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Erythema Multiforme Major

Aka: Erythema Multiforme Major, Toxic Epidermal Necrolysis, Stevens-Johnson Syndrome, Stevens Johnson Syndrome, Stevens Johnson, Drug-Induced Skin Reaction

II. Definitions

  1. General
    1. Acute Life-threatening Hypersensitivity Skin Reaction
  2. Stevens Johnson Syndrome
    1. Affects 10% of body surface area
  3. Toxic Epidermal Necrolysis
    1. Affects 30% of body surface area

III. Pathophysiology

  1. Severe clinical variant of Erythema Multiforme
  2. Previously thought to be part of same spectrum as Erythema Multiforme Minor
    1. Now thought to be clinically distinct

IV. Epidemiology

  1. Most common in winter and early spring
  2. More common in adult women, but affects children of either gender equally
  3. Incidence U.S.
    1. Stevens Johnson Syndrome: 1 to 7 per million people per year
    2. Toxic Epidermal Necrolysis: 0.4 to 1.5 per million people per year

V. Causes

  1. Drug-Induced Skin Reaction (50% of Stevens Johnson and 90% of Toxic Epidermal Necrolysis)
    1. Highest risk with higher doses and rapid drug introduction
    2. Antibiotics
      1. Trimethoprim-Sulfamethoxazole (Bactrim) - most common
      2. Other antibiotics have also been implicated (Cephalosporins, Penicillins, Quinolones)
    3. Anticonvulsants
      1. Carbamazepine
      2. Phenytoin
      3. Phenobarbital
      4. Valproic Acid
    4. Acetaminophen
    5. Allopurinol
    6. NSAIDs
    7. Corticosteroids
    8. Vaccinations
  2. Infectious disease
    1. HIV Infection
    2. Herpes Simplex Virus
    3. Mycoplasma
    4. Hepatitis A
  3. Other causes
    1. Connective tissue disease (e.g. Systemic Lupus Erythematosus)
    2. Pregnancy
    3. Radiotherapy
    4. Vasculitis

VI. Symptoms

  1. Prodrome (onset 1-3 weeks after causative exposure)
    1. Fever
    2. Malaise
    3. Headache
    4. Cough
    5. Diffuse pain
  2. Sudden widespread skin eruption (onset 1-3 days after exposure)
    1. See below

VII. Signs

  1. Patient may appear toxic (esp. Toxic Epidermal Necrolysis)
  2. Pathognomonic target lesions
    1. Widespread skin involvement
    2. See Erythema Multiforme for course and specific description
    3. Positive Nikolsky Sign in both Stevens-Johnson and Toxic Epidermal Necrolysis
    4. Epidermal detachment is >30% in Toxic Epidermal Necrolysis (10% in Stevens-Johnson)
  3. Mucosal bullae (severe in 20% of cases)
    1. Oral Mucosa may lead to inadequate oral intake
    2. Respiratory bullae may lead to respiratory distress or respiratory failure
  4. Ocular involvement (30% of children, 70% of adults)
    1. May cause Conjunctivitis, Keratitis, Endophthalmitis
  5. Lesion distribution
    1. Starts on face and trunk and spreads peripherally

VIII. Diagnosis

  1. Clinical diagnosis
  2. Skin biopsy confirms the diagnosis
    1. Apoptosis and necrosis
    2. Keratinocyte vacuolization
    3. Dermal-epidermal separation
    4. Perivascular Lymphocyte infiltration

IX. Complications

  1. Secondary Skin Infections and Sepsis (Staphylococcus aureus, Pseudomonas)
    1. Most common cause of death
  2. Pulmonary involvement (Hypoxemia, hypercarbia, respiratory failure)
  3. Renal involvement (Acute Glomerulonephritis, Acute Renal Failure)
  4. Corneal scarring (related to Corneal Ulceration)
  5. Gastrointestinal conditions (ileus, Acute Hepatitis, hepatocellular necrosis)

