Otolaryngology Book

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Erythema Multiforme Major

Aka: Erythema Multiforme Major, Toxic Epidermal Necrolysis, Stevens-Johnson Syndrome, Stevens Johnson
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  1. 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
  2. Pathophysiology
    1. Severe clinical variant of Erythema Multiforme
    2. Previously thought to be part of same spectrum as minor
      1. Now thought to be clinically distinct
  3. Epidemiology
    1. Most common in winter and early spring
    2. More common in males by 2:1 ratio
    3. Ages affected most commonly 20-40 years
  4. Causes
    1. Medication (50% of Stevens Johnson and 90% of Toxic Epidermal Necrolysis)
      1. Antibiotics (esp. Sulfa drugs)
      2. Seizure medications
      3. NSAIDs
      4. Vaccinations
    2. Other causes
      1. Infectious disease
      2. Connective tissue disease
      3. Pregnancy
  5. Symptoms
    1. Prodrome (onset 1-3 weeks after causative exposure)
      1. Fever
      2. Malaise
      3. Headache
      4. Cough
    2. Sudden widespread skin eruption (onset 1-3 days after exposure)
      1. See below
  6. Signs
    1. Pathognomonic target lesions
      1. Widespread skin involvement
      2. See Erythema Multiforme for course and specific description
    2. Mucosal bullae (severe in 20% of cases)
      1. Oral Mucosa may lead to inadequate oral intake
      2. Respiratory may lead to respiratory distress
      3. Conjunctiva may lead to ocular complications
    3. Lesion distribution
      1. Starts on face and trunk and spreads peripherally
  7. Diagnosis
    1. Clinical diagnosis
    2. Skin biopsy can confirm the diagnosis
  8. Complications
    1. Secondary infection and Sepsis
    2. Multiorgan failure (Renal Failure, Respiratory failure)
    3. Corneal scarring (related to Corneal Ulceration)
  9. Labs
    1. No lab tests are specific for Stevens Johnson syndrome
  10. Management
    1. Intensive care unit (or burn unit) admission
    2. Withdraw all potential causative agents
    3. Fluid Resuscitation
    4. Wound care
    5. Antibiotics only where specifically indicated
    6. IV-Ig has been used in some cases
    7. Ophthalmology consult
  11. Prognosis
    1. Mortality
      1. Stevens Johnson: 5%
      2. Toxic Epidermal Necrolysis: 30%
  12. References
    1. Parrillo (2007) Curr Allergy Asthma Rep 7(4): 243-7
    2. Usatine (2010) Am Fam Physician 82(7): 773-80

Stevens-Johnson Syndrome (C0038325)

