Zespół stevens-johnsona – Diagnostyka i diagnoza

Zespół stevens-johnsona
Diagnostyka i diagnoza

Zespół Stevens-Johnsona (SJS) to ciężka, potencjalnie zagrażająca życiu reakcja polekowa, charakteryzująca się martwicą pełnej grubości naskórka i zajęciem co najmniej dwóch błon śluzowych. Diagnostyka opiera się na obrazie klinicznym, wywiadzie oraz potwierdzeniu histopatologicznym w biopsji skóry, gdzie obserwuje się martwicę naskórka, apoptozę keratynocytów i pęcherze podnaskórkowe. Klasyfikacja SJS zależy od powierzchni ciała z oddzieleniem naskórka: <10% dla SJS, 10-30% dla postaci nakładającej się SJS/TEN oraz >30% dla toksycznej nekrolizy naskórka (TEN). Wczesne objawy obejmują gorączkę, objawy grypopodobne oraz zmiany błon śluzowych (światłowstręt, pieczenie spojówek, dysfagia). Diagnostyka różnicowa obejmuje m.in. rumień wielopostaciowy, AGEP, DRESS oraz pęcherzyce. SCORTEN, punktowa skala oceny ciężkości, uwzględnia m.in. wiek >40 lat, oddzielenie naskórka >10%, stężenie mocznika >10 mmol/l i glukozy >14 mmol/l, pozwalając oszacować śmiertelność od 3,2% (wynik 0-1) do 90% (wynik ≥5).

Objawy i rozwój zespołu Stevens-Johnsona
Diagnostyka i rozpoznanie zespołu Stevens-Johnsona

Kryteria rozpoznania
Biopsja skóry
Badania laboratoryjne
Biomarkery w diagnostyce SJS
Badania obrazowe
Ocena ciężkości choroby – SCORTEN
Rozpoznanie różnicowe

Określenie przyczyny reakcji
Postępowanie i leczenie
Rokowanie i monitorowanie
Wnioski i podsumowanie

Kolejne rozdziały

Objawy i rozwój zespołu Stevens-Johnsona

Zespół Stevens-Johnsona (SJS) to rzadka, ale potencjalnie zagrażająca życiu reakcja skórna, charakteryzująca się rozległą martwicą naskórka i złuszczaniem się skóry. Reakcja ta w większości przypadków jest wywołana przez leki (ponad 80% przypadków), chociaż może również wystąpić w wyniku infekcji, zwłaszcza Mycoplasma pneumoniae, wirusa HIV, chorób nowotworowych czy czynników genetycznych.¹

Początkowe objawy zespołu Stevens-Johnsona (SJS) mogą obejmować objawy grypopodobne, w tym gorączkę, złe samopoczucie, ból głowy, kaszel i/lub katar, które występują na 1-3 dni przed pojawieniem się zmian skórnych.¹ Wczesne objawy zajęcia błon śluzowych obejmują światłowstręt, swędzenie spojówek, pieczenie i trudności w połykaniu.¹ Charakterystyczna wysypka często opisywana jest jako zlewne rumieniowe wykwity plamiste z purpurowymi środkami, które tworzą pęcherze i łuszczą się.¹

Reakcje polekowe mogą wystąpić podczas stosowania leku lub do dwóch tygodni po jego odstawieniu.¹ Typowy przebieg kliniczny SJS rozpoczyna się w ciągu 8 tygodni (zwykle 4-30 dni) po pierwszym kontakcie z czynnikiem przyczynowym.¹

Diagnostyka i rozpoznanie zespołu Stevens-Johnsona

Rozpoznanie zespołu Stevens-Johnsona jest przede wszystkim kliniczne i opiera się na dokładnym badaniu fizykalnym, wywiadzie medycznym oraz ocenie objawów. Kluczowym elementem diagnostyki jest wykluczenie innych możliwych przyczyn zmian pęcherzowych.¹²

Kryteria rozpoznania

Nie istnieją formalne kryteria diagnostyczne dla SJS, jednak rozpoznanie ustala się na podstawie kilku charakterystycznych cech:¹²

Obecność charakterystycznych zmian skórnych plamisto-rumieniowych o typie tarcz (zmiany tarczowate)
Zajęcie co najmniej dwóch błon śluzowych
Niedawna ekspozycja na lek
Odpowiadające cechy histopatologiczne w biopsji skóry

Klasyfikacja zespołu Stevens-Johnsona opiera się na procentowej powierzchni skóry objętej oddzieleniem naskórka:¹²

Zespół Stevens-Johnsona (SJS) – oddzielenie naskórka obejmuje mniej niż 10% powierzchni ciała
Toksyczna nekroliza naskórka (TEN) – oddzielenie naskórka obejmuje więcej niż 30% powierzchni ciała
Postać nakładająca się SJS/TEN – oddzielenie naskórka obejmuje 10-30% powierzchni ciała

Pomocny w diagnostyce SJS i TEN jest dodatni objaw Nikolskiego, polegający na oddzielaniu się naskórka pod wpływem bocznego nacisku na pozornie niezmienioną skórę.¹²

Biopsja skóry

Biopsja skóry, chociaż nie zawsze wymagana, jest przydatna do potwierdzenia diagnozy i wykluczenia innych możliwych przyczyn.¹² W przypadkach, gdy cechy kliniczne (np. zmiany tarczowate przechodzące w pęcherze, zajęcie oczu i błon śluzowych, objaw Nikolskiego, złuszczanie się płatów skóry) są niejednoznaczne, biopsja jest zalecana.¹

Typowe cechy histopatologiczne w zespole Stevens-Johnsona obejmują:¹²

Martwicę pełnej grubości naskórka
Apoptozę keratynocytów
Zmiany wakuolarne na granicy skórno-naskórkowej
Pęcherze podnaskórkowe
Łagodny naciek z komórek T CD8+

Bezpośrednie badanie immunofluorescencyjne biopsji skóry jest ujemne, co wskazuje, że choroba nie jest spowodowana odkładaniem się przeciwciał w skórze.¹²

W przypadku, gdy potrzebna jest szybka diagnoza, biopsja mrożeniowa może być nieocenionym narzędziem.¹² Ostatnie badania sugerują, że ex vivo konfokalna mikroskopia laserowa może służyć jako bezpieczna, szybka, nieinwazyjna alternatywa dla biopsji skóry, chociaż nie jest jeszcze powszechnie dostępna.¹

Badania laboratoryjne

Nie istnieją specyficzne badania laboratoryjne (poza biopsją), które mogłyby definitywnie ustalić diagnozę zespołu Stevens-Johnsona.¹² Jednak różne badania mogą być pomocne w ocenie ciężkości choroby, wykluczeniu innych stanów i monitorowaniu powikłań:¹²

Morfologia krwi z rozmazem – może wykazać prawidłową liczbę białych krwinek lub niespecyficzną leukocytozę, limfopenię, neutropenię, trombocytopenię
Biochemia – glukoza, elektrolity, magnez, fosforany, mocznik, kreatynina
Próby wątrobowe
Badanie białka C-reaktywnego (CRP)
Gazometria tętnicza i saturacja tlenem
Badania układu krzepnięcia

Posiewy skóry i krwi są zalecane, ponieważ częstość występowania poważnych zakażeń krwi i sepsy przyczynia się do zwiększonej chorobowości i śmiertelności.¹