X. Labs

  1. No lab tests are specific for Stevens Johnson Syndrome

XI. Management

  1. Withdraw all potential causative agents
  2. Intensive care unit (or burn unit) admission for SCORTEN Score >2
  3. Supportive care
    1. Fluid Resuscitation with 2 ml/kg/BSA%
    2. Nutritional support
    3. Opioid Analgesics are typically required
    4. Wound care
    5. Antibiotics only where specifically indicated
    6. Avoid Systemic Corticosteroids (use not supported by evidence)
  4. Specific immunologic agents that have been used in stevens-johnson and TEN
    1. Intravenous Immunoglobulin
    2. Cyclosporine
    3. Methotrexate
    4. N-Acetylcysteine
    5. Infliximab
    6. Azathioprine
    7. Cyclophosphamide
  5. Other measures
    1. Ophthalmology consult for eye involvement

XII. Prognosis

  1. See SCORTEN Score
  2. Mortality due to Sepsis, multi-organ failure
    1. Stevens Johnson: 1-3%
    2. Toxic Epidermal Necrolysis: 30-50%

XIII. Prevention

  1. Patient Education when prescribing drugs associated with Stevens-Johnson
    1. Immediately stop suspected drug (see causes above) if symptoms suggestive of Stevens-Johnson
    2. Skin pain, redness or Blister development with systemic symptoms (e.g. fever, Pharyngitis)
    3. Seek medical attention and do not reintroduce the suspected medication
    4. (2013) Presc Lett 20(10): 59

XIV. References

  1. Long (2016) Crit Dec Emerg Med 30(7):3-10
  2. Mockenhaupt (2008) J Invest Dermatol 128(1):35-44 [PubMed]
  3. Parrillo (2007) Curr Allergy Asthma Rep 7(4): 243-7 [PubMed]
  4. Usatine (2010) Am Fam Physician 82(7): 773-80 [PubMed]

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Ontology: Toxic Epidermal Necrolysis (C0014518)

