykle ważna dla pozytywnych wyników leczenia. Zła higiena jamy ustnej z płytką nazębną i zapaleniem dziąseł może zmniejszyć i opóźnić efekty leczenia.17

Zalecenia dotyczące higieny jamy ustnej obejmują:1819

  • Stosowanie bardzo miękkich szczoteczek do zębów
  • Używanie past do zębów o łagodnym smaku i bez mięty
  • Stosowanie łagodnych płukanek do jamy ustnej
  • Unikanie płukanek zawierających alkohol
  • Regularne wizyty kontrolne u dentysty

20

Instrukcje dotyczące higieny jamy ustnej do stosowania w domu powinny być dostosowane do stopnia zajęcia błony śluzowej. Gdy obecne są znaczne zmiany w jamie ustnej, łagodna pielęgnacja, w tym szczoteczki o bardzo miękkim włosiu, pasty do zębów o łagodnym smaku i delikatne płukanki do jamy ustnej, mogą być wszystkim, co pacjent jest w stanie tolerować. Niektórzy pacjenci mogą nie być w stanie używać nici dentystycznej z powodu krwawienia i bólu. W tym czasie ważniejsze stają się częste profesjonalne czyszczenia zębów.21

Po zmniejszeniu się owrzodzeń jamy ustnej i związanego z nimi bólu można zalecić standardową rutynę pielęgnacji, w tym regularne szczotkowanie i używanie nici dentystycznej.22 Należy również zachęcać do stosowania płukanek antyseptycznych, takich jak glukonian chlorheksydyny 0,2% lub roztwory nadtlenku wodoru 1:4.23

Kompleksowe podejście do opieki w pemfigusie

Zarządzanie bólem

Ból jest powszechnym objawem w pemfigusie i wymaga odpowiedniego postępowania:2425

  • Stosowanie miejscowych środków znieczulających, takich jak lidokaina, na bolesne owrzodzenia jamy ustnej
  • Podawanie leków przeciwbólowych przed rozpoczęciem procedur pielęgnacyjnych skóry
  • Stosowanie pastylki znieczulające do jamy ustnej w celu zmniejszenia bólu owrzodzeń
  • W przypadku silnego bólu – stosowanie systemowych leków przeciwbólowych

26

Zapobieganie infekcjom

Pacjenci z rozległymi zmianami powinni być izolowani, aby zmniejszyć ryzyko zakażenia krzyżowego. Mogą wystąpić wtórne infekcje bakteryjne (MRSA, pseudomonas) lub wirusowe (HSV). Skuteczne jest stosowanie środków przeciwdrobnoustrojowych.27

Zalecenia obejmują:28

  • Stosowanie antybiotyków i leków przeciwgrzybiczych w celu kontroli lub zapobiegania infekcjom
  • Regularne monitorowanie pod kątem objawów infekcji
  • Utrzymywanie czystości skóry i jamy ustnej
  • Stosowanie miejscowych środków przeciwdrobnoustrojowych

29

Wsparcie żywieniowe

Odżywianie jest kluczowym aspektem opieki ze względu na utratę białka i innych podstawowych składników w wydzielinie surowiczo-krwistej:3031

  • W przypadku ciężkich owrzodzeń jamy ustnej może być konieczne podawanie płynów i elektrolitów dożylnie
  • W przypadku trudności z jedzeniem – podawanie pokarmu dożylnie
  • Zalecanie diety łagodnej lub otrzymywanie składników odżywczych przez dożylnie
  • Unikanie potraw pikantnych, kwaśnych lub twardych w przypadku pęcherzy w jamie ustnej
  • Małe, częste posiłki lub przekąski o wysokiej zawartości białka i kalorii pomagają utrzymać stan odżywienia

3233

Współpraca w zespole interdyscyplinarnym

Opieka nad pacjentem z pemfigusem wymaga współpracy wielu specjalistów:3435

  • Dermatolog – podstawowa diagnoza i leczenie
  • Dentysta – regularne kontrole i wsparcie w utrzymaniu higieny jamy ustnej
  • Dietetyk – opracowanie diety dostosowanej do potrzeb pacjenta
  • Okulista – ocena i leczenie zajęcia oczu
  • Pielęgniarka – kompleksowa opieka nad ranami i monitorowanie stanu pacjenta
  • Laryngolog – w przypadku zajęcia górnych dróg oddechowych
  • Gastroenterolog – w przypadku zajęcia przełyku

3637

Współpraca między specjalistami ułatwia dyskusję i opracowanie potencjalnych strategii postępowania z różnych punktów widzenia, unikając „silosów opieki” z ryzykiem, jakie niesie ze sobą sytuacja, gdy każdy specjalista koncentruje się na własnych priorytetach klinicznych bez wspólnego myślenia.38

Leczenie systemowe wspierające opiekę

Opieka pielęgniarska w pemfigusie ściśle wiąże się z leczeniem systemowym, które ma na celu kontrolę choroby i łagodzenie objawów:3940

  • Kortykosteroidy – leki pierwszego rzutu, zazwyczaj w postaci wysokich dawek prednizonu lub prednisolonu
  • Leki immunosupresyjneazatiopryna, mykofenolan mofetylu, cyklofosfamid – stosowane w celu zmniejszenia dawki steroidów
  • Rytuksymab – przeciwciało monoklonalne anty-CD20, coraz częściej stosowane jako lek pierwszego rzutu
  • Dożylne immunoglobuliny (IVIG) – stosowane w niektórych przypadkach
  • Antybiotyki – w przypadku infekcji lub profilaktycznie

4142

Leczenie pemfigusu jest zwykle prowadzone w trzech etapach:43

  1. Kontrola: Wysokie dawki leków kontrolują rozprzestrzenianie się pęcherzy i rozpoczynają gojenie istniejących
  2. Konsolidacja: Stałe dawki leków kontynuują gojenie pęcherzy, aż większość z nich ustąpi
  3. Podtrzymanie: Zmniejszone dawki leków zapobiegają powstawaniu nowych pęcherzy

44

Leczenie miejscowe

Oprócz leczenia systemowego, istotną rolę odgrywa leczenie miejscowe:4546

  • Miejscowe steroidy – w postaci żeli, maści lub płukanek do jamy ustnej
  • Środki antyseptyczne – do oczyszczania ran i zapobiegania infekcjom
  • Opatrunki na rany – pomagające w gojeniu pęcherzy i nadżerek
  • Kąpiele i okłady – łagodzące lub osuszające, w zależności od potrzeb

47

W przypadku zmian w jamie ustnej, leczenie miejscowe może obejmować:48

  • Płukanki do ust z deksametazonem („swish and spit”)
  • Miejscowe steroidy w żelu lub na bazie okluzyjnej, najlepiej stosowane przed snem
  • Płukanki z chlorheksydyną lub łagodnym środkiem odkażającym
  • Produkty zwane „magic mouth” pomagające przy bolesnych ranach w jamie ustnej

4950

Edukacja pacjenta i wsparcie psychospołeczne

Edukacja pacjenta jest kluczowym elementem opieki pielęgniarskiej w pemfigusie:5152

  • Instrukcje dotyczące pielęgnacji ran i stosowania leków miejscowych
  • Zalecenia dotyczące higieny jamy ustnej dostosowane do stanu pacjenta
  • Informacje o możliwych skutkach ubocznych leków i jak sobie z nimi radzić
  • Rozpoznawanie objawów infekcji i kiedy szukać pomocy medycznej
  • Strategie radzenia sobie z bólem i dyskomfortem

53

Pemfigus może być trudny do życia z nim, szczególnie jeśli wpływa na codzienne czynności lub powoduje utratę snu i stres. Wsparcie psychospołeczne obejmuje:5455

  • Zachęcanie do dołączenia do grup wsparcia (online lub osobiście)
  • Utrzymywanie domu (lub przynajmniej pokoju) w chłodzie i ciszy, wypełnionym różnymi zajęciami jak TV, filmy i książki
  • Regularna komunikacja z pracodawcą
  • Zwiększanie świadomości na temat choroby wśród rodziny i przyjaciół
  • Wsparcie psychologiczne w razie potrzeby

56

Zalecenia dotyczące stylu życia

Pacjenci z pemfigusem mogą podjąć pewne kroki w celu zarządzania swoimi objawami:5758

  • Dbanie o pęcherze zgodnie z zaleceniami lekarza
  • Unikanie przebywania na słońcu lub stosowanie kremów z filtrem przeciwsłonecznym
  • Stosowanie mydeł i balsamów przeznaczonych dla skóry wrażliwej, bez zapachów
  • Noszenie lekkiej, ciemnej odzieży sportowej z technologią chłodzącą
  • Unikanie potraw pikantnych, kwaśnych lub cytrusowych, które mogą nasilać objawy
  • Unikanie czynności, które mogą uszkodzić skórę (np. sporty kontaktowe)