Nieprawidłowe wyniki badań laboratoryjnych mogą obejmować podwyższony poziom białka ostrej fazy, hipoalbuminemię, łagodnie podwyższone poziomy enzymów wątrobowych, mikroskopową hematurię i łagodną leukocytozę.¹

Biomarkery w diagnostyce SJS

Nowe badania koncentrują się na biomarkerach dla diagnostyki i prognozowania SJS/TEN. Granulizyna jest mediatorem cytotoksycznym zaangażowanym w śmierć keratynocytów i jest wysoko wyrażana w płynie pęcherzowym i surowicy w SJS/TEN.¹ Podwyższone poziomy granulizyny w surowicy pobrane w pierwszych dniach wysypki polekowej mogą przewidywać rozwój SJS/TEN.¹ Granulizynę w surowicy można również wykorzystać jako predyktor rozwoju SJS/TEN 24 dni przed oddzieleniem skóry lub rozwojem zmian śluzówkowych.¹

W surowicy pacjentów z zespołem Stevens-Johnsona zazwyczaj podwyższone są poziomy następujących biomarkerów:¹

Czynnik martwicy nowotworów alfa (TNF-alfa)
Rozpuszczalny receptor interleukiny 2
Interleukina 6
Białko C-reaktywne (CRP)

Badacze odkryli również, że poziomy białka S100A8/A9 wykazują istotną korelację ze skalą SCORTEN, a poziomy S100A8/A9, G-CSF i EPO korelują ze śmiertelnością wewnątrzszpitalną.¹ Ponadto określone biomarkery surowicy, takie jak IFN-γ, sFasL, MPO i SCF, wykazały istotne powiązania z cechami histologicznymi SJS/TEN.¹

Badania obrazowe

W zależności od objawów, lekarz może zlecić badania obrazowe, takie jak rentgen klatki piersiowej, w celu sprawdzenia występowania zapalenia płuc.¹² Inne badania, które mogą być wskazane w zależności od zajęcia narządów wewnętrznych to bronchoskopia, ezofagogastroduodenoskopia (EGD) i kolonoskopia.¹

Ocena ciężkości choroby – SCORTEN

SCORTEN to punktowa skala oceny ciężkości choroby, która została opracowana do przewidywania śmiertelności w przypadkach SJS i TEN.¹ Jeden punkt przyznawany jest za każde z następujących siedmiu kryteriów przy przyjęciu:¹

Wiek > 40 lat
Obecność nowotworu złośliwego
Częstość akcji serca > 120/min
Początkowe oddzielenie naskórka > 10% powierzchni ciała
Stężenie mocznika w surowicy > 10 mmol/l (28 mg/dl)
Stężenie glukozy w surowicy > 14 mmol/l (252 mg/dl)
Stężenie dwuwęglanów w surowicy < 20 mmol/l

Ryzyko zgonu z powodu SJS/TEN zależy od uzyskanego wyniku:¹²

Wynik SCORTEN	Przewidywana śmiertelność
0-1	3,2%
2	12,1%
3	35,3%
4	58,3%
≥5	90%

Rozpoznanie różnicowe

Rozpoznanie różnicowe SJS obejmuje:¹²

Ostra uogólniona osutka krostkowa (AGEP)
Zespół nadwrażliwości polekowej (DHS/DRESS)
Rumień wielopostaciowy (zwłaszcza we wczesnym stadium reakcji)
Pęcherzyca zwykła
Pemfigoid pęcherzowy
Linijna dermatoza IgA
Pęcherzyca paraneoplastyczna
Uogólniona osutka polekowa o charakterze trwałym
Zespół oparzonej skóry wywołany gronkowcem (SSSS)

Odróżnienie rumienia wielopostaciowego większego (EMM) od SJS może być trudne. Oba charakteryzują się rozległą wysypką i zajęciem błon śluzowych. Jednak EMM zwykle prezentuje typowe zmiany tarczowate, głównie zajmując skórę, podczas gdy SJS manifestuje się rozległymi rumieniowymi plamami i pęcherzami, często prowadząc do poważnego złuszczania skóry i błon śluzowych, z wyższym ryzykiem powikłań.¹

Dodatkowo, w przypadku SJS zwykle zajęte są dwie lub więcej powierzchni śluzówkowych, a pacjenci często doświadczają objawów ogólnoustrojowych poprzedzających objawy skórne.¹

Określenie przyczyny reakcji

Identyfikacja czynnika wywołującego SJS jest kluczowa dla odpowiedniego postępowania. W przypadku pacjentów przyjmujących wiele leków znanych z wywoływania SJS/TEN, opracowano algorytm ALDEN do określenia prawdopodobnej przyczyny.¹

Dokładna chronologia kolejnych zdarzeń prowadzących do choroby oraz chronologia przyjmowania leków w miesiącach poprzedzających hospitalizację są istotne, z porównaniem informacji ze wszystkich możliwych źródeł (pacjent, rodzina, lekarz rodzinny, apteka itd.).¹

U dorosłych ponad 80% przypadków SJS jest związanych z lekami. Najczęściej odpowiedzialne leki to:¹²

Leki przeciwdrgawkowe (karbamazepina, fenytoina, fenobarbital, lamotrygina)
Antybiotyki (trimetoprim-sulfametoksazol, penicyliny)
Niesteroidowe leki przeciwzapalne (NLPZ)
Allopurynol
Leki chemioterapeutyczne

Istnieje również rosnące uznanie dla potencjalnych genetycznych predyspozycji do SJS/TEN. Badania HLA mogą odgrywać rolę w stratyfikacji ryzyka populacji pacjentów jako opcja przed rozpoczęciem leczenia lekami mogącymi wywołać SJS/TEN.¹²

Postępowanie i leczenie

Szybkie rozpoznanie SJS/TEN ma kluczowe znaczenie dla przerwania stosowania czynnika wywołującego, rozpoczęcia terapii wspomagającej i adjuwantowej oraz poprawy wyników leczenia.¹

Po postawieniu diagnozy pacjent powinien zostać natychmiast przyjęty do szpitala, najlepiej na oddział intensywnej terapii i/lub oddział oparzeń.¹² Podstawowe zasady leczenia obejmują:¹

Jak najszybsze przerwanie stosowania podejrzanego leku (szacuje się, że śmiertelność zmniejsza się o 30% dziennie, gdy lek wywołujący reakcję zostanie wcześnie odstawiony)
Jeśli nie można zidentyfikować leku wywołującego, należy rozważyć przerwanie stosowania WSZYSTKICH leków

Leczenie zespołu Stevens-Johnsona obejmuje:¹

Przerwanie stosowania leku, który spowodował problem
Stosowanie żywności wysokoenergetycznej, ewentualnie przez sondę, w celu wspomagania gojenia
Stosowanie antybiotyków w razie potrzeby, aby zapobiec infekcji
Podawanie leków przeciwbólowych
Leczenie w szpitalu, możliwe nawet na oddziale intensywnej terapii lub oddziale oparzeń
Korzystanie z zespołów specjalistycznych z dermatologii i okulistyki (jeśli dotknięte są oczy)
W niektórych przypadkach leczenie dożylnymi immunoglobulinami, cyklosporyną, dożylnymi steroidami lub przeszczepami błony owodniowej (dla oczu)