Definition (CHV) An exfoliative skin disease caused by a toxic reaction to drugs, infections, and substance exposure.
Definition (CHV) An exfoliative skin disease caused by a toxic reaction to drugs, infections, and substance exposure.
Definition (CHV) An exfoliative skin disease caused by a toxic reaction to drugs, infections, and substance exposure.
Definition (CHV) An exfoliative skin disease caused by a toxic reaction to drugs, infections, and substance exposure.
Definition (CHV) An exfoliative skin disease caused by a toxic reaction to drugs, infections, and substance exposure.
Definition (CHV) An exfoliative skin disease caused by a toxic reaction to drugs, infections, and substance exposure.
Definition (CHV) An exfoliative skin disease caused by a toxic reaction to drugs, infections, and substance exposure.
Definition (NCI) A systemic, serious, and life-threatening disorder characterized by erythematous and necrotic lesions in the skin and mucous membranes that are associated with bullous detachment of the epidermis. The epidermal and mucous membranes detachment leads to sepsis and may be fatal. The lesions appear throughout the body and occupy more than 30% of the body surfaces. It is a hypersensitivity reaction usually caused by drugs (e.g., sulfonamides, nonsteroidal anti-inflammatory drugs, anticonvulsants, and antiretroviral drugs).
Definition (NCI_CTCAE) A disorder characterized by greater than 30% total body skin area separation of dermis. The syndrome is thought to be a hypersensitivity complex affecting the skin and the mucous membranes.
Definition (MSH) An exfoliative disease of skin seen primarily in adults and characterized by flaccid bullae and spreading erythema so that the skin has the appearance of being scalded. It results primarily from a toxic reaction to various drugs, but occasionally occurs as a result of infection, neoplastic conditions, or other exposure.
Concepts Disease or Syndrome (T047)
MSH D004816 , D013262
ICD9 695.15
ICD10 L51.2
SnomedCT 23067006, 200919006, 156362004, 267848009, 311031000009104
English Toxic Epidermal Necrolysis, LYELL SYNDROME, TOXIC EPIDERMAL NECROLYSIS, TEN - Toxic epidermal necrolys, Toxic epidermal necrolysis [Lyell], toxic epidermal necrolysis, toxic epidermal necrolysis (TEN), toxic epidermal necrolysis (diagnosis), TEN (toxic epidermal necrolysis), Lyell's disease, Toxic epideoneal necrolysis, Necrolysis epidermal toxic (Lyell type), Lyell syndrome, Syndrome Lyell's, TEN, Lyell Syndrome, Toxic epidrml necrolysis, Lyell toxic epidermal necrolysis, subepidermal type, lyell, lyell syndrome, lyell's syndrome, lyells, lyells syndrome, toxic epidermal necrolyses, Epidermal Necrolyses, Toxic, Scalded Skin Syndrome, Nonstaphylococcal, SCALDED SKIN SYNDROME NONSTAPH, Lyell's Syndrome, Syndromes, Lyell's, Necrolyses, Toxic Epidermal, TOX EPIDERMAL NECROLYSIS, EPIDERMAL NECROLYSIS TOX, Epidermal Necrolysis, Toxic, Nonstaphylococcal Scalded Skin Syndrome, Syndrome, Lyell's, Necrolysis, Toxic Epidermal, Toxic Epidermal Necrolyses, Lyell's Syndromes, NONSTAPH SCALDED SKIN SYNDROME, Leyell's syndrome, Toxic necrolysis, Necrolysis - toxic, Antibiotic necrolysis, Toxic epidermal necrolysis (disorder), Toxic epidermal necrolysis, Lyell's toxic epidermal necrolysis, subepidermal type, Lyell's syndrome, TEN - Toxic epidermal necrolysis, Lyell's toxic epidermal necrolysis, subepidermal type (disorder), Lyell, necrolysis; toxic epidermal