5960

Szczególne sytuacje kliniczne

Ciężkie przypadki pemfigusu

W ciężkich przypadkach pemfigusu, z rozległym zajęciem skóry, pacjenci są zazwyczaj hospitalizowani w oddziale oparzeń.61 Opieka w takich przypadkach obejmuje:6263

  • Intensywna pielęgnacja ran, podobna do leczenia ciężkich oparzeń
  • Monitorowanie równowagi płynów i elektrolitów
  • Żywienie dożylne w przypadku ciężkich owrzodzeń jamy ustnej
  • Intensywna kontrola infekcji
  • Regularne monitorowanie ciśnienia krwi i poziomu glukozy we krwi

64

Pemfigus paraneoplastyczny

Pemfigus paraneoplastyczny to rzadka odmiana choroby związana z obecnością nowotworu. Odpowiedź na leczenie jest generalnie słaba, szczególnie w przypadku zmian śluzówkowych.65 Opieka obejmuje:6667

  • Leczenie podstawowego nowotworu, gdy jest to możliwe
  • Stosowanie ciepłych okładów, nieprzylepnych opatrunków i miejscowych maści antybiotykowych
  • Silne leki immunosupresyjne, choć często są nieskuteczne
  • Terapia respiracyjna w przypadku niewydolności oddechowej spowodowanej zajęciem płuc
  • Konsultacje specjalistyczne (pulmonologia, okulistyka, gastroenterologia, laryngologia)

68

Monitorowanie i długoterminowa opieka

Pemfigus wymaga regularnego monitorowania i długoterminowej opieki:6970

  • Regularne wizyty kontrolne w celu dostosowania dawek leków
  • Monitorowanie działań niepożądanych leków (osteoporoza, infekcje, nadciśnienie, cukrzyca)
  • Regularne badania krwi i moczu w celu weryfikacji skuteczności leczenia
  • Kontrola stanu odżywienia
  • Regularne wizyty u dentysty

7172

Pielęgniarka odgrywa ważną rolę w monitorowaniu długoterminowych skutków leczenia kortykosteroidami i lekami immunosupresyjnymi.73 Pacjenci powinni być obserwowani rutynowo, aż do zakończenia leczenia i pozostawania w remisji.74

Powikłania i ich zapobieganie

Możliwe powikłania pemfigusu obejmują:7576

  • Infekcje skóry i sepsa – które mogą być zagrażające życiu
  • Niedożywienie – spowodowane bolesnymi owrzodzeniami jamy ustnej utrudniającymi jedzenie
  • Skutki uboczne leków – nadciśnienie, infekcje, cukrzyca
  • Próchnica i choroby dziąseł – z powodu trudności w utrzymaniu higieny jamy ustnej
  • Ból podczas stosunków seksualnych i ból podczas oddawania moczu lub stolca (jeśli narządy płciowe lub odbyt są dotknięte)

7778

Zapobieganie tym powikłaniom obejmuje:7980

  • Właściwa pielęgnacja ran w celu zapobiegania infekcjom
  • Regularna higiena jamy ustnej
  • Monitorowanie stanu odżywienia i w razie potrzeby suplementacja
  • Regularne wizyty kontrolne
  • Profilaktyka osteoporozy u pacjentów długotrwale przyjmujących kortykosteroidy

81

Wnioski i znaczenie opieki pielęgniarskiej

Opieka pielęgniarska odgrywa kluczową rolę w zarządzaniu pemfigusem i poprawie jakości życia pacjentów.82 Pielęgniarki są zaangażowane w monitoring i interwencje pielęgniarskie, które są niezbędne do osiągnięcia satysfakcjonujących efektów leczniczych.83

Wdrożenie protokołu opieki pielęgniarskiej wykazało skuteczność w poprawie klinicznych wyników zdrowotnych pacjentów z pemfigusem.84 Zaleca się utworzenie jednostki edukacyjnej dla opieki pielęgniarskiej nad pacjentami z pemfigusem w oddziałach dermatologicznych szpitali oraz przeprowadzenie podobnych badań na większej próbie probabilistycznej w celu generalizacji wyników.85

Kompleksowe podejście do opieki pielęgniarskiej, obejmujące zarówno aspekty fizyczne, jak i psychospołeczne, jest kluczowe dla skutecznego zarządzania tą złożoną chorobą autoimmunologiczną i poprawy jakości życia pacjentów z pemfigusem.8687

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  1. 13.04.2026
  2. www.leksykon.com.pl