Podstawą leczenia pozostaje zoptymalizowana opieka wspomagająca.¹ Wysokie ryzyko infekcji uzasadnia powtarzane pobieranie próbek skóry do analiz bakteriologicznych.¹ Ocena i leczenie bólu jest priorytetem w fazie ostrej, szczególnie podczas zabiegów pielęgnacyjnych, które są wykonywane kilka razy dziennie.¹

U pacjentów z SJS/TEN zalecana jest początkowa objętość płynów dożylnych wynosząca 2 ml/kg masy ciała pomnożona przez % powierzchni ciała z oddzieleniem naskórka w pierwszych 24 godzinach.¹

Należy mieć wysokie podejrzenie dotyczące wtórnych infekcji, które najczęściej są spowodowane przez S. aureus i Pseudomonas aeruginosa. Chociaż nie zaleca się profilaktycznego stosowania antybiotyków, posiewy skóry mogą pomóc w ukierunkowaniu terapii.¹

Badania wykazały, że niestety nie było korzyści w przeżywalności u osób leczonych ogólnoustrojowymi kortykosteroidami w porównaniu do osób leczonych zwykłą opieką.¹ Obecnie nie ma konsensusu dotyczącego stosowania różnych terapii immunomodulujących w ostrym leczeniu SJS.¹

Rokowanie i monitorowanie

Rokowanie zależy od kilku czynników, w tym zajętej powierzchni ciała, chorób współistniejących, wieku i kilku oznak choroby ogólnoustrojowej w momencie przyjęcia.¹ Ogólna śmiertelność waha się od 1 do 9% w SJS i od 25 do 35% w TEN.¹

Prognozy są związane z szybką identyfikacją leku przyczynowego i jego odstawieniem. Kluczowe jest szybkie ustalenie właściwej diagnozy klinicznej, aby można było przerwać stosowanie leku powodującego i rozpocząć odpowiednie leczenie.¹

Ponieważ SJS/TEN może początkowo występować z łagodnymi objawami we wczesnej fazie choroby, ale stan postępuje przez kilka dni, istotne jest ścisłe monitorowanie i regularna ponowna ocena.¹

W przypadku pacjentów, którzy otrzymują diagnozę SJS, kluczowe znaczenie ma leczenie dla rokowania. Im wcześniej można uzyskać odpowiednią opiekę, tym lepiej można zarządzać potencjalnymi powikłaniami, które mogą pojawić się wraz z postępem choroby.¹

Wnioski i podsumowanie

Zespół Stevens-Johnsona jest rzadką, ale potencjalnie śmiertelną reakcją skórną, która wymaga szybkiego rozpoznania i leczenia. Diagnostyka opiera się głównie na badaniu klinicznym, dokładnym wywiadzie medycznym oraz biopsji skóry, która potwierdza diagnozę poprzez ukazanie martwicy naskórka.

Kluczowe znaczenie dla wyników leczenia ma szybka identyfikacja i odstawienie leku wywołującego oraz natychmiastowe rozpoczęcie leczenia wspomagającego. Pacjenci powinni być leczeni w specjalistycznych ośrodkach, najlepiej na oddziałach oparzeń lub intensywnej terapii, aby zapewnić optymalne zarządzanie tą złożoną i potencjalnie zagrażającą życiu chorobą.

Postępy w badaniach biomarkerów i ocenie genetycznych predyspozycji mogą w przyszłości przyczynić się do poprawy diagnostyki i profilaktyki SJS/TEN, szczególnie w przypadku pacjentów, którzy muszą przyjmować leki znane z wysokiego ryzyka wywoływania tych ciężkich reakcji skórnych.