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) 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.
Definition (MSH) A variant of bullous erythema multiforme. It ranges from mild skin and mucous membrane lesions to a severe, sometimes fatal systemic disorder. Ocular symptoms include ulcerative conjunctivitis, keratitis, iritis, uveitis, and sometimes blindness. The cause of the disease is unknown.
Concepts Disease or Syndrome (T047)
MSH D013262
ICD9 695.13, 695.12
ICD10 L51.1, L51.9
SnomedCT 238819008, 73442001, 156362004, 267848009
English STEVENS JOHNSON SYNDROME, ERYTHEMA MULTIFORME EXUDATIVUM, FEBRILE MUCOCUTANEOUS SYNDROME, STEVENS-JOHNSON SYNDROME, SYNDROME STEVENS-JOHNSON, Stevens Johnson Syndrome, Erythema Multiforme Bullosum, Erythema Multiforme Bullosum (Stevens Johnson Syndrome), Stevens-Johnson Syndrome, Erythema multiforme, Stevens-Johnson type, Stevens-Johnson syndrome (EM major), Stevens-Johnson syndrome (diagnosis), Bullous erythema multiforme (disorder), Erythema exudativum multiforme, erythema multiforme major, erythema multiforme major (diagnosis), Stevens Johnson syndrome, Syndrome Stevens-Johnson, Stevens Jonhson syndrome, STEVEN JOHNSON SYNDROME, Erythema multiforme maj, Erythema multiforme major NOS, Stevens-Johnson Syndrome [Disease/Finding], johnson stevens syndrome, johnson steven syndrome, johnsons stevens syndrome, stevens johnsons syndrome, stevens-johnson syndrome, 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, 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, Stevens-Johnson synd., Erythema Multiforme Major, 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 bulleus erythema multiforme, erythema exsudativum multiforme, syndroom van Stevens-Johnson, Stevens-Johnson reactie, erythema multiforme major, erytheem; multiforme, bulleus, Bulleus erythema exsudativum multiforme, Stevens-Johnson-syndroom, Syndroom, Stevens-Johnson-
French Erythème polymorphe bulleux, Erythème multiforme majeur, Erythème exsudatif multiforme, Syndrome de Stevens-Jonhson, SYNDROME DE STEVENS-JOHNSON, Ectodermose érosive pluriorificielle, Syndrome de Stevens-Johnson, Ectodermose pluri-orificielle, Syndrome de Fiessinger-Rendu
German bulloeses Erythema multiforme, Erythema exudativum multiforme, Erythema multiforme exudativum, Stevens-Johnson Syndrom, Syndrom Stevens-Johnson, Stevens Johnson-Reaktion, Erythema multiforme major, Stevens Jonhson-Syndrom, Erythema exsudativum multiforme, Bulloeses Erythema exsudativum multiforme, STEVENS-JOHNSON SYNDROM, Erythema multiforme exsudativum majus, Stevens-Johnson-Syndrom
Portuguese Eritema esxudativo multiforme, Eritema multiforme major, Eritema bolhoso multiforme, Eritema multiforme exsudativo, Reacção de Stevens-Johnson, Eritema exsudativo multiforme, SINDROMA DE STEVENS JOHNS, Síndrome de Stevens-Johnson
Spanish Reacción de Stevens Johnson, Eritema exudativo multiforme, Eritema multiforme ampolloso, Síndrome de Stevens Jonhson, Eritema multiforme mayor, Eritema multiforme exudativo, Síndrome de Stevens Johnson, Stevens-Johnson synd., Stevens - Johnson syndrome, Bullous erythema multiforme, SINDROME DE STEVENS JOHNSON, ectodermosis erosiva pluriorificial, eritema multiforme bulloso, eritema multiforme exudativo, eritema multiforme mayor, síndrome de Stevens - Johnson (trastorno), síndrome de Stevens - Johnson, Sindrome de Stevens-Johnson, Síndrome de Stevens-Johnson
Japanese スティーブンス・ジョンソン症候群, 重症型多形紅斑, スティーブンス・ジョンソン型反応, 多形滲出性紅斑, 多形水疱性紅斑, ﾀｹｲｼﾝｼｭﾂｾｲｺｳﾊﾝ, ｼﾞｭｳｼｮｳｶﾞﾀﾀｹｲｺｳﾊﾝ, ｽﾃｨｰﾌﾞﾝｽｼﾞｮﾝｿﾝｼｮｳｺｳｸﾞﾝ, ｽﾃｨｰﾌﾞﾝｽｼﾞｮﾝｿﾝｶﾞﾀﾊﾝﾉｳ, ﾀｹｲｽｲﾎｳｾｲｺｳﾊﾝ
Swedish Stevens-Johnsons syndrom
Czech Stevensův-Johnsonův syndrom, Bulózní multiformní erytém, Erythema exudativum multiforme, Těžká forma multiformního erytému, Stevens Johnsonova reakce, Stevens Johnsonův syndrom, Stevens-Johnsonův syndrom
Finnish Stevens-Johnsonin oireyhtymä
Russian ERITEMA EKSSUDATIVNAIA ZLOKACHESTVENNAIA, BADERA DERMATOSTOMATIT, STIVENSA-DZHONSONA SINDROM, БАДЕРА ДЕРМАТОСТОМАТИТ, СТИВЕНСА-ДЖОНСОНА СИНДРОМ, ЭРИТЕМА ЭКССУДАТИВНАЯ ЗЛОКАЧЕСТВЕННАЯ
Korean 수포성 다형홍반
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, Bullosus erythema multiforme, Stevens-Johnson-syndroma, Erythema multiforme major
Sources
Derived from the NIH UMLS (Unified Medical Language System)


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