Materiały źródłowe

  • #1 Managing pemphigus in primary care
    https://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. […] The correct diagnosis is the first step in managing pemphigus. The presence of painful, flaccid blisters, erosions, or ulcers on the skin and/or mucosal surfaces (oral cavity, genitals) is the first obvious sign. However, confirmation typically requires laboratory testing. […] 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.
  • #2 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.
  • #3 Pemphigus vulgaris
    https://www.pcds.org.uk/clinical-guidance/pemphigus-vulgaris
    Pemphigus is derived from the Greek pemphix meaning blister or bubble. Pemphigus vulgaris is a rare immunobullous condition that is characterised by blisters and erosions on the skin and mucous membranes, most commonly the mouth. The blisters in both pemphigus vulgaris, and its even less common vegetating form, pemphigus vegetans, are suprabasal. Pemphigus vulgaris is the most common subtype of pemphigus, accounting for 70% of all pemphigus cases worldwide. […] 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. Patients should minimise activities that may traumatise the skin and mucous membranes during active phases.
  • #4 Pemphigus – Diagnosis and treatment – Mayo Clinic
    https://www.mayoclinic.org/diseases-conditions/pemphigus/diagnosis-treatment/drc-20350409
    Your healthcare professional may start by talking with you about your medical history and symptoms and examining the affected area. […] Treatment for pemphigus usually starts with medicines to ease symptoms and prevent new blisters. These may include steroids and medicines that target the immune system. […] Many people with pemphigus get better, especially if treatment is started early. But it may take years and can require taking medicine for a long time. […] Taking good care of your wounds can help prevent infection and scarring. Talk with your healthcare professional about how best to care for your wounds and control pain. […] Regular follow-up with a dentist is important for people with pemphigus. Talk with your dentist about how to best take care of your teeth and gums. […] Pemphigus may be difficult to live with, especially if it affects your daily activities or causes lost sleep or stress. You may find it helpful to talk to others with the disease. You may want to connect with a support group in person or online. Ask your healthcare professional for suggestions.
  • #5 Pemphigus: causes, symptoms, and treatment here – TeleMed2U
    https://www.telemed2u.com/dermatology/pemphigus
    Pemphigus is a group of rare diseases that cause blisters and sores throughout the body on mucous membranes and skin. […] This chronic (long term) condition has no cure but can be controlled with medication. […] Treatment usually begins with medications to stop more blisters from forming. […] The earlier treatment starts, the more effective it will be. […] Many people improve slowly with medications. […] Others may need to take medication for the rest of their life to keep symptoms from returning. […] Without treatment, pemphigus tends to worsen. […] Some types of pemphigus can become life-threatening without treatment. […] Lifestyle changes and home remedies should be included in treatment plans. […] Follow your doctors wound-care instructions to prevent infections and scars. […] Pemphigus can be very difficult to live with and you may benefit from a support group, and regular stress reduction techniques or activities.
  • #6 Management of chronic wounds in patients with pemphigus | CWCMR
    https://www.dovepress.com/management-of-chronic-wounds-in-patients-with-pemphigus-peer-reviewed-fulltext-article-CWCMR
    Pemphigus is a group of chronic IgG-mediated autoimmune blistering diseases that involves both the skin and mucous membranes. […] The primary goal of treatment is to induce durable remission, decrease blister formation, prevent infections and promote healing of blisters and erosions. […] 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. […] 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. […] When the disease becomes severe with extensive skin involvement, patients are typically admitted to the burn unit.
  • #7 Pemphigus Vulgaris Treatment & Management: Medical Care, Complications, Diet
    https://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. […] Treatment with corticosteroids has improved the overall mortality, but significant morbidity remains. Much of the mortality and morbidity in patients with pemphigus vulgaris is now related to the adverse effects of such therapy. […] Immunosuppressive drugs are steroid-sparing and should be considered early in the course of the disease.
  • #8 Pemphigus Vulgaris – UF Health
    https://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. […] 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.
  • #9 Wound Care in Immunobullous Disease | IntechOpen
    https://www.intechopen.com/chapters/58481
    When patients who have extensive raw areas are hospitalized, they must be isolated to reduce cross-infection. Secondary bacterial (MRSA, pseudomonas) or viral (HSV) infections can occur. The use of antimicrobials is effective. Additionally, it is important to routinely monitor blood pressure and blood glucose levels. […] In severe disease, the premise of care is very similar to patients being treated for partial thickness burn therapy. Frequently in pemphigus vulgaris and bullous pemphigoid, the body is covered with intact bullae and partial-thickness wounds from head to toe. […] In severe pemphigus and pemphigoid, various goals of wound care management have been identified. These include relieving severe pain, preventing infection and decreasing bioburden, enhancing regeneration of the dermis and the epidermis, protecting the periwound skin from maceration, encouraging patient mobility and quality of life, providing nutritional support for tissue repair, as well as treating the underlying cause of the wound. […] Ultimately, the wound care plan focuses on reducing pain, preventing infection by gentle cleansing and preventing scarring by providing a moist environment. Additionally, nutrition is a key aspect of care, due to the loss of protein and other essential components in the serous sanguineous drainage.
  • #10 Wound Care in Immunobullous Disease | IntechOpen
    https://www.intechopen.com/chapters/58481
    The chapter introduces the steps to achieving proper wound care in immunobullous disease. It describes the clinical characteristics and nature of wounds formed in pemphigus versus pemphigoid diseases. Namely, pemphigus diseases typically result in acantholysis in the epidermis and the formation of flaccid blisters. In contrast, bullous pemphigoid presents with basal keratinocyte hemidesmosomes in the dermoepidermal junction, which results in a split at the dermoepidermal junction and clinically forms tense blisters. Therefore, there is a separate protocol for treating the wounds in each of these diseases, which must take additional patient specific factors into consideration. […] Patients suffering from immunobullous disease have open wounds with a tendency to develop new blisters, which presents a unique problem when addressing wound care. These patients also have fragile skin limiting the use of adhesives. Immunosuppressant treatments increase infection risk and also prednisone slows wound healing but is needed to treat the disease. Therefore, there are many complexities surrounding proper wound care specific to these patients.
  • #11 Pemphigus Vulgaris Treatment & Management: Medical Care, Complications, Diet
    https://emedicine.medscape.com/article/1064187-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. […] 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.
  • #12 Self-Care: Comfort Techniques and Dressings – IPPF
    https://www.pemphigus.org/self-care-comfort-techniques-and-dressings/
    Pemphigus and pemphigoid (P/P) patients may agree that it can be very difficult and painful in the early months of their condition. […] The following suggestions may be helpful, such as learning how to care for our skin by taking and applying topical medications, cleansers, moisturizers, dressings, and staying comfortable. […] When applying or changing dressings, wash your hands with soap and water, and use gloves. […] My dermatologist had previously instructed me on the exact amount to use when applying topical steroid to avoid damaging the skin. […] Additionally, I made sure that the opening or mouth of the tube of medication didn’t touch my skin in order to avoid infection. […] Make sure you review exactly how to use these and any products when caring for your skin including how often to check and change your dressings with your health care provider.
  • #13 Self-Care: Comfort Techniques and Dressings – IPPF
    https://www.pemphigus.org/self-care-comfort-techniques-and-dressings/
    Pemphigus and pemphigoid (P/P) patients may agree that it can be very difficult and painful in the early months of their condition. […] The following suggestions may be helpful, such as learning how to care for our skin by taking and applying topical medications, cleansers, moisturizers, dressings, and staying comfortable. […] When applying or changing dressings, wash your hands with soap and water, and use gloves. […] My dermatologist had previously instructed me on the exact amount to use when applying topical steroid to avoid damaging the skin. […] Additionally, I made sure that the opening or mouth of the tube of medication didn’t touch my skin in order to avoid infection. […] Make sure you review exactly how to use these and any products when caring for your skin including how often to check and change your dressings with your health care provider.
  • #14 Wound Care in Immunobullous Disease | IntechOpen
    https://www.intechopen.com/chapters/58481
    The best approach to the care of blistered skin has not yet been definitively established. There is currently controversy on how to deal with small tense blisters. While some resources recommend daily rupturing of tense blisters for reducing lateral extension of the blister edges, other resources advocate for leaving blisters intact, to prevent secondary infection. However, large blisters should be aspirated with a sterile needle, to keep the blister roof in place. Raw areas need to be cleaned by antiseptics or normal saline and then covered by a non-adhesive dressing. Excessive skin manipulation and trauma should be avoided in active pemphigus vulgaris. […] The wound care in treating pemphigus vulgaris and bullous pemphigoid depends on the severity of the disease, the location of the lesions, as well as the total body surface area (TBSA) covered with lesions. While there have been several case studies published with different recommendations regarding wound care, each is individualized to the unique needs of the specific patient, and there is not yet one specific standard of care.
  • #15 Management of chronic wounds in patients with pemphigus | CWCMR
    https://www.dovepress.com/management-of-chronic-wounds-in-patients-with-pemphigus-peer-reviewed-fulltext-article-CWCMR