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Materiały źródłowe

#1 Stevens-Johnson Syndrome: A Perplexing Diagnosis
https://pmc.ncbi.nlm.nih.gov/articles/PMC7176330/
Stevens-Johnson syndrome/toxic epidermal necrolysis is a spectrum of mucocutaneous reactions that can occur due to drug reactions, infections with Mycoplasma pneumonia, human immunodeficiency virus (HIV), cancer, and genetics. […] This case emphasizes the importance of pattern recognition of Stevens-Johnson syndrome, as this is a rare but serious condition that must be recognized and treated appropriately. The reaction is most commonly due to medications; however, a thorough history and physical exam are vital to diagnosing this potentially fatal condition. […] A definitive diagnosis of Stevens-Johnson syndrome/toxic epidermal necrolysis is made by skin biopsy showing full-thickness dermal necrosis in the absence of immunoglobulin deposition. […] This case shows the importance of pattern recognition in Steven Johnson’s syndrome which must be recognized and treated appropriately as it is a rare but serious condition. The reaction is most commonly due to medications; however, a thorough history and physical exam is vital in diagnosing this potentially fatal condition.
#1 Stevens-Johnson Syndrome (SJS) and Toxic Epidermal Necrolysis (TEN) â Diagnosis : Emergency Care BC
https://emergencycarebc.ca/clinical_resource/clinical-summary/stevens-johnson-syndrome-sjs-and-toxic-epidermal-necrolysis-ten-diagnosis/
Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) are severe life-threatening Type IV hypersensitivity mucocutaneous reactions characterized by extensive skin detachment and necrosis. […] Emergency physicians have an important role to play in the early detection of SJS/TEN, as early detection and withdrawal of the offending agent greatly improves mortality/morbidity. […] There are no formal diagnostic criteria for SJS/TEN. […] SJS may start as a febrile illness with malaise, headache, cough, and/or rhinorrhea 1-3 days before skin lesions appear. […] Signs of early mucosal involvement include photophobia, conjunctival itching, burning, and dysphagia. […] Physical examination should include: Approximation of the total affected BSA via the rule of nines (Parkland Formula for Burns). […] Investigations to order: CBC + differential, glucose, extended electrolytes, BUN, Creatinine, total protein, albumin, alkaline phosphatase, ALT, AST, ESR or CRP. […] Skin biopsy for Histopathologic analysis should be obtained via a large punch biopsy or deep shave biopsy.
#1 Stevens-Johnson Syndrome and Toxic Epidermal Necrolysis Mimics â Differential Diagnosis and Initial Management – emDocs
https://www.emdocs.net/stevens-johnson-syndrome-and-toxic-epidermal-necrolysis-mimics-differential-diagnosis-and-initial-management/
Stevens-Johnson syndrome (SJS) is a life-threatening skin condition that exists on a spectrum of severity. It begins with a prodrome of high-grade fever, flu like symptoms, skin tenderness and blistering. The characteristic rash is commonly described as a confluent erythematous macular rash with purpuric centers that blister and peel. SJS involves at least 10% body surface area (BSA); toxic epidermal necrolysis (TEN) is diagnosed if the rash involves at least 30% BSA. The hallmark mucosal involvement can result in symptoms such as conjunctival itching and pain with swallowing. The rash typically begins on the torso and face and spreads to the rest of the body, usually sparing the palms and soles. This rash is importantly Nikolsky sign positive, where lateral pressure on intact skin causes shedding of the epidermis (1). This is an important distinguishing feature in differentiating SJS/TEN from the other life-threatening rashes discusses later.
#1 Stevens-Johnson syndrome – Symptoms & causes – Mayo Clinic
https://www.mayoclinic.org/diseases-conditions/stevens-johnson-syndrome/symptoms-causes/syc-20355936
Stevens-Johnson syndrome requires immediate medical attention. Seek emergency medical care if you experience signs and symptoms of this condition. Drug-induced reactions may occur during the use of a medication or up to two weeks after discontinuing it. […] Your health care provider may not be able to identify its exact cause, but usually the condition is triggered by medication, an infection or both. You may react to medication while you’re using it or up to two weeks after you’ve stopped using it. […] Associated Procedures include Skin biopsy.
#1 Stevens-Johnson Syndrome: What a Pharmacist Should Know
https://www.uspharmacist.com/article/stevens-johnson-syndrome-what-a-pharmacist-should-know-41888
Stevens-Johnson syndrome (SJS) is a rare, life-threatening mucocutaneous reaction that is often drug-induced. […] Despite reports of more than 100 drugs being taken in patients who develop SJS/TEN, a few classes such as antibiotics and anticonvulsants are thought to carry the highest risk. […] The early recognition of severe cutaneous reactions is especially important to reduce fatal outcomes. […] The typical clinical course of SJS begins within 8 weeks (usually 4 to 30 days) following the first exposure to the causative agent. […] Prompt identification and removal of the highly suspected agent is vital. […] If a causative agent is identified, rechallenge with that medication is not recommended, and if unintentionally done may cause a rapid recurrence of symptoms. […] Prompt initiation of appropriate treatment can potentially reduce the morbidity and mortality associated with SJS. […] Currently there is no consensus on the use of various immunomodulating therapies for acute treatment of SJS.
#1 Stevens-Johnson syndrome – Diagnosis & treatment – Mayo Clinic
https://www.mayoclinic.org/diseases-conditions/stevens-johnson-syndrome/diagnosis-treatment/drc-20355942
Tests and procedures used to diagnose Stevens-Johnson syndrome include: […] Health care providers often can identify Stevens-Johnson syndrome based on your medical history, including a review of your current and recently stopped medications, and a physical exam. […] To confirm the diagnosis, and rule out other possible causes, your health care provider removes a sample of skin for laboratory testing (biopsy). […] To rule out an infection, your health care provider takes a sample of skin, tissue or fluid for laboratory testing (culture). […] Depending on your symptoms, your health care provider may have you undergo an imaging such as a chest X-ray to check for pneumonia. […] These are used to confirm infection or other possible causes.
#1
https://link.springer.com/article/10.1007/s40257-024-00889-6
Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) are the most severe cutaneous adverse reactions that are typically drug-induced in adults. […] Although many cases of SJS/TEN have been reported in the literature, there is no consensus regarding diagnostic criteria or treatment. […] This review is intended to provide physicians with a comprehensive but practical SJS/TEN roadmap to guide diagnosis and management. […] There are no standard diagnostic criteria for SJS/TEN, though the presence of macular targetoid lesions, involvement of two mucous membranes, recent drug exposure, and corresponding histopathology are all suggestive. […] Histologic features of SJS/TEN include full thickness epidermal necrosis, keratinocyte apoptosis, basal vacuolar change, subepidermal bullae, subepidermal clefting, and mild T cell infiltrate.
#1 Stevens-Johnson syndrome and toxic epidermal necrolysis: Management, prognosis, and long-term sequelae – UpToDate
https://www.uptodate.com/contents/stevens-johnson-syndrome-and-toxic-epidermal-necrolysis-management-prognosis-and-long-term-sequelae
Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) are severe, mucocutaneous, adverse reactions in most cases induced by medications. Clinical features include fever, prodromal symptoms, mucositis, and extensive epidermal detachment. SJS and TEN are considered a disease continuum and are collectively known as epidermal necrolysis. […] The classification of the disease is based on the total body surface area (BSA) involved with skin detachment: SJS is defined as skin detachment affecting <10 percent of the BSA. TEN is defined as skin detachment affecting >30 percent of the BSA. SJS/TEN overlap is defined as skin detachment involving 10 to 30 percent of the BSA.
#1 StevensâJohnson syndrome – Wikipedia
https://en.wikipedia.org/wiki/Stevens%E2%80%93Johnson_syndrome
The diagnosis of Stevens-Johnson syndrome is based on involvement of less than 10% of the skin. […] It is known as TEN when more than 30% of the skin is involved and considered an intermediate form when 10-30% is involved. […] A positive Nikolsky’s sign is helpful in the diagnosis of SJS and TEN. […] A skin biopsy is helpful, but not required, to establish a diagnosis of SJS and TEN.