Spanish NECROLISIS EPIDERMICA TOXICA, SINDROME DE LYELL, NET, Lyell, Síndrome de Lyell, Enfermedad de Lyell, Necrólisis epidérmica tóxica (tipo Lyell), necrólisis epidérmica tóxica de Lyell, tipo subepidérmico (trastorno), necrólisis epidérmica tóxica de Lyell, tipo subepidérmico, síndrome de Lyell, tipo subepidérmico, Necrolisis epidérmica tóxica
Italian Necrolisi tossica epidermica, Malattia di Lyell, Lyell, Sindrome di Lyell, Necrolisi epidermica tossica (tipo Lyell), Necrolisi epidermica tossica
Dutch toxische epidermale necrolyse (Lyell-type), ziekte van Lyell, Leyll-syndroom, Lyell, necrolyse; toxisch epidermaal, Toxische epidermale necrolyse [Lyell], toxische epidermale necrolyse, Epidermolysis acuta toxica, Lyell-syndroom, Necrolyse, toxische epidermale (TEN), Syndroom van Lyell, TEN, Toxische epidermale necrolyse
French Syndrome de Debré-Lamy-Lamotte, Lyell, NET, Nécrolyse épidermique toxique (de type Lyell), Maladie de Lyell, NECROLYSE TOXIQUE EPIDERMIQUE, SYNDROME DE LYELL, Nécroépidermolyse aiguë, Érythrodermie bulleuse avec épidermolyse, Nécrose toxique de l'épiderme, Nécrolyse épidermique toxique, NET (Nécrolyse Épidermique Toxique), Épidermolyse bulleuse toxique, Épidermolyse nécrosante suraiguë, Syndrome de Lyell, Nécrolyse épidermique aiguë
German TEN, Lyell, Syndrom Lyell, Lyell-Krankheit, toxische epidermale Nekrolyse, Lyell-Syndrom, Nekrolyse epidermal toxisch (Lyell-Typ), LYELL SYNDROM, TOXISCH EPIDERMALE NEKROLYSE, Toxische epidermale Nekrolyse [Lyell-Syndrom], Epidermale Nekrolyse, toxische, Epidermolysis toxica acuta, Lyell-Syndrom, toxisches, Toxische epidermale Nekrolyse, Symptom der verbrühten Haut, Toxisches Lyell-Syndrom, Epidermolysis acuta toxica
Portuguese Síndroma de Lyell, Doença de Lyell, Necrólise epidérmica tóxica (tipo Lyell), Síndrome de Lyell, NECROLISE EPIDERMICA TOXIC, SINDROMA DE LYELL, Necrólise epidérmica tóxica
Japanese 中毒性表皮壊死�解症（ライエル型）, 中毒性表皮壊死�解症, ライエル, ＴＥＮ, ﾗｲｴﾙﾋﾞｮｳ, �ｭｳﾄﾞｸｾｲﾋｮｳﾋｴｼﾕｳｶｲｼｮｳﾗｲｴﾙｶﾞﾀ, ﾗｲｴﾙｼｮｳｺｳｸﾞ�, ﾗｲｴﾙ, �ｭｳﾄﾞｸｾｲﾋｮｳﾋｴｼﾕｳｶｲｼｮｳ, TEN, 表皮壊死�解-中毒性, Lyell症候群, TEN型薬疹, テン型薬疹, ライエル病, ライエル症候群, リエル症候群, 中毒性皮膚�死�解, 中毒性皮膚壊死�解, 中毒性表皮�死�解, 中毒性表皮壊死剥脱症, 中毒性表皮壊死�解, 中毒性表皮�解壊死型薬疹, 中毒性表皮�解症, �ブドウ��性皮膚剥離症候群, Lyell病, 中毒性表皮壊死症型薬疹, 熱傷様皮膚症候群-�ブドウ��性, �ブドウ��性熱傷様皮膚症候群
Swedish Epidermal nekrolys, toxisk
Finnish Toksinen epidermaalinen nekrolyysi
Czech Lyellův syndrom, Lyellův syndrom "opařené kůže", Toxická epidermální nekrolýza, Lyellova choroba, Toxická epidermální nekrolýza ( Lyellova typu), necrolysis epidermalis toxica, epidermolýza toxická nekrotizující
Korean 중�성 표피 괴사용해[��엘]
Polish Toksyczna nekroliza naskórka, Toksyczna martwica naskórka, Martwica naskórkowa toksyczna, Zespół Lyella, Martwica naskórka toksyczna
Hungarian Epidermalis necrolysis toxikus (Lyell-típusú), TEN, Lyell, Lyell-syndroma, Lyell-betegség, necrolysis epidermalis toxica, Lyell syndroma, Toxikus epidermalis necrolysis
Norwegian Epidermal nekrolyse, toksisk, Toksisk epidermal nekrolyse, Lyells syndrom
Croatian LYELLOV SINDROM, EPIDERMALNA NEKROLIZA, TOKSIÄŒNA
Derived from the NIH UMLS (Unified Medical Language System)