    Nutrition is a crucial aspect of care, due to the loss of protein and other essential components in the serous sanguineous drainage. […] Measures aimed at managing cutaneous wounds are additional important aspects of the management of pemphigus. […] The ideal dressing material should provide a moist environment to the wound while shielding it from bacterial invasion. […] In patients with pemphigus, wounds are superficial, and dressings such as films, hydrogels, and PMDs would be appropriate choices.
  • #16 Pemphigus (includes “pemphigus vulgaris” [“PV”], “pemphigus vegetans”, “pemphigus foliaceus”, “pemphigus erythematosus”, “paraneoplastic pemphigus”, and “drug-related pemphigus”)1 – CDHO
    https://cdho.org/factsheets/pemphigus/
    Severe oral erosions interfere with patients/clients proper eating and drinking, as well as hinder nutrient intake. Intravenous (IV) feeding may be needed in very severe cases. […] Oral prophylaxis should ideally be performed prior to the initiation of systemic or topical therapy. […] Routine oral hygiene (including brushing) is often compromised due to pain in the mouth. […] During the active oral disease stage, patient/client follow-up is recommended as frequently as every 4 to 6 weeks for debridement, and it should include monitoring for oral candidiasis. […] The dental hygienist should anticipate that the patient/client may experience pain and bleeding during procedures, and plan for this by scheduling extra time and using suction and gauze as needed. […] Simple hand scaling instruments are often most effective, particularly in patients/clients with severe mucosal disease. […] Disease flares following dental work are fairly common. […] The dental hygienist plays a role in monitoring the patient/client for long-term effects of corticosteroid and immunosuppressive treatment.
  • #17 Disease Management – IPPF
    https://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.
  • #18 Pemphigus vulgaris
    https://www.nhs.uk/conditions/pemphigus-vulgaris/
    If a GP thinks you could have pemphigus vulgaris, they can refer you to a skin specialist (dermatologist) for tests and treatment. […] Blisters caused by pemphigus vulgaris usually come and go over time. There’s no cure, but treatment can help. […] The main treatments are: steroid tablets, other medicines such as azathioprine, methotrexate and rituximab. […] It usually takes a few weeks for the blisters to heal. Once this happens, you’ll probably need treatment for several years to help stop the blisters coming back. […] 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.
  • #19 Pemphigus: 10 tips for managing
    https://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. […] Understand that it can take time to get pemphigus under control. Some patients need months or years, but pemphigus is nearly always controllable. […] Take care of mouth sores. If pemphigus causes sores in your mouth, you can ease the pain by: […] Keeping your mouth clean. Poor oral hygiene can worsen mouth sores. Your dermatologist can recommend a toothbrush, toothpaste, and mouthwash that are least likely to cause pain. […] Tell your dermatologist about eye problems. Blisters can form on the tissue lining an eyelid.
  • #20 Disease Management – IPPF
    https://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.
  • #21 Disease Management – IPPF
    https://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.
  • #22 Disease Management – IPPF
    https://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.
  • #23 Disease Management – IPPF
    https://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.
  • #24 pemphigus | Taber’s Medical Dictionary
    https://nursing.unboundmedicine.com/nursingcentral/view/Tabers-Dictionary/740558/all/pemphigus?q=ACS
    PATIENT CARE All of the therapeutic drugs may cause severe side effects, and the patient must be closely monitored. Pain is common in pemphigus and must be managed. If the patient’s mouth ulcers are painful, topical anesthetics such is lidocaine are helpful. If toothbrushing is painful or contraindicated the patient should be instructed in using a mouth rinse such as a normal saline solution or half-strength hydrogen peroxide and to avoid mouthwashes that contain alcohol. Oral lesions that do not heal within two weeks must be reported.
  • #25 Pemphigus Vulgaris – UF Health
    https://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. […] 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.
  • #26 Pemphigus vulgaris: MedlinePlus Medical EncyclopediaLock
    https://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. […] 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.
  • #27 Wound Care in Immunobullous Disease | IntechOpen
    https://www.intechopen.com/chapters/58481
    When patients who have extensive raw areas are hospitalized, they must be isolated to reduce cross-infection. Secondary bacterial (MRSA, pseudomonas) or viral (HSV) infections can occur. The use of antimicrobials is effective. Additionally, it is important to routinely monitor blood pressure and blood glucose levels. […] In severe disease, the premise of care is very similar to patients being treated for partial thickness burn therapy. Frequently in pemphigus vulgaris and bullous pemphigoid, the body is covered with intact bullae and partial-thickness wounds from head to toe. […] In severe pemphigus and pemphigoid, various goals of wound care management have been identified. These include relieving severe pain, preventing infection and decreasing bioburden, enhancing regeneration of the dermis and the epidermis, protecting the periwound skin from maceration, encouraging patient mobility and quality of life, providing nutritional support for tissue repair, as well as treating the underlying cause of the wound. […] Ultimately, the wound care plan focuses on reducing pain, preventing infection by gentle cleansing and preventing scarring by providing a moist environment. Additionally, nutrition is a key aspect of care, due to the loss of protein and other essential components in the serous sanguineous drainage.
  • #28 Pemphigus vulgaris: MedlinePlus Medical EncyclopediaLock
    https://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. […] 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.
  • #29 Pemphigus – Symptoms and causes – Mayo Clinic
    https://www.mayoclinic.org/diseases-conditions/pemphigus/symptoms-causes/syc-20350404
    Pemphigus is easier to control if caught and treated early. It’s usually treated with medicines that you take long term. The sores may heal slowly or not at all. The condition can become life-threatening if the sores become infected. […] See a healthcare professional if you have blisters that don’t heal in the mouth or on the skin or genital mucous membranes. […] Possible complications of pemphigus include: Infection of the skin. Infection that spreads to your bloodstream, also called sepsis. This type of infection can be life-threatening. Malnutrition, because painful mouth sores make it difficult to eat. Side effects from the medicine used to treat pemphigus. Examples are high blood pressure and infection.
  • #30 Management of chronic wounds in patients with pemphigus | CWCMR
    https://www.dovepress.com/management-of-chronic-wounds-in-patients-with-pemphigus-peer-reviewed-fulltext-article-CWCMR
    Nutrition is a crucial aspect of care, due to the loss of protein and other essential components in the serous sanguineous drainage. […] Measures aimed at managing cutaneous wounds are additional important aspects of the management of pemphigus. […] The ideal dressing material should provide a moist environment to the wound while shielding it from bacterial invasion. […] In patients with pemphigus, wounds are superficial, and dressings such as films, hydrogels, and PMDs would be appropriate choices.
  • #31 Pemphigus Vulgaris – UF Health
    https://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. […] 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.
  • #32 Phemphigus | PPT
    https://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. […] The oral cavity is inspected daily, and any changes are reported. […] Antimicrobial agents are administered as prescribed, and response to treatment is assessed. […] The patient is encouraged to maintain adequate oral fluid intake. […] Small, frequent meals or snacks of high-protein, high-calorie foods help maintain nutritional status.
  • #33 Pemphigus Vulgaris: Symptoms, Causes & Treatment
    https://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.
  • #34 The management of pemphigus vulgaris and mucous membrane pemphigoid in a joint oral medicine and dermatology clinic: a five-year narrative review | British Dental Journal
    https://www.nature.com/articles/s41415-024-7074-8
    Pemphigus disease and mucous membrane pemphigoid are autoimmune blistering diseases (AIBDs) which may involve both oral and extra-oral tissues. […] This interdisciplinary approach aimed to address the medical management challenges due to the multisite nature of these AIBDs. […] We reflect on the experiential benefits of this multidisciplinary clinic extended beyond immediate patient benefits to areas such as specialist training, both from a dermatologist’s and oral physician’s perspective. […] Reinforce the benefits of multidisciplinary teamworking for both patients and clinicians in the management of autoimmune blistering diseases. […] The care for patients with multisite AIBDs requires input from an oral physician, dermatologist, otolaryngologist and ophthalmologist to combine expertise in order to institute appropriate therapy and improve outcomes.
  • #35 Pemphigus Vulgaris Treatment & Management: Medical Care, Complications, Diet
    https://emedicine.medscape.com/article/1064187-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. […] 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.
  • #36 The management of pemphigus vulgaris and mucous membrane pemphigoid in a joint oral medicine and dermatology clinic: a five-year narrative review | British Dental Journal
    https://www.nature.com/articles/s41415-024-7074-8
    The BJOMDCC facilitates discussion between physicians, with exploration of potential management strategies from different viewpoints, thereby avoiding care silos’ with the risk that this brings when each specialist focusses on their own clinical priorities without joined-up thinking. […] Topical steroid therapy is considered first-line treatment for oral mucosal involvement in AIBDs. […] In such cases, a stepladder approach with the addition of systemic steroid therapy in combination with one or more immunomodulatory or immunosuppressant medications is recommended by current guidelines. […] Conventional systemic immunosuppression remains the mainstay approach within the BJOMDCC, reflecting the severity and multisite involvement in this cohort. […] Combination therapy with two or more immunomodulant or immunosuppressant medications was identified in 93% and 88% of patients with PV and MMP, respectively.
  • #37 Paraneoplastic Pemphigus Treatment & Management: Medical Care, Surgical Care, Consultations
    https://emedicine.medscape.com/article/1064452-treatment