#1 Stevens-Johnson Syndrome (SJS) and Toxic Epidermal Necrolysis (TEN) – Dermatologic Disorders – Merck Manual Professional Edition
https://www.merckmanuals.com/professional/dermatologic-disorders/hypersensitivity-and-reactive-skin-disorders/stevens-johnson-syndrome-sjs-and-toxic-epidermal-necrolysis-ten
Diagnosis is usually obvious by appearance of initial lesions and clinical syndrome. […] Diagnosis is often obvious from appearance of lesions and rapid progression of symptoms. Histologic examination of sloughed skin shows necrotic epithelium, a distinguishing feature. […] Confirm the diagnosis by biopsy (showing necrotic epithelium) if clinical characteristics (eg, target lesions progressing to bullae, ocular and mucous membrane involvement, Nikolsky sign, desquamation in sheets) are inconclusive.
#1 Stevens-Johnson Syndrome Workup: Approach Considerations, Histologic Findings
https://emedicine.medscape.com/article/1197450-workup
Bronchoscopy, esophagogastroduodenoscopy (EGD), and colonoscopy may be indicated. […] Minimal dermal inflammatory cell infiltrate and full-thickness necrosis of epidermis are typical histopathologic findings in patients with Stevens-Johnson syndrome. […] The epidermal-dermal junction shows changes, ranging from vacuolar alteration to subepidermal blisters. […] CD8+ T cells that recognize major histocompatibility complex I (MHC-I) modified by an antigen may produce skin lesions of Stevens-Johnson syndrome, or they may be produced by T cells that recognize an antigen that is restricted by MHC-I. […] Conjunctival biopsies from patients with active ocular disease show subepithelial plasma cells and lymphocyte infiltration. […] Immunohistology of the conjunctiva reveals numerous HLA-DRpositive cells in the substantia propria, vessel walls, and epithelium. […] In vivo confocal microscopy may be a useful tool for therapeutic indications and follow-up of ocular problems associated with Stevens-Johnson syndrome.
#1 Stevens Johnson Syndrome / Toxic Epidermal Necrolysis. SJS/TEN
https://dermnetnz.org/topics/stevens-johnson-syndrome-toxic-epidermal-necrolysis
SJS/TEN is suspected clinically and classified based on the skin surface area detached at maximum extent. […] The diagnosis may, therefore, change during the first few days in the hospital. […] If the test is available, elevated levels of serum granulysin taken in the first few days of a drug eruption may be predictive of SJS/TEN. […] Skin biopsy is usually required to confirm the clinical diagnosis and to exclude staphylococcal scalded skin syndrome (SSSS) and other generalised rashes with blisters. […] The direct immunofluorescence test on the skin biopsy is negative, indicating the disease is not due to deposition of antibodies in the skin. […] Blood tests do not help to make the diagnosis but are essential to make sure fluid and vital nutrients have been replaced, to identify complications and to assess prognostic factors.
#1 Stevens Johnson Syndrome/Toxic Epidermal Necrolysis – Dermatology Advisor
https://www.dermatologyadvisor.com/home/decision-support-in-medicine/infectious-diseases/stevens-johnson-syndrome-toxic-epidermal-necrolysis/
Stevens-Johnson Syndrome (SJS) and toxic epidermal necrolysis (TEN) are severe reactive blistering diseases along a continuum of disease severity. […] The diagnosis of TEN is most commonly made clinically. However, in cases where the diagnosis is unclear for either SJS or TEN, skin biopsy may be helpful. When the diagnosis is needed quickly, frozen sections can be invaluable. […] Key principles of therapy: Discontinued offending agent as soon as possible (estimated reduced mortality by 30% per day when the offending drug is stopped early). […] If the culprit drug cannot be identified, stopping ALL medications should strongly be considered. […] The prognosis depends on several factors including BSA involved, comorbidities, age, and several signs of systemic disease at the time of admission. […] Overall mortality ranges from 1 to 9% in SJS and 25 to 35% in TEN.
#1
https://link.springer.com/article/10.1007/s40257-024-00889-6
New research has supported that ex vivo confocal laser scanning microscopy may serve as a safe, rapid, non-invasive alternative to skin biopsy, though this is not yet widely available. […] Much recent research has focused on biomarkers for SJS/TEN diagnosis and prognostication. Granulysin is a cytotoxic mediator involved in keratinocyte death and is highly expressed in SJS/TEN blister fluid and serum. […] Serum granulysin may also be used as a predictor of SJS/TEN development 24 days prior to skin detachment or development of mucosal lesions. […] HLA testing has a role in risk stratification of patient populations as an option prior to starting SJS/TEN culprit drugs.
#1 Stevens-Johnson Syndrome Workup: Approach Considerations, Histologic Findings
https://emedicine.medscape.com/article/1197450-workup
There are no specific laboratory studies (other than biopsy) that can definitively establish the diagnosis of Stevens-Johnson syndrome. […] Serum levels of the following typically are elevated in patients with Stevens-Johnson syndrome: Tumor necrosis factor (TNF)-alpha, Soluble interleukin 2-receptor, Interleukin 6, C-reactive protein. […] However, none of these serologic tests is used routinely in diagnosing and managing Stevens-Johnson syndrome. […] A complete blood count (CBC) may reveal a normal white blood cell (WBC) count or a nonspecific leukocytosis. […] Skin and blood cultures have been advocated because the incidence of serious bacterial bloodstream infections and sepsis contribute to morbidity and mortality. […] Skin biopsy specimens demonstrate that the bullae are subepidermal.
#1 Stevens-Johnson syndrome and toxic epidermal necrolysis – Symptoms, diagnosis and treatment | BMJ Best Practice US
https://bestpractice.bmj.com/topics/en-us/237?locale=th
1st tests to order include skin biopsy, blood cultures, complete blood count, glucose, magnesium, phosphate, blood urea nitrogen, bicarbonate, serum electrolytes, serum creatinine, liver function tests, C-reactive protein (CRP), arterial blood gases and saturation of oxygen, chest x-ray, coagulation studies, and skin swab from lesional skin. […] Investigations to avoid include erythrocyte sedimentation rate (ESR). […] Tests to consider include direct immunofluorescence.
#1 Stevens-Johnson Syndrome
https://www.contemporarypediatrics.com/view/stevens-johnson-syndrome
A 13-year-old boy was brought to the emergency department (ED) with a generalized itchy rash of 2 days’ duration. […] The ophthalmology consultant diagnosed mucopurulent conjunctivitis with episcleritis and recommended lubrication with artificial tears and erythromycin ophthalmic ointment. […] A skin biopsy is indicated only when the diagnosis is uncertain. Histopathological findings include a mononuclear perivascular cell infiltrate in the dermis, basal layer edema, subepidermal blister formation, and epidermal cell necrosis. […] SJS is usually a clinical diagnosis. Abnormal laboratory findings may include an elevated sedimentation rate, hypoalbuminemia (as in this patient), mildly elevated liver enzyme levels, microscopic hematuria, and mild leukocytosis. […] The most common complication is sepsis (most often caused by Staphylococcus aureus followed by Pseudomonas aeruginosa). […] Diagnosis should prompt immediate discontinuation of all potential causative drugs.
#1 Correlations between histopathologic findings, serum biomarker levels, and clinical outcomes in StevensâJohnson syndrome/toxic epidermal necrolysis (SJS/TEN) | Scientific Reports
https://www.nature.com/articles/s41598-023-40812-3
Additionally, serum MPO levels exhibited a significant association with the degree of epidermal necrosis. […] S100A8/A9 levels showed a significant correlation with the SCORTEN, and S100A8/A9, G-CSF, and EPO levels correlated with in-hospital mortality rate. […] Specific serum biomarkers, such as IFN-, sFasL, MPO, and SCF, exhibited significant associations with the histologic characteristics of SJS/TEN.
#1 Stevens Johnson Syndrome / Toxic Epidermal Necrolysis. SJS/TEN
https://dermnetnz.org/topics/stevens-johnson-syndrome-toxic-epidermal-necrolysis
In vitro diagnostic tests for drug allergies including SJS/TEN are under investigation. […] Patch testing rarely identifies the culprit in SJS/TEN, and is not recommended. […] SCORTEN is an illness severity score that has been developed to predict mortality in SJS and TEN cases. […] The risk of dying from SJS/TEN depends on the score. […] The category cannot always be defined with certainty on initial presentation.
#1 Stevens-Johnson Syndrome – StatPearls – NCBI Bookshelf
https://www.ncbi.nlm.nih.gov/books/NBK459323/