Ontology: Stevens-Johnson Syndrome (C0038325)

Definition (MSH) Rare cutaneous eruption characterized by extensive KERATINOCYTE apoptosis resulting in skin detachment with mucosal involvement. It is often provoked by the use of drugs (e.g., antibiotics and anticonvulsants) or associated with PNEUMONIA, MYCOPLASMA. It is considered a continuum of Toxic Epidermal Necrolysis.
Definition (NCI) A systemic, serious, and life-threatening disorder characterized by lesions in the skin and mucous membranes that may lead to necrosis. The lesions may appear anywhere in the body but they occur more commonly in the palms, soles, dorsum of the hands, and extensor surfaces. The lesions are vesicular or necrotic in the center, surrounded by an erythematous zone and occupy less than 10% of the body surfaces. The appearance of the mucocutaneous lesions is preceded by an upper respiratory tract infection. It is an immune complex hypersensitivity reaction usually caused by drugs (e.g., sulfa, phenytoin, penicillin), viruses (e.g., herpes simplex, influenza, hepatitis), and malignancies (e.g., carcinoma and lymphoma).
Definition (NCI_CTCAE) A disorder characterized by less than 10% total body skin area separation of dermis. The syndrome is thought to be a hypersensitivity complex affecting the skin and the mucous membranes.
Concepts Disease or Syndrome (T047)
MSH D013262
ICD9 695.13
ICD10 L51.1
SnomedCT 156362004, 267848009, 238819008, 73442001
English STEVENS JOHNSON SYNDROME, Stevens Johnson Syndrome, Stevens-Johnson Syndrome, Stevens-Johnson syndrome (EM major), Stevens-Johnson syndrome (diagnosis), Erythema exudativum multiforme, Stevens Johnson syndrome, Syndrome Stevens-Johnson, Stevens Jonhson syndrome, Stevens-Johnson Syndrome [Disease/Finding], johnson stevens syndrome, johnson steven syndrome, johnsons stevens syndrome, stevens johnsons syndrome, stevens-johnson syndrome, erythema multiforme major, johnsons steven syndrome, steven johnson syndrome, bullous erythema multiforme, erythema multiforme exudativum, steven johnsons syndrome, steven jonhson syndrome, johnson reaction stevens, erythema exsudativum multiforme, stevens johnson reaction, Stevens Johnson reaction, Stevens - Johnson syndrome, Bullous erythema multiforme (disorder), Stevens-Johnson synd., Erythema exsudativum multiforme, Stevens-Johnson syndrome, Bullous erythema multiforme, Ectodermosis erosiva pluriorificialis, Erythema multiforme bullosum, Erythema multiforme exudativum, Erythema multiforme major, Stevens-Johnson syndrome (disorder), Stevens-Johnson, ectodermosis erosiva pluriorificialis, erythema; multiforme bullosum, stevens johnson syndrome
Italian Sindrome di Stevens -Johnson, Eritema essudativo polimorfo, Sindrome di Stevens Johnson, Reazione di Stevens Johnson, Eritema polimorfo maggiore, Eritema multiforme bolloso, Sindrome di Stevens-Johnson
Dutch erythema exsudativum multiforme, syndroom van Stevens-Johnson, Stevens-Johnson reactie, erythema multiforme major, bulleus erythema multiforme, erytheem; multiforme, bulleus, Bulleus erythema exsudativum multiforme, Stevens-Johnson-syndroom, Syndroom, Stevens-Johnson-
French Ectodermose érosive pluriorificielle, Erythème exsudatif multiforme, Syndrome de Stevens-Jonhson, SYNDROME DE STEVENS-JOHNSON, Erythème polymorphe bulleux, Erythème multiforme majeur, SSJ (Syndrome de Stevens-Johnson), Dermatostomatite, Ectodermose érosive pluri-orificielle, Syndrome de Baader, Syndrome de Stevens-Johnson, Syndrome de Fiessinger-Rendu