    Treating the underlying malignancy may control autoantibody production, and intravenous gammaglobulin (IVIG) at the time of surgery may help prevent the development of bronchiolitis obliterans. However, once this develops, it is typically not reversible. […] For solid neoplasms, curative resection should be attempted when appropriate, but this does not halt disease progression, especially when malignant. If surgery results in decreased autoantibody production, the paraneoplastic pemphigus may improve. IVIG before, during, and after the surgery may block autoantibody released from the tumor. […] Respiratory therapy may be beneficial when pulmonary involvement from paraneoplastic pemphigus causes respiratory insufficiency. Consultations from a pulmonary medicine specialist, an ophthalmologist, a gastroenterologist, and an otolaryngologist should be obtained when appropriate.
  • #38 The management of pemphigus vulgaris and mucous membrane pemphigoid in a joint oral medicine and dermatology clinic: a five-year narrative review | British Dental Journal
    https://www.nature.com/articles/s41415-024-7074-8
    The BJOMDCC facilitates discussion between physicians, with exploration of potential management strategies from different viewpoints, thereby avoiding care silos’ with the risk that this brings when each specialist focusses on their own clinical priorities without joined-up thinking. […] Topical steroid therapy is considered first-line treatment for oral mucosal involvement in AIBDs. […] In such cases, a stepladder approach with the addition of systemic steroid therapy in combination with one or more immunomodulatory or immunosuppressant medications is recommended by current guidelines. […] Conventional systemic immunosuppression remains the mainstay approach within the BJOMDCC, reflecting the severity and multisite involvement in this cohort. […] Combination therapy with two or more immunomodulant or immunosuppressant medications was identified in 93% and 88% of patients with PV and MMP, respectively.
  • #39 Managing pemphigus in primary care
    https://www.healthcert.com/blog/how-to-manage-pemphigus
    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. […] 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.
  • #40 Pemphigus Vulgaris Treatment & Management: Medical Care, Complications, Diet
    https://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. […] Treatment with corticosteroids has improved the overall mortality, but significant morbidity remains. Much of the mortality and morbidity in patients with pemphigus vulgaris is now related to the adverse effects of such therapy. […] Immunosuppressive drugs are steroid-sparing and should be considered early in the course of the disease.
  • #41 Initial management of pemphigus vulgaris and pemphigus foliaceus – UpToDate
    https://www.uptodate.com/contents/initial-management-of-pemphigus-vulgaris-and-pemphigus-foliaceus
    Initial management of pemphigus vulgaris and pemphigus foliaceus […] Systemic glucocorticoids and rituximab are the mainstays of therapy for pemphigus vulgaris and pemphigus foliaceus and are usually highly effective for obtaining control of disease. […] Other immunomodulatory agents, such as azathioprine and mycophenolate mofetil, are commonly prescribed in conjunction with systemic glucocorticoids in an attempt to minimize the risk for adverse effects of long-term, high-dose glucocorticoid therapy. […] The initial management of pemphigus vulgaris and pemphigus foliaceus will be reviewed here. […] SUPPORTIVE CARE includes skin care, management of oral symptoms, and patient support.
  • #42 Pemphigus: Diagnosis and treatment
    https://www.aad.org/public/diseases/a-z/pemphigus-treatment
    Pemphigus cannot be cured, but with treatment, most people can control their pemphigus. […] Treatment can reduce (and sometimes clear) the blisters and sores caused by all types of pemphigus. Treatment can also prevent pemphigus from worsening. […] A treatment plan for pemphigus may include one or more of the following: […] Your dermatologist may include baths and wound dressings in your treatment plan. This can help heal blisters and sores. […] Taking both a corticosteroid like prednisone, and an immunosuppressant medication like azathioprine to quiet the immune system, may deliver better results. […] While in the hospital, treatment can also be given to help get pemphigus under control. […] To keep pemphigus under control, you may need to see both a dermatologist and your primary care physician.
  • #43 Pemphigus: Types, Symptoms, Causes & Treatment
    https://my.clevelandclinic.org/health/diseases/21130-pemphigus
    Pemphigus is a group of autoimmune skin conditions that cause sores, blisters or fluid-filled bumps to form on your skin and mucus membranes. Pemphigus is sometimes confused with other autoimmune blistering skin conditions such as bullous pemphigoid, lupus erythematosus and Hailey-Hailey disease. Pemphigus is a lifelong condition that can be managed with ongoing medical treatment. Treatment is unique to each person diagnosed with pemphigus and could include: Taking medicine to prevent infections and help your skin heal. Wound care for blisters and sores. 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. Consolidation: Steady doses of medications continue healing blisters until most clear up. Maintenance: Reduced levels of medications keep new blisters from forming. You can take steps at home to manage your symptoms of pemphigus by: Caring for your blisters as directed by your healthcare provider. Staying out of the sunlight or covering your skin with sunscreen or wearing protective clothing to prevent UV ray damage to your skin. Using soaps and lotions designed for sensitive skin, without fragrances to prevent skin irritation. Your healthcare provider will monitor your condition regularly and order blood and/or urine tests to verify that your treatment is working. If you have trouble eating, contact your healthcare provider. They may recommend taking nutritional supplements to avoid malnutrition. If left untreated, symptoms can affect your overall health and could cause life-threatening symptoms. Your healthcare provider will work closely with you to help you manage your symptoms and eliminate discomfort, especially to prevent infections. Be patient with your body to heal and try not to injure your blisters or skin sores.
  • #44 Pemphigus: Types, Symptoms, Causes & Treatment
    https://my.clevelandclinic.org/health/diseases/21130-pemphigus
    Pemphigus is a group of autoimmune skin conditions that cause sores, blisters or fluid-filled bumps to form on your skin and mucus membranes. Pemphigus is sometimes confused with other autoimmune blistering skin conditions such as bullous pemphigoid, lupus erythematosus and Hailey-Hailey disease. Pemphigus is a lifelong condition that can be managed with ongoing medical treatment. Treatment is unique to each person diagnosed with pemphigus and could include: Taking medicine to prevent infections and help your skin heal. Wound care for blisters and sores. 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. Consolidation: Steady doses of medications continue healing blisters until most clear up. Maintenance: Reduced levels of medications keep new blisters from forming. You can take steps at home to manage your symptoms of pemphigus by: Caring for your blisters as directed by your healthcare provider. Staying out of the sunlight or covering your skin with sunscreen or wearing protective clothing to prevent UV ray damage to your skin. Using soaps and lotions designed for sensitive skin, without fragrances to prevent skin irritation. Your healthcare provider will monitor your condition regularly and order blood and/or urine tests to verify that your treatment is working. If you have trouble eating, contact your healthcare provider. They may recommend taking nutritional supplements to avoid malnutrition. If left untreated, symptoms can affect your overall health and could cause life-threatening symptoms. Your healthcare provider will work closely with you to help you manage your symptoms and eliminate discomfort, especially to prevent infections. Be patient with your body to heal and try not to injure your blisters or skin sores.
  • #45 Pemphigus Vulgaris: A Complete Overview — DermNet
    https://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. Other immunosuppressive drugs (mostly off-label) used to reduce the dose of steroids and may be required by patients with pemphigus vulgaris for years. Appropriate wound care is particularly important, as this should promote healing of blisters and erosions. Patients should minimise activities that may traumatise the skin and mucous membranes during active phases of the disease. Oral hygiene and proper dental care are essential. Topical therapy for cutaneous pemphigus vulgaris may include topical steroids and emollients. Treatment of mucosal pemphigus vulgaris may include various formulations of a topical steroid, intralesional steroid, topical tacrolimus, or topical ciclosporin. […] At optimal therapy, patients may continue to experience mild disease activity. Other considerations include vaccination (live vaccines are contraindicated), bone prophylaxis, baseline ophthalmological assessment, and psychological support if needed.
  • #46 Autoimmune Bullous Dermatoses: A Review | AAFP
    https://www.aafp.org/pubs/afp/issues/2002/0501/p1861.html
    Treatment of mild lesions of the skin and oral mucosa consists of topical corticosteroids in a gel or occlusive base, which is best used before bedtime. A swish and spit dexamethasone (Roxane) mouthwash can be helpful for oral lesions. Dapsone has been shown to be of benefit in some cases. In severe cases of cicatricial pemphigoid, systemic steroids are prescribed, with or without dapsone. Because of the severity of the sequelae of these lesions, aggressive early treatment is essential. For severe ocular involvement, patients are treated with systemic steroids, along with cyclophosphamide or azathioprine. Prednisone is usually given for three to six months, with the cyclophosphamide or azathioprine continued for one year. At that point, the medications may be decreased, and if the patient remains free of disease, the medications may be discontinued.
  • #47 Pemphigus – what are the symptoms and how to treat effectively? • OT.CO Clinic
    https://otcoclinic.com/blog/pemphigus-what-are-the-symptoms-and-how-effectively-treat/
    Pemphigus is a disease of the skin and mucous membranes that causes blisters. […] Pemphigus is a disease for which two-pronged treatment must be applied. This involves the use of both topical agents to soothe and reduce the lesions, and systemic agents to calm the autoimmune reaction. […] For topical treatment, suspensions for brushing mucous membranes in the mouth, medicated baths and ointments are recommended, depending on the area where the lesions are located. […] In terms of systemic treatment, corticosteroids are also used, but in tablet form (most commonly prednisone or prednisolone), as well as immunosuppressive drugs such as azathioprine, which reduce the activity of the immune system. […] For those affected by pemphigus, the OT.CO Clinic offers tailored services in diagnosing and treating this condition. […] Treatment focuses on managing symptoms and calming immune responses through topical and systemic therapies.