Stevens-Johnson syndrome and toxic epidermal necrolysis are acute, rare, and potentially fatal skin reactions involving loss of skin and, in some cases, mucosal membranes accompanied by systemic symptoms. Medications are causative in over 80 percent of cases. […] This activity illustrates the evaluation and treatment of Stevens-Johnson syndrome and of toxic epidermal necrolysis and reviews the role of the interprofessional team in managing those with these conditions. […] Summarize differential diagnoses of Stevens-Johnson syndrome and of toxic epidermal necrolysis. […] The severity of Stevens-Johnson syndrome/toxic epidermal necrolysis is assessed using SCORTEN. One point is scored for each of the following seven criteria at admission. […] The risk of dying from Stevens-Johnson syndrome/toxic epidermal necrolysis depends on the score.
#1 How Doctors Diagnose Stevens-Johnson Syndrome (SJS) | Dunn Sheehan, LLP
https://www.dunnsheehan.com/news-insights/how-is-stevens-johnson-syndrome-diagnosed
Understanding the diagnosis process can help you advocate for yourself or a loved one if symptoms arise. […] Stevens-Johnson Syndrome can be initially mistaken for acute generalized exanthematous pustulosis (AGEP) and drug hypersensitivity syndrome (DHS), which are also severe cutaneous adverse reactions to drugs. SJS could also be confused with erythema multiforme early in the progression of the reaction. Further medical investigations aid in accurate diagnosis.
#1
https://eurekaselect.com/public/article/133845
Both Stevens-johnson syndrome (SJS) and Toxic-epidermal necrolysis (TEN) are generally medication-induced pathological conditions that mostly affect the epidermis and mucus membranes. […] The diagnosis of SJS is made mostly on the gross observations of clinical symptoms, and confirmed by the histopathological examination of dermal biopsies of the patients. […] The differential diagnoses consist of the exclusion of Pemphigus vulgaris, bullous pemphigoid, linear IgA dermatosis, paraneoplastic pemphigus, disseminated fixed bullous drug eruption, acute generalized exanthematous pustulosis (AGEP), and staphylococcal scalded skin syndrome (SSSS). […] Urgent medical attention is needed for early diagnosis, estimation of the SCORTEN prognosis, identification and discontinuation of the causative agent as well as highdose injectable Ig therapeutic interventions along with specialized supportive care.
#1 Erythema multiforme major/Stevens-Johnson syndrome: a diagnostic challenge | Anales de PediatrÃa
https://www.analesdepediatria.org/en-erythema-multiforme-major-stevens-johnson-syndrome-diagnostic-articulo-S2341287924002643
A male adolescent aged 14 years presented with fever, conjunctivitis, sore throat, oral ulcers, swollen lips and rash with onset 5 days prior. […] At this point, Stevens-Johnson syndrome (SJS) or erythema multiforme major (EMM) were suspected. […] The findings of the skin biopsy supported the diagnosis of SJS. […] Distinguishing between EMM and SJS is challenging. Both are characterized by widespread rash and mucous membrane involvement. However, EMM usually presents with typical target lesions mainly involving the skin, while SJS manifests with widespread erythematous macules and blisters, often leading to severe skin and mucous membrane detachment, with a higher risk of complications. […] In addition, in the case of SJS, two or more mucosal surfaces are usually involved and the patients tend to experience systemic symptoms preceding the cutaneous manifestations. […] Understanding these nuances is essential for accurate diagnosis and timely intervention.
#1 Stevens-Johnson Syndrome – StatPearls – NCBI Bookshelf
https://www.ncbi.nlm.nih.gov/books/NBK459323/
In patients on multiple drugs known to cause Stevens-Johnson syndrome/toxic epidermal necrolysis, the algorithm ALDEN has been developed to determine the likely cause. […] Patients should undergo interprofessional assessment in a specialized hospital environment. […] Care of a patient with Stevens-Johnson syndrome/toxic epidermal necrolysis requires supportive care, including cessation of the suspected causative drug(s) and hospital admission: preferably to an intensive care and/or burn unit. […] The management of SJS is interprofessional. A number of specialists are usually involved in the care of these patients, including a dermatologist, intensivist, ophthalmologist, pulmonologist, nephrologist, plastic surgeon, and gastroenterologist, functioning as an interprofessional team. […] The outcomes of patients with SJS depend on the extent and severity of skin involvement.
#1 Epidermal necrolysis French national diagnosis and care protocol (PNDS; protocole national de diagnostic et de soins) | Orphanet Journal of Rare Diseases | Full Text
https://ojrd.biomedcentral.com/articles/10.1186/s13023-018-0793-7
Diagnosis is essentially clinical. […] The presence of at least three of these clinical criteria renders the diagnosis of EN probable. […] Diagnosis cannot be established on the basis of laboratory tests or imaging. […] A skin biopsy, with pathology examination and direct immunofluorescence analysis, is required to confirm the diagnosis and to rule out other bullous diseases with a similar clinical presentation (such as linear IgA bullous dermatosis). […] It is essential to note the precise chronology of the successive events leading to the disease and the chronology of drug intake during the months preceding hospitalization, comparing information from all possible sources (patient, family, family doctor, pharmacy etc.). […] The suspect drug(s) should be withdrawn promptly, as this has been shown to improve prognosis.
#1 Stevens-Johnson Syndrome and Toxic Epidermal Necrolysis Mimics â Differential Diagnosis and Initial Management – emDocs
https://www.emdocs.net/stevens-johnson-syndrome-and-toxic-epidermal-necrolysis-mimics-differential-diagnosis-and-initial-management/
Risk factors for developing SJS/TEN include patients with active cancer, HIV, women, and taking many medications. The common medication offenders include trimethoprim-sulfamethoxazole, phenobarbital, carbamazepine, lamotrigine, acetaminophen, and chemotherapeutic agents. Penicillins are cited as the most common antibiotic agent to cause SJS/TEN. It is unclear if this is due to how frequently penicillins are prescribed or if the antibiotic was being used to treat an early skin manifestation of SJS as a bacterial infection (3). Therefore, a careful history and timeline should be taken to assess the true trigger. […] The mainstay of SJS/TEN treatment is supportive care. First, stop the offending agent. Local wound care, pain control, and IV fluids should be continued through the course of the illness, much like you would treat a burn patient (3,4). A retrospective analysis in 2010 recommended an initial IV fluid volume of 2 ml/kg body weight multiplied by the % body surface area of epidermal detachment in the first 24 hours (5). Have a high clinical suspicion for secondary infections which are most commonly due to S. aureus and Pseudomonas aeruginosa. Although prophylactic antibiotics are not recommended, skin cultures can help guide therapy (6). Studies have shown that there unfortunately was no survival benefit to those treated with systemic corticosteroids when compared to those treated with usual care (7). A useful decision tool in determining the clinical setting best suited for management of your patient is the SCORTEN (seen below) scale which can assess severity of disease; those with a score greater than equal to 2 are recommended for ICU or Burn center admission (8).
#1 StevensâJohnson Syndrome and Toxic Epidermal Necrolysis: A Review of Diagnosis and Management
https://www.mdpi.com/1648-9144/57/9/895
StevensâJohnson Syndrome (SJS) and Toxic Epidermal Necrolysis (TEN) are rare diseases that are characterized by widespread epidermal necrosis and sloughing of skin. They are associated with significant morbidity and mortality, and early diagnosis and treatment is critical in achieving favorable outcomes for patients. […] Rapid diagnosis of SJS/TEN is critical in order to discontinue the offending agent, begin supportive and adjunctive therapies, and improve outcomes. However, the clinical presentation can be similar to a number of other blistering disorders, and diagnosis is not always straightforward. Given that the diagnosis of SJS/TEN is time sensitive, frozen sections can be utilized for more rapid decision making. […] There are a number of studies that have investigated potential diagnostic markers of the disease, with early studies focusing on the role of granulysin.
#1 Stevens-Johnson Syndrome: Causes, Diagnosis, and Treatment
https://www.healthline.com/health/stevens-johnson-syndrome