German Erythema exudativum multiforme, Erythema multiforme exudativum, Stevens-Johnson Syndrom, Syndrom Stevens-Johnson, Stevens Johnson-Reaktion, Stevens Jonhson-Syndrom, Erythema exsudativum multiforme, Bulloeses Erythema exsudativum multiforme, STEVENS-JOHNSON SYNDROM, bulloeses Erythema multiforme, Erythema multiforme major, Erythema multiforme exsudativum majus, Stevens-Johnson-Syndrom
Portuguese Eritema esxudativo multiforme, Eritema multiforme exsudativo, Reacção de Stevens-Johnson, Eritema exsudativo multiforme, SINDROMA DE STEVENS JOHNS, Eritema bolhoso multiforme, Eritema multiforme major, Síndrome de Stevens-Johnson
Spanish Reacción de Stevens Johnson, Eritema exudativo multiforme, Síndrome de Stevens Jonhson, Eritema multiforme exudativo, Síndrome de Stevens Johnson, SINDROME DE STEVENS JOHNSON, Eritema multiforme ampolloso, Eritema multiforme mayor, ectodermosis erosiva pluriorificial, eritema multiforme bulloso, eritema multiforme exudativo, eritema multiforme mayor, síndrome de Stevens - Johnson (trastorno), síndrome de Stevens - Johnson, Síndrome de Stevens-Johnson
Japanese スティーブンス・ジョンソン症候群, スティーブンス・ジョンソン型�応, 多形滲出性紅斑, ﾀｹｲｼ�ｼｭﾂｾｲｺｳﾊ�, ｽﾃｨｰﾌﾞ�ｽｼﾞｮ�ｿ�ｼｮｳｺｳｸﾞ�, ｽﾃｨｰﾌﾞ�ｽｼﾞｮ�ｿ�ｶﾞﾀﾊ�ﾉｳ, ｼﾞｭｳｼｮｳｶﾞﾀﾀｹｲｺｳﾊ�, 多形水疱性紅斑, ﾀｹｲｽｲﾎｳｾｲｺｳﾊ�, �症型多形紅斑
Swedish Stevens-Johnsons syndrom
Czech Stevensův-Johnsonův syndrom, Erythema exudativum multiforme, Stevens Johnsonova reakce, Stevens Johnsonův syndrom, Stevens-Johnsonův syndrom, Těžká forma multiformního erytému, Bulózní multiformní erytém
Finnish Stevens-Johnsonin oireyhtymä
Russian ERITEMA EKSSUDATIVNAIA ZLOKACHESTVENNAIA, BADERA DERMATOSTOMATIT, STIVENSA-DZHONSONA SINDROM, �ЕКРОЛИЗ ЭПИДЕРМ�ЛЬ�ЫЙ ТОКСИЧЕСКИЙ, Л�ЙЕЛЛ� СИ�ДРОМ, LAIELLA SINDROM, ЭПИДЕРМ�ЛЬ�ЫЙ ТОКСИЧЕСКИЙ �ЕКРОЛИЗ, EPIDERMAL'NYI TOKSICHESKII NEKROLIZ, NEKROLIZ EPIDERMAL'NYI TOKSICHESKII, Б�ДЕР� ДЕРМ�ТОСТОМ�ТИТ, СТИВЕ�С�-ДЖО�СО�� СИ�ДРОМ, ЭРИТЕМ� ЭКССУД�ТИВ��Я ЗЛОК�ЧЕСТВЕ���Я
Korean 수�성 다형�반
Polish Zespół Stevensa-Johnsona
Hungarian Erythema multiforme exudativum, Stevens Johnson-syndroma, Stevens-Johnson reakció, Stevens-Johnson syndroma, Stevens-Johnson-szndroma, Erythema exsudativum multiforme, Erythema exudativum multiforme, Stevens-Johnson-syndroma, Bullosus erythema multiforme, Erythema multiforme major
Norwegian Stevens-Johnsons syndrom, SJS
Derived from the NIH UMLS (Unified Medical Language System)

Ontology: Erythema Multiforme Major (C3241919)

Definition (NCI) A severe, sometimes life-threatening, form of erythema multiforme. It is considered to be a hypersensitivity skin reaction triggered by a variety of stimuli, including infections and medication. It is characterized by raised, edematous papules in the extremities; involvement of one or more mucous membranes; and epidermal detachment involving less than ten percent of the total body surface area.
Concepts Disease or Syndrome (T047)
ICD9 695.12
ICD10 L51.9
English Erythema multiforme major, erythema multiforme major, erythema multiforme major (diagnosis), Erythema multiforme maj, Erythema multiforme major NOS, Erythema Multiforme Major
Derived from the NIH UMLS (Unified Medical Language System)

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