  • #48 Pemphigus vulgaris
    https://www.nhs.uk/conditions/pemphigus-vulgaris/
    If a GP thinks you could have pemphigus vulgaris, they can refer you to a skin specialist (dermatologist) for tests and treatment. […] Blisters caused by pemphigus vulgaris usually come and go over time. There’s no cure, but treatment can help. […] The main treatments are: steroid tablets, other medicines such as azathioprine, methotrexate and rituximab. […] It usually takes a few weeks for the blisters to heal. Once this happens, you’ll probably need treatment for several years to help stop the blisters coming back. […] 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.
  • #49 Self-Care: Comfort Techniques and Dressings – IPPF
    https://www.pemphigus.org/self-care-comfort-techniques-and-dressings/
    I established a positive and team-focused relationship with three local pharmacies where I ordered my medications and care products. […] I continue to work with my dentist, dental hygienist, and oral surgeon to manage my oral hygiene. […] I found it helpful to wear lightweight, dark-colored athletic clothing with cooling wick technology. […] I learned that if my clothes let me breathe, felt cool and light I had something to work with. […] My family and I developed a schedule after guidance from my medical team. […] I found using a product called magic mouth helped greatly with my painful mouth sores. […] I worked with a dietician to identify foods that both met my nutritional needs and kept me from gaining weight due to little activity and medication. […] I recommend keeping your house (or at least a room where you spend your time in) cool, quiet, and filled with various activities like TV, movies, and books. […] I stayed in constant contact with my employer, and as a result they were very supportive. […] My family and I are very well-informed about bullous pemphigoid (BP), and we’re a part of the IPPF community.
  • #50 Autoimmune Bullous Dermatoses: A Review | AAFP
    https://www.aafp.org/pubs/afp/issues/2002/0501/p1861.html
    Treatment of mild lesions of the skin and oral mucosa consists of topical corticosteroids in a gel or occlusive base, which is best used before bedtime. A swish and spit dexamethasone (Roxane) mouthwash can be helpful for oral lesions. Dapsone has been shown to be of benefit in some cases. In severe cases of cicatricial pemphigoid, systemic steroids are prescribed, with or without dapsone. Because of the severity of the sequelae of these lesions, aggressive early treatment is essential. For severe ocular involvement, patients are treated with systemic steroids, along with cyclophosphamide or azathioprine. Prednisone is usually given for three to six months, with the cyclophosphamide or azathioprine continued for one year. At that point, the medications may be decreased, and if the patient remains free of disease, the medications may be discontinued.
  • #51 Pemphigus: 10 tips for managing
    https://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. […] Understand that it can take time to get pemphigus under control. Some patients need months or years, but pemphigus is nearly always controllable. […] Take care of mouth sores. If pemphigus causes sores in your mouth, you can ease the pain by: […] Keeping your mouth clean. Poor oral hygiene can worsen mouth sores. Your dermatologist can recommend a toothbrush, toothpaste, and mouthwash that are least likely to cause pain. […] Tell your dermatologist about eye problems. Blisters can form on the tissue lining an eyelid.
  • #52 Self-Care: Comfort Techniques and Dressings – IPPF
    https://www.pemphigus.org/self-care-comfort-techniques-and-dressings/
    Pemphigus and pemphigoid (P/P) patients may agree that it can be very difficult and painful in the early months of their condition. […] The following suggestions may be helpful, such as learning how to care for our skin by taking and applying topical medications, cleansers, moisturizers, dressings, and staying comfortable. […] When applying or changing dressings, wash your hands with soap and water, and use gloves. […] My dermatologist had previously instructed me on the exact amount to use when applying topical steroid to avoid damaging the skin. […] Additionally, I made sure that the opening or mouth of the tube of medication didn’t touch my skin in order to avoid infection. […] Make sure you review exactly how to use these and any products when caring for your skin including how often to check and change your dressings with your health care provider.
  • #53 Pemphigus – Diagnosis and treatment – Mayo Clinic
    https://www.mayoclinic.org/diseases-conditions/pemphigus/diagnosis-treatment/drc-20350409
    Your healthcare professional may start by talking with you about your medical history and symptoms and examining the affected area. […] Treatment for pemphigus usually starts with medicines to ease symptoms and prevent new blisters. These may include steroids and medicines that target the immune system. […] Many people with pemphigus get better, especially if treatment is started early. But it may take years and can require taking medicine for a long time. […] Taking good care of your wounds can help prevent infection and scarring. Talk with your healthcare professional about how best to care for your wounds and control pain. […] Regular follow-up with a dentist is important for people with pemphigus. Talk with your dentist about how to best take care of your teeth and gums. […] Pemphigus may be difficult to live with, especially if it affects your daily activities or causes lost sleep or stress. You may find it helpful to talk to others with the disease. You may want to connect with a support group in person or online. Ask your healthcare professional for suggestions.
  • #54 Pemphigus – Diagnosis and treatment – Mayo Clinic
    https://www.mayoclinic.org/diseases-conditions/pemphigus/diagnosis-treatment/drc-20350409
    Your healthcare professional may start by talking with you about your medical history and symptoms and examining the affected area. […] Treatment for pemphigus usually starts with medicines to ease symptoms and prevent new blisters. These may include steroids and medicines that target the immune system. […] Many people with pemphigus get better, especially if treatment is started early. But it may take years and can require taking medicine for a long time. […] Taking good care of your wounds can help prevent infection and scarring. Talk with your healthcare professional about how best to care for your wounds and control pain. […] Regular follow-up with a dentist is important for people with pemphigus. Talk with your dentist about how to best take care of your teeth and gums. […] Pemphigus may be difficult to live with, especially if it affects your daily activities or causes lost sleep or stress. You may find it helpful to talk to others with the disease. You may want to connect with a support group in person or online. Ask your healthcare professional for suggestions.
  • #55 Pemphigus: Diagnosis, Treatment, and Steps to Take
    https://www.niams.nih.gov/health-topics/pemphigus/diagnosis-treatment-and-steps-to-take
    Early diagnosis is important, so if you have blisters on the skin or in the mouth that do not go away, it is important to see a doctor as soon as you can. […] Treatment typically depends on the severity and stage of the disease. […] Symptoms of pemphigus may go away after many years of treatment, but most people need to continue taking medications to keep the disease under control. […] Be sure to report any problems or side effects from medications to your doctor. […] In some cases, a person with pemphigus may need to be hospitalized to treat health problems that the disease or its treatment can cause. […] Blisters in the mouth may make brushing and flossing your teeth painful, so talk to your dentist about ways to keep your teeth and gums healthy. […] Your dermatologist may recommend baths and wound dressings to help heal the skin sores and blisters. […] Pemphigus and its treatments can be debilitating and cause lost time at work, weight loss, sleep problems, and emotional distress. […] Remember to follow the recommendations of your health care providers.
  • #56 Self-Care: Comfort Techniques and Dressings – IPPF
    https://www.pemphigus.org/self-care-comfort-techniques-and-dressings/
    I established a positive and team-focused relationship with three local pharmacies where I ordered my medications and care products. […] I continue to work with my dentist, dental hygienist, and oral surgeon to manage my oral hygiene. […] I found it helpful to wear lightweight, dark-colored athletic clothing with cooling wick technology. […] I learned that if my clothes let me breathe, felt cool and light I had something to work with. […] My family and I developed a schedule after guidance from my medical team. […] I found using a product called magic mouth helped greatly with my painful mouth sores. […] I worked with a dietician to identify foods that both met my nutritional needs and kept me from gaining weight due to little activity and medication. […] I recommend keeping your house (or at least a room where you spend your time in) cool, quiet, and filled with various activities like TV, movies, and books. […] I stayed in constant contact with my employer, and as a result they were very supportive. […] My family and I are very well-informed about bullous pemphigoid (BP), and we’re a part of the IPPF community.
  • #57 Pemphigus: Types, Symptoms, Causes & Treatment
    https://my.clevelandclinic.org/health/diseases/21130-pemphigus
    Pemphigus is a group of autoimmune skin conditions that cause sores, blisters or fluid-filled bumps to form on your skin and mucus membranes. Pemphigus is sometimes confused with other autoimmune blistering skin conditions such as bullous pemphigoid, lupus erythematosus and Hailey-Hailey disease. Pemphigus is a lifelong condition that can be managed with ongoing medical treatment. Treatment is unique to each person diagnosed with pemphigus and could include: Taking medicine to prevent infections and help your skin heal. Wound care for blisters and sores. 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. Consolidation: Steady doses of medications continue healing blisters until most clear up. Maintenance: Reduced levels of medications keep new blisters from forming. You can take steps at home to manage your symptoms of pemphigus by: Caring for your blisters as directed by your healthcare provider. Staying out of the sunlight or covering your skin with sunscreen or wearing protective clothing to prevent UV ray damage to your skin. Using soaps and lotions designed for sensitive skin, without fragrances to prevent skin irritation. Your healthcare provider will monitor your condition regularly and order blood and/or urine tests to verify that your treatment is working. If you have trouble eating, contact your healthcare provider. They may recommend taking nutritional supplements to avoid malnutrition. If left untreated, symptoms can affect your overall health and could cause life-threatening symptoms. Your healthcare provider will work closely with you to help you manage your symptoms and eliminate discomfort, especially to prevent infections. Be patient with your body to heal and try not to injure your blisters or skin sores.