If you receive a diagnosis of SJS, you’ll immediately be admitted to a hospital, potentially the intensive care unit (ICU), a specialized burn unit, or dermatology unit. […] There is no specific test or criteria to diagnose SJS. […] A doctor, often a specialist like a dermatologist, will diagnose your condition based on your medical history and the symptoms you’re experiencing. […] Doctors may take a skin sample, called a biopsy, or perform other diagnostic tests to help confirm SJS and rule out other conditions.
#1 Stevens-Johnson Syndrome (SJS): Causes, Rash & Treatments
https://my.clevelandclinic.org/health/diseases/17656-stevens-johnson-syndrome
How is Stevens-Johnson syndrome (SJS) diagnosed? […] Healthcare providers diagnose SJS and TEN: […] By taking a skin biopsy. […] How is Stevens-Johnson syndrome treated? […] Treatments for Stevens-Johnson syndrome include: […] Stopping the medication that has caused the problem. […] Using high-calorie food, possibly by tube-feeding, to promote healing. […] Using antibiotics when needed to prevent infection. […] Providing pain relief medications. […] Treating you in a hospital, possibly even in an intensive care or burn unit. […] Using specialist teams from dermatology and ophthalmology (if your eyes are affected). […] In some cases, treating you with IV immunoglobulin, cyclosporine, IV steroids, or amniotic membrane grafts (for your eyes).
#1 Epidermal necrolysis French national diagnosis and care protocol (PNDS; protocole national de diagnostic et de soins) | Orphanet Journal of Rare Diseases | Full Text
https://ojrd.biomedcentral.com/articles/10.1186/s13023-018-0793-7
In cases of doubtful causality, the Reference Center should be contacted urgently. […] The classification of EN depends on the maximum affected area of the body (detached/detachable area). […] The SCORTEN score is used, at admission, to evaluate the risk of death on the basis of seven clinical and biological parameters. […] It is important to provide the patient and his/her family with information about the disease and its severity, upon arrival at the specialized unit. […] Treatment is essentially supportive, as no effective etiological treatment has been identified. […] All suspect drugs should be stopped as soon as possible, but the continuity of management should be ensured. […] The complete drug notoriety list is available from the Regiscar website. […] The high risk of infection justifies the repeated collection of skin specimens for bacteriological analyses. […] The evaluation and treatment of pain is a priority in acute phase management, particularly during wound care, which is performed several times daily. […] The main treatment remains optimized supportive care.
#1 SciELO Brazil – Stevens-Johnson syndrome and toxic epidermal necrolysis: a review Stevens-Johnson syndrome and toxic epidermal necrolysis: a review
https://www.scielo.br/j/ramb/a/D75h5BfLmz5Tw5JnMTXtPjF/
Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) are uncommon, acute and potentially life-threatening adverse cutaneous drug reactions. […] Early diagnosis of the disease, recognition of the causal agent and the immediate withdrawal of the drug are the most important actions, as the course of the disease is often rapid and fatal. […] There are no laboratory tests to point out the drug causing the disorder and, therefore, diagnosis is clinical. […] A good history, with emphasis on the use of drugs and the occurrence of previous infections, and thorough physical examination are essential. […] Drug provocation tests are contraindicated, since a subsequent exposure to the agent could trigger a new severe episode of SJS/TEN. […] Skin biopsy is an additional final examination and reveals necrosis in all layers of the epidermis caused by apoptosis of keratinocytes and epidermal detachment, while the dermis displays minimum inflammatory changes. […] Prognosis is linked to rapid identification of the causative drug and its discontinuation. It is crucial to quickly establish proper clinical diagnosis, so that the causative drug may be discontinued and appropriate treatment initiated.
#1 Stevens-Johnson syndrome and toxic epidermal necrolysis – Symptoms, diagnosis and treatment | BMJ Best Practice US
https://bestpractice.bmj.com/topics/en-us/237
Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) are severe skin detachments with mucocutaneous complications associated with a history of medication use, most commonly anticonvulsants, antibiotics, and nonsteroidal anti-inflammatory drugs. […] SJS/TEN may present with mild symptoms in early disease, but the condition progresses over several days; close monitoring and regular reassessment is essential. […] Once a diagnosis is established, the causative agent should be identified and withdrawn; the patient should receive skin care and supportive management. […] Transfer to a burn center, a specialized wound care center, or a dermatology intensive care unit is recommended for patients diagnosed with SJS/TEN. […] Patients at risk may need human leukocyte antigen screening if they are to receive medications such as carbamazepine, allopurinol, or trimethoprim/sulfamethoxazole.
#1 Why Early Stevens-Johnson Syndrome Treatment Matters | Dunn Sheehan, LLP
https://www.dunnsheehan.com/news-insights/the-importance-of-early-diagnosis-in-stevens-johnson-syndrome-treatment-and-recovery
When you are diagnosed with Stevens-Johnson syndrome, treatment is crucial to your prognosis. The sooner you can obtain appropriate care, the better you and your doctor can manage potential complications that can arise as the condition progresses. […] Stevens-Johnson syndrome leads to a rash and the formation of blisters that break open, causing the upper layers of your skin to peel. […] Treating SJS begins with stopping the medication that initially caused the condition. Other symptoms associated with SJS can be treated with antibiotics, painkillers, and other drugs. The effectiveness of these interventions can increase the earlier your doctor introduces them. […] Symptoms of SJS can begin appearing as quickly as one day to several weeks after starting a new medication. Initially, you may experience flu-like symptoms, including fever, body aches, and a sore throat.
#2 Stevens-Johnson Syndrome: Practice Essentials, Background, Pathophysiology
https://emedicine.medscape.com/article/1197450-overview
Minimal dermal inflammatory cell infiltrate and full-thickness necrosis of the epidermis are typical histopathologic findings in patients with Stevens-Johnson syndrome. Histopathologic examination of the skin can also reveal the following: […] Ocular examination can demonstrate the following: […] There are no specific laboratory studies (other than biopsy) that can definitively establish the diagnosis of Stevens-Johnson syndrome (see Clinical and Workup).
#2
https://link.springer.com/article/10.1007/s40257-024-00889-6
Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) are the most severe cutaneous adverse reactions that are typically drug-induced in adults. […] Although many cases of SJS/TEN have been reported in the literature, there is no consensus regarding diagnostic criteria or treatment. […] This review is intended to provide physicians with a comprehensive but practical SJS/TEN roadmap to guide diagnosis and management. […] There are no standard diagnostic criteria for SJS/TEN, though the presence of macular targetoid lesions, involvement of two mucous membranes, recent drug exposure, and corresponding histopathology are all suggestive. […] Histologic features of SJS/TEN include full thickness epidermal necrosis, keratinocyte apoptosis, basal vacuolar change, subepidermal bullae, subepidermal clefting, and mild T cell infiltrate.
#2 StevensâJohnson syndrome – Wikipedia
https://en.wikipedia.org/wiki/Stevens%E2%80%93Johnson_syndrome
The diagnosis of Stevens-Johnson syndrome is based on involvement of less than 10% of the skin. […] It is known as TEN when more than 30% of the skin is involved and considered an intermediate form when 10-30% is involved. […] A positive Nikolsky’s sign is helpful in the diagnosis of SJS and TEN. […] A skin biopsy is helpful, but not required, to establish a diagnosis of SJS and TEN.
#2 Stevens-Johnson Syndrome and Toxic Epidermal Necrolysis Mimics â Differential Diagnosis and Initial Management – emDocs
https://www.emdocs.net/stevens-johnson-syndrome-and-toxic-epidermal-necrolysis-mimics-differential-diagnosis-and-initial-management/