  • #58 Pemphigus: 10 tips for managing
    https://www.aad.org/public/diseases/a-z/pemphigus-self-care
    Avoid sun and heat if you have pemphigus foliaceus or drug-induced pemphigus. If you’ve been diagnosed with pemphigus foliaceus or one of its subtypes, such as drug-induced pemphigus or fogo selvagem, you want to: […] Partner with your dermatologist. Your dermatologist can be your strongest ally. Dermatologists help patients with pemphigus find treatment that works. No one treatment works for everyone. Your dermatologist can also help you manage possible side effects and answer questions about the disease. […] If you remember only one thing from this site, it should be that today pemphigus is nearly always controllable. By keeping your dermatology appointments, you can partner with your dermatologist to make that a reality.
  • #59 Pemphigus vulgaris
    https://www.nhs.uk/conditions/pemphigus-vulgaris/
    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. […] Pemphigus vulgaris can lead to other problems, such as: blisters and sore patches becoming infected, which can cause life-threatening problems such as sepsis, weight loss and malnutrition because of pain when eating and drinking, tooth decay and gum disease because it can be painful to look after your teeth and gums, pain during sex and pain when peeing or pooing (if your genitals or anus are affected). […] You’ll have regular check-ups to look for these problems and may be referred to other specialists (such as a dietitian) if needed. […] Living with pemphigus vulgaris can be very difficult.
  • #60 Self-Care: Comfort Techniques and Dressings – IPPF
    https://www.pemphigus.org/self-care-comfort-techniques-and-dressings/
    I established a positive and team-focused relationship with three local pharmacies where I ordered my medications and care products. […] I continue to work with my dentist, dental hygienist, and oral surgeon to manage my oral hygiene. […] I found it helpful to wear lightweight, dark-colored athletic clothing with cooling wick technology. […] I learned that if my clothes let me breathe, felt cool and light I had something to work with. […] My family and I developed a schedule after guidance from my medical team. […] I found using a product called magic mouth helped greatly with my painful mouth sores. […] I worked with a dietician to identify foods that both met my nutritional needs and kept me from gaining weight due to little activity and medication. […] I recommend keeping your house (or at least a room where you spend your time in) cool, quiet, and filled with various activities like TV, movies, and books. […] I stayed in constant contact with my employer, and as a result they were very supportive. […] My family and I are very well-informed about bullous pemphigoid (BP), and we’re a part of the IPPF community.
  • #61 Management of chronic wounds in patients with pemphigus | CWCMR
    https://www.dovepress.com/management-of-chronic-wounds-in-patients-with-pemphigus-peer-reviewed-fulltext-article-CWCMR
    Pemphigus is a group of chronic IgG-mediated autoimmune blistering diseases that involves both the skin and mucous membranes. […] The primary goal of treatment is to induce durable remission, decrease blister formation, prevent infections and promote healing of blisters and erosions. […] 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. […] 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. […] When the disease becomes severe with extensive skin involvement, patients are typically admitted to the burn unit.
  • #62 Pemphigus Vulgaris – UF Health
    https://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. […] 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.
  • #63 Pemphigus vulgaris: MedlinePlus Medical EncyclopediaLock
    https://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. […] 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.
  • #64 Wound Care in Immunobullous Disease | IntechOpen
    https://www.intechopen.com/chapters/58481
    When patients who have extensive raw areas are hospitalized, they must be isolated to reduce cross-infection. Secondary bacterial (MRSA, pseudomonas) or viral (HSV) infections can occur. The use of antimicrobials is effective. Additionally, it is important to routinely monitor blood pressure and blood glucose levels. […] In severe disease, the premise of care is very similar to patients being treated for partial thickness burn therapy. Frequently in pemphigus vulgaris and bullous pemphigoid, the body is covered with intact bullae and partial-thickness wounds from head to toe. […] In severe pemphigus and pemphigoid, various goals of wound care management have been identified. These include relieving severe pain, preventing infection and decreasing bioburden, enhancing regeneration of the dermis and the epidermis, protecting the periwound skin from maceration, encouraging patient mobility and quality of life, providing nutritional support for tissue repair, as well as treating the underlying cause of the wound. […] Ultimately, the wound care plan focuses on reducing pain, preventing infection by gentle cleansing and preventing scarring by providing a moist environment. Additionally, nutrition is a key aspect of care, due to the loss of protein and other essential components in the serous sanguineous drainage.
  • #65 Paraneoplastic Pemphigus Treatment & Management: Medical Care, Surgical Care, Consultations
    https://emedicine.medscape.com/article/1064452-treatment
    Response to treatment paraneoplastic pemphigus is generally poor, especially for mucosal lesions. Initial care is aimed at treating superinfection, if present. Warm compresses, nonadherent wound dressings, and topical antibiotic ointment are helpful. Potent immunosuppressive agents are required to decrease blistering, but they are often ineffective. High-dose corticosteroids are first-line therapy for paraneoplastic pemphigus, followed by steroid-sparing agents such as azathioprine, cyclosporine, and mycophenolate mofetil. In general, the skin lesions of paraneoplastic pemphigus are more responsive to therapy than mucosal lesions. […] Other therapeutic options for paraneoplastic pemphigus include plasmapheresis, immunophoresis, intravenous gammaglobulin, and stem cell ablation therapy with high-dose cyclophosphamide without stem cell rescue. Rituximab has been tried in several patients with mixed results. Alemtuzumab, an agent that targets CD52, has also been used with success. Successful treatment of refractory PNP with obinutuzumab, a humanized anti-CD20 monoclonal antibody, has been reported.
  • #66 Paraneoplastic Pemphigus Treatment & Management: Medical Care, Surgical Care, Consultations
    https://emedicine.medscape.com/article/1064452-treatment
    Response to treatment paraneoplastic pemphigus is generally poor, especially for mucosal lesions. Initial care is aimed at treating superinfection, if present. Warm compresses, nonadherent wound dressings, and topical antibiotic ointment are helpful. Potent immunosuppressive agents are required to decrease blistering, but they are often ineffective. High-dose corticosteroids are first-line therapy for paraneoplastic pemphigus, followed by steroid-sparing agents such as azathioprine, cyclosporine, and mycophenolate mofetil. In general, the skin lesions of paraneoplastic pemphigus are more responsive to therapy than mucosal lesions. […] Other therapeutic options for paraneoplastic pemphigus include plasmapheresis, immunophoresis, intravenous gammaglobulin, and stem cell ablation therapy with high-dose cyclophosphamide without stem cell rescue. Rituximab has been tried in several patients with mixed results. Alemtuzumab, an agent that targets CD52, has also been used with success. Successful treatment of refractory PNP with obinutuzumab, a humanized anti-CD20 monoclonal antibody, has been reported.
  • #67 Paraneoplastic Pemphigus Treatment & Management: Medical Care, Surgical Care, Consultations
    https://emedicine.medscape.com/article/1064452-treatment
    Treating the underlying malignancy may control autoantibody production, and intravenous gammaglobulin (IVIG) at the time of surgery may help prevent the development of bronchiolitis obliterans. However, once this develops, it is typically not reversible. […] For solid neoplasms, curative resection should be attempted when appropriate, but this does not halt disease progression, especially when malignant. If surgery results in decreased autoantibody production, the paraneoplastic pemphigus may improve. IVIG before, during, and after the surgery may block autoantibody released from the tumor. […] Respiratory therapy may be beneficial when pulmonary involvement from paraneoplastic pemphigus causes respiratory insufficiency. Consultations from a pulmonary medicine specialist, an ophthalmologist, a gastroenterologist, and an otolaryngologist should be obtained when appropriate.
  • #68 Paraneoplastic Pemphigus Treatment & Management: Medical Care, Surgical Care, Consultations
    https://emedicine.medscape.com/article/1064452-treatment
    Treating the underlying malignancy may control autoantibody production, and intravenous gammaglobulin (IVIG) at the time of surgery may help prevent the development of bronchiolitis obliterans. However, once this develops, it is typically not reversible. […] For solid neoplasms, curative resection should be attempted when appropriate, but this does not halt disease progression, especially when malignant. If surgery results in decreased autoantibody production, the paraneoplastic pemphigus may improve. IVIG before, during, and after the surgery may block autoantibody released from the tumor. […] Respiratory therapy may be beneficial when pulmonary involvement from paraneoplastic pemphigus causes respiratory insufficiency. Consultations from a pulmonary medicine specialist, an ophthalmologist, a gastroenterologist, and an otolaryngologist should be obtained when appropriate.
  • #69 Managing pemphigus in primary care
    https://www.healthcert.com/blog/how-to-manage-pemphigus
    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. […] 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.
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  • #71 Pemphigus: Types, Symptoms, Causes & Treatment
    https://my.clevelandclinic.org/health/diseases/21130-pemphigus
    Pemphigus is a group of autoimmune skin conditions that cause sores, blisters or fluid-filled bumps to form on your skin and mucus membranes. Pemphigus is sometimes confused with other autoimmune blistering skin conditions such as bullous pemphigoid, lupus erythematosus and Hailey-Hailey disease. Pemphigus is a lifelong condition that can be managed with ongoing medical treatment. Treatment is unique to each person diagnosed with pemphigus and could include: Taking medicine to prevent infections and help your skin heal. Wound care for blisters and sores. 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. Consolidation: Steady doses of medications continue healing blisters until most clear up. Maintenance: Reduced levels of medications keep new blisters from forming. You can take steps at home to manage your symptoms of pemphigus by: Caring for your blisters as directed by your healthcare provider. Staying out of the sunlight or covering your skin with sunscreen or wearing protective clothing to prevent UV ray damage to your skin. Using soaps and lotions designed for sensitive skin, without fragrances to prevent skin irritation. Your healthcare provider will monitor your condition regularly and order blood and/or urine tests to verify that your treatment is working. If you have trouble eating, contact your healthcare provider. They may recommend taking nutritional supplements to avoid malnutrition. If left untreated, symptoms can affect your overall health and could cause life-threatening symptoms. Your healthcare provider will work closely with you to help you manage your symptoms and eliminate discomfort, especially to prevent infections. Be patient with your body to heal and try not to injure your blisters or skin sores.
  • #72 Pemphigus in Dogs | PetMD
    https://www.petmd.com/dog/conditions/skin/pemphigus-dogs
    Treatment is usually effective for pemphigus foliaceus, even in severe cases. Most dogs with less severe pemphigus improve within two to six weeks of starting treatment. […] However, most patients with pemphigus vulgaris are humanely euthanized due to their condition, even with aggressive treatment. […] Dogs being treated for pemphigus should have checkups at least every six months with their vet. […] For dogs with pemphigus, avoid sunlight, especially during warmer months. Pet-safe sunscreens may be helpful, though care should be taken to avoid getting the sunscreen into their eyes. […] Most dogs with pemphigus should continue year-round parasite prevention. Fleas can significantly worsen your dogs discomfort by causing itchiness, secondary infections, and allergic reactions.
  • #73 Pemphigus (includes “pemphigus vulgaris” [“PV”], “pemphigus vegetans”, “pemphigus foliaceus”, “pemphigus erythematosus”, “paraneoplastic pemphigus”, and “drug-related pemphigus”)1 – CDHO
    https://cdho.org/factsheets/pemphigus/
    Severe oral erosions interfere with patients/clients proper eating and drinking, as well as hinder nutrient intake. Intravenous (IV) feeding may be needed in very severe cases. […] Oral prophylaxis should ideally be performed prior to the initiation of systemic or topical therapy. […] Routine oral hygiene (including brushing) is often compromised due to pain in the mouth. […] During the active oral disease stage, patient/client follow-up is recommended as frequently as every 4 to 6 weeks for debridement, and it should include monitoring for oral candidiasis. […] The dental hygienist should anticipate that the patient/client may experience pain and bleeding during procedures, and plan for this by scheduling extra time and using suction and gauze as needed. […] Simple hand scaling instruments are often most effective, particularly in patients/clients with severe mucosal disease. […] Disease flares following dental work are fairly common. […] The dental hygienist plays a role in monitoring the patient/client for long-term effects of corticosteroid and immunosuppressive treatment.
  • #74 Pemphigus: Symptoms and Treatment | Doctor
    https://patient.info/doctor/pemphigus
    Pemphigus treatment and management1 13 […] There are differences of opinion on diagnosis and treatment, which require more research. General measures include advising patients with painful oral lesions to have soft diets and use soft toothbrushes. Topical analgesics or anaesthetics may give some symptomatic relief. […] Treatment mainly relies on immune system suppression to prevent new lesion formation and heal existing bullous skin and/or mucous lesions. For all forms of pemphigus, corticosteroids are considered as a first-line therapy, with or without adjuvant therapies. […] Generally, mild pemphigus treatment involves lower steroid doses compared to moderate/severe disease treatment, which involves higher steroid doses with the addition of steroid-sparing adjuvant agents. […] Rituximab efficacy is higher when it is administered early in the course of the disease. Therefore, it should be considered as first-line treatment to improve efficacy and reduce cumulative doses of corticosteroids and their side effects. […] For all forms, the most important factor is treating quickly and effectively and to taper corticosteroids to avoid recurrence. […] Patients should be followed up routinely until all treatment has been stopped, and the patient remains in remission.
  • #75 Pemphigus – Symptoms and causes – Mayo Clinic
    https://www.mayoclinic.org/diseases-conditions/pemphigus/symptoms-causes/syc-20350404
    Pemphigus is easier to control if caught and treated early. It’s usually treated with medicines that you take long term. The sores may heal slowly or not at all. The condition can become life-threatening if the sores become infected. […] See a healthcare professional if you have blisters that don’t heal in the mouth or on the skin or genital mucous membranes. […] Possible complications of pemphigus include: Infection of the skin. Infection that spreads to your bloodstream, also called sepsis. This type of infection can be life-threatening. Malnutrition, because painful mouth sores make it difficult to eat. Side effects from the medicine used to treat pemphigus. Examples are high blood pressure and infection.
  • #76 Pemphigus vulgaris
    https://www.nhs.uk/conditions/pemphigus-vulgaris/
    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. […] Pemphigus vulgaris can lead to other problems, such as: blisters and sore patches becoming infected, which can cause life-threatening problems such as sepsis, weight loss and malnutrition because of pain when eating and drinking, tooth decay and gum disease because it can be painful to look after your teeth and gums, pain during sex and pain when peeing or pooing (if your genitals or anus are affected). […] You’ll have regular check-ups to look for these problems and may be referred to other specialists (such as a dietitian) if needed. […] Living with pemphigus vulgaris can be very difficult.
  • #77 Pemphigus – Symptoms and causes – Mayo Clinic
    https://www.mayoclinic.org/diseases-conditions/pemphigus/symptoms-causes/syc-20350404
    Pemphigus is easier to control if caught and treated early. It’s usually treated with medicines that you take long term. The sores may heal slowly or not at all. The condition can become life-threatening if the sores become infected. […] See a healthcare professional if you have blisters that don’t heal in the mouth or on the skin or genital mucous membranes. […] Possible complications of pemphigus include: Infection of the skin. Infection that spreads to your bloodstream, also called sepsis. This type of infection can be life-threatening. Malnutrition, because painful mouth sores make it difficult to eat. Side effects from the medicine used to treat pemphigus. Examples are high blood pressure and infection.
  • #78 Pemphigus Vulgaris: Cause, Types, and Symptoms
    https://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.
  • #79 Pemphigus – Diagnosis and treatment – Mayo Clinic
    https://www.mayoclinic.org/diseases-conditions/pemphigus/diagnosis-treatment/drc-20350409
    Your healthcare professional may start by talking with you about your medical history and symptoms and examining the affected area. […] Treatment for pemphigus usually starts with medicines to ease symptoms and prevent new blisters. These may include steroids and medicines that target the immune system. […] Many people with pemphigus get better, especially if treatment is started early. But it may take years and can require taking medicine for a long time. […] Taking good care of your wounds can help prevent infection and scarring. Talk with your healthcare professional about how best to care for your wounds and control pain. […] Regular follow-up with a dentist is important for people with pemphigus. Talk with your dentist about how to best take care of your teeth and gums. […] Pemphigus may be difficult to live with, especially if it affects your daily activities or causes lost sleep or stress. You may find it helpful to talk to others with the disease. You may want to connect with a support group in person or online. Ask your healthcare professional for suggestions.
  • #80 Pemphigus Vulgaris – UF Health
    https://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. […] 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.
  • #81 Pemphigus Vulgaris Treatment & Management: Medical Care, Complications, Diet
    https://emedicine.medscape.com/article/1064187-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. […] 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.
  • #82 Nursing Care of Patients with Severe Pemphigus | Li | Journal of Nursing
    https://nursing.usp-pl.com/index.php/Nursing/article/view/175
    Nursing care of patients with severe pemphigus is critical to monitor and nurse patients’ clinical conditions. This article explores the nursing methods of patients with severe pemphigus, hoping to improve the treatment effect through clinical observation and nursing intervention. […] It would be difficult to achieve satisfactory curative effect only by such treatment.
  • #83 Nursing Care of Patients with Severe Pemphigus | Li | Journal of Nursing
    https://nursing.usp-pl.com/index.php/Nursing/article/view/175
    Nursing care of patients with severe pemphigus is critical to monitor and nurse patients’ clinical conditions. This article explores the nursing methods of patients with severe pemphigus, hoping to improve the treatment effect through clinical observation and nursing intervention. […] It would be difficult to achieve satisfactory curative effect only by such treatment.
  • #84 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.
  • #85 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.
  • #86 Managing pemphigus in primary care
    https://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. […] The correct diagnosis is the first step in managing pemphigus. The presence of painful, flaccid blisters, erosions, or ulcers on the skin and/or mucosal surfaces (oral cavity, genitals) is the first obvious sign. However, confirmation typically requires laboratory testing. […] 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.
  • #87 Treatment and Research for Pemphigus | Center for Medical Dermatology + Immunology Research
    https://dermimmunology.com/services/pemphigus
    Unfortunately, pemphigus requires lifelong management through continuous medical care. […] A personalized and tailored treatment plan is integral to addressing pemphigus. […] Collaborating with you, we tailor an advanced treatment plan to control the spread of pemphigus and alleviate blister formation. […] Our comprehensive approach merges state-of-the-art research and specialized care, delivering unparalleled expertise to individuals with complex, chronic skin conditions such as pemphigus.