Stevens-Johnson syndrome (SJS) is a life-threatening skin condition that exists on a spectrum of severity. It begins with a prodrome of high-grade fever, flu like symptoms, skin tenderness and blistering. The characteristic rash is commonly described as a confluent erythematous macular rash with purpuric centers that blister and peel. SJS involves at least 10% body surface area (BSA); toxic epidermal necrolysis (TEN) is diagnosed if the rash involves at least 30% BSA. The hallmark mucosal involvement can result in symptoms such as conjunctival itching and pain with swallowing. The rash typically begins on the torso and face and spreads to the rest of the body, usually sparing the palms and soles. This rash is importantly Nikolsky sign positive, where lateral pressure on intact skin causes shedding of the epidermis (1). This is an important distinguishing feature in differentiating SJS/TEN from the other life-threatening rashes discusses later.
#2 Stevens-Johnson Syndrome (SJS) and Toxic Epidermal Necrolysis (TEN) – Skin Disorders – Merck Manual Consumer Version
https://www.merckmanuals.com/home/skin-disorders/hypersensitivity-and-reactive-skin-disorders/stevens-johnson-syndrome-sjs-and-toxic-epidermal-necrolysis-ten
Doctors can usually diagnose Stevens-Johnson syndrome and toxic epidermal necrolysis by the appearance of the affected skin and mucous membranes, by their symptoms (pain rather than itching), by how quickly the skin symptoms progress, and by how much of the skin is affected. […] A sample of skin may be removed and examined under a microscope (called a skin biopsy).
#2 Stevens-Johnson Syndrome | Concise Medical Knowledge
https://www.lecturio.com/concepts/stevens-johnson-syndrome/
The diagnosis is clinical, based on history and physical exam findings. […] Skin biopsy is not required for diagnosis, but can confirm and rule out other conditions. […] Keratinocyte necrosis is the hallmark finding. […] Apoptotic keratinocytes are scattered in the basal layer of the epidermis in early lesions. […] Full-thickness epidermal necrosis and subepidermal bullae later in the disease. […] Direct immunofluorescence is negative.
#2 StevensâJohnson Syndrome and Toxic Epidermal Necrolysis: A Review of Diagnosis and Management
https://www.mdpi.com/1648-9144/57/9/895
StevensâJohnson Syndrome (SJS) and Toxic Epidermal Necrolysis (TEN) are rare diseases that are characterized by widespread epidermal necrosis and sloughing of skin. They are associated with significant morbidity and mortality, and early diagnosis and treatment is critical in achieving favorable outcomes for patients. […] Rapid diagnosis of SJS/TEN is critical in order to discontinue the offending agent, begin supportive and adjunctive therapies, and improve outcomes. However, the clinical presentation can be similar to a number of other blistering disorders, and diagnosis is not always straightforward. Given that the diagnosis of SJS/TEN is time sensitive, frozen sections can be utilized for more rapid decision making. […] There are a number of studies that have investigated potential diagnostic markers of the disease, with early studies focusing on the role of granulysin.
#2 Stevens-Johnson syndrome and toxic epidermal necrolysis – Symptoms, diagnosis and treatment | BMJ Best Practice US
https://bestpractice.bmj.com/topics/en-us/237?locale=th
1st tests to order include skin biopsy, blood cultures, complete blood count, glucose, magnesium, phosphate, blood urea nitrogen, bicarbonate, serum electrolytes, serum creatinine, liver function tests, C-reactive protein (CRP), arterial blood gases and saturation of oxygen, chest x-ray, coagulation studies, and skin swab from lesional skin. […] Investigations to avoid include erythrocyte sedimentation rate (ESR). […] Tests to consider include direct immunofluorescence.
#2 Stevens-Johnson Syndrome (SJS): Causes and Treatments
https://www.webmd.com/skin-problems-and-treatments/stevens-johnson-syndrome
To figure out whether you have Stevens-Johnson syndrome, your health care provider will start by asking questions about your medical history, including any medications you’ve recently started or stopped taking. They’ll also take a look at your rash. You may need more tests. They include: […] Skin biopsy, which involves removing a small sample of your skin. It will go to a lab to be tested. […] Culture, which involves removing skin, tissue, or fluid to send for testing. This can rule out infections that could be causing your condition. […] Imaging of your lungs to check for pneumonia, if your symptoms suggest it’s causing your SJS […] Blood test, which involves taking a sample of your blood. It then goes to a lab to check for signs of infection. […] Your health care team also will look at your pain level, how fast the rash has spread, and how much of your skin is affected.
#2 Stevens-Johnson Syndrome – StatPearls – NCBI Bookshelf
https://www.ncbi.nlm.nih.gov/books/NBK459323/
Stevens-Johnson syndrome and toxic epidermal necrolysis are acute, rare, and potentially fatal skin reactions involving loss of skin and, in some cases, mucosal membranes accompanied by systemic symptoms. Medications are causative in over 80 percent of cases. […] This activity illustrates the evaluation and treatment of Stevens-Johnson syndrome and of toxic epidermal necrolysis and reviews the role of the interprofessional team in managing those with these conditions. […] Summarize differential diagnoses of Stevens-Johnson syndrome and of toxic epidermal necrolysis. […] The severity of Stevens-Johnson syndrome/toxic epidermal necrolysis is assessed using SCORTEN. One point is scored for each of the following seven criteria at admission. […] The risk of dying from Stevens-Johnson syndrome/toxic epidermal necrolysis depends on the score.
#2 Erythema multiforme major/Stevens-Johnson syndrome: a diagnostic challenge | Anales de PediatrÃa
https://www.analesdepediatria.org/en-erythema-multiforme-major-stevens-johnson-syndrome-diagnostic-articulo-S2341287924002643
A male adolescent aged 14 years presented with fever, conjunctivitis, sore throat, oral ulcers, swollen lips and rash with onset 5 days prior. […] At this point, Stevens-Johnson syndrome (SJS) or erythema multiforme major (EMM) were suspected. […] The findings of the skin biopsy supported the diagnosis of SJS. […] Distinguishing between EMM and SJS is challenging. Both are characterized by widespread rash and mucous membrane involvement. However, EMM usually presents with typical target lesions mainly involving the skin, while SJS manifests with widespread erythematous macules and blisters, often leading to severe skin and mucous membrane detachment, with a higher risk of complications. […] In addition, in the case of SJS, two or more mucosal surfaces are usually involved and the patients tend to experience systemic symptoms preceding the cutaneous manifestations. […] Understanding these nuances is essential for accurate diagnosis and timely intervention.
#2 Stevens-Johnson syndrome and toxic epidermal necrolysis – Symptoms, diagnosis and treatment | BMJ Best Practice US
https://bestpractice.bmj.com/topics/en-us/237
Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) are severe skin detachments with mucocutaneous complications associated with a history of medication use, most commonly anticonvulsants, antibiotics, and nonsteroidal anti-inflammatory drugs. […] SJS/TEN may present with mild symptoms in early disease, but the condition progresses over several days; close monitoring and regular reassessment is essential. […] Once a diagnosis is established, the causative agent should be identified and withdrawn; the patient should receive skin care and supportive management. […] Transfer to a burn center, a specialized wound care center, or a dermatology intensive care unit is recommended for patients diagnosed with SJS/TEN. […] Patients at risk may need human leukocyte antigen screening if they are to receive medications such as carbamazepine, allopurinol, or trimethoprim/sulfamethoxazole.
#2 Orphanet: Stevens-Johnson syndrome/toxic epidermal necrolysis spectrum
https://www.orpha.net/en/disease/detail/95455
Diagnosis is suspected on clinical presentation and confirmed by skin biopsy which will reveal full-thickness epidermal necrosis and the absence of antibody deposits by direct immunofluorescence. […] Medication history will find a suspect drug in 85% of cases. […] Allergology work-up may help assessing the culprit in case of multiple suspects, and to find alternatives. […] Assessing possible genetic predisposition may help identifying the culprit drug in predisposed populations (e.g. Chinese Hans for carbamazepine and allopurinol).
#2 Stevens-Johnson syndrome
https://www.nhs.uk/conditions/stevens-johnson-syndrome/
Stevens-Johnson syndrome is a rare but serious skin reaction that’s usually caused by taking certain medicines. It needs to be treated immediately in hospital. […] Stevens-Johnson syndrome is treated in hospital, often in an intensive care unit. […] These may be signs of a serious reaction like Stevens-Johnson syndrome and may need immediate treatment in hospital.