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Toxic Epidermal Necrolysis. Kristine Scruggs, MD AM Report July 28, 2009. Definition. SJS/TEN: Lesions: Small blisters on dusky purpuric macules or atypical targets Mucosal involvement common Prodrome of fever and malaise common Stevens-Johnson Syndrome: Rare areas of confluence.

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toxic epidermal necrolysis

Toxic Epidermal Necrolysis

Kristine Scruggs, MD

AM Report

July 28, 2009

definition
Definition
  • SJS/TEN:
    • Lesions: Small blisters on dusky purpuric macules or atypical targets
    • Mucosal involvement common
    • Prodrome of fever and malaise common
  • Stevens-Johnson Syndrome:
    • Rare areas of confluence.
    • Detachment </= 10% BSA
  • Toxic Epidermal Necrolysis:
    • Confluent erythema is common.
    • Outer layer of epidermis separates easily from basal layer with lateral pressure.
    • Large sheet of necrotic epidermis often present.
    • >30% BSA involved.
presentation
Presentation
  • Fever (often >39) and flu-like illness 1-3 days before mucocutaneous lesions appear
  • Confluent erythema
  • Facial edema or central facial involvement
  • Lesions are painful
  • Palpable purpura
  • Skin necrosis, blisters and/or epidermal detachment
  • Mucous membrane erosions/crusting, sore throat
  • Visual Impairment (secondary to ocular involvement)
  • Rash 1-3 weeks after exposure, or days after 2nd exposure
epidemiology
2-7/million people/year

SJS: age 25-47, TEN: age 46-63

Women: >60%

Poor prognosis:

Intestinal/Pulmonary involvement

Greater extent of detachment

Older age

Mortality:

SJS: 5%

TEN: 30%

Risk Factors:

HIV infection

Genetic factors

Certain HLA types

“Slow acetylators”

Polymorphisms in IL4 receptor gene

Concomitant viral infections

Underlying immunologic diseases

Physical factors

UV light, radiation therapy

Malignancy

Higher doses of known offenders

Epidemiology
pathogenesis
Pathogenesis
  • Secondary to cytotoxicity and delayed hypersensitivity reaction to the offending agent.
  • Antigen is either the implicated drug or a metabolite.
  • Histopathology:
    • Granulysin (cytolytic protein produced

by cytotoxic T cells and NK cells)

    • Expression of HLA-DR and

intracellular adhesion molecule

(ICAM)-1 by

      • Keratinocytes
      • CD4 cells (in dermis)
      • CD8 T cells (in epidermis)
    • Apoptosis of keratinocytes

facilitated by

      • TNF-alpha, perforin and granzyme

secretion

      • fas-ligand expression (cell death receptor)

Subepidermal split with cell-poor bullous.

Epidermis shows full thickness necrosis.

etiologies
Etiologies
  • Medications (Odds Ratio for exposure in hospitalized pts):
    • Sulfonamide antibiotics (172)
    • Allopurinol (52)
    • Amine antiepileptics
      • Phenytoin (53)
      • Carbamazepine (90)
    • Lamotrigine
    • NSAIDs (72)
  • Infections (e.g. Mycoplasma pneumonia)
  • Other: Vaccinations, Systemic diseases, Chemical exposure, Herbal medicines, Foods
differential diagnosis for vesicular or bullous rash
Differential Diagnosis for Vesicular or Bullous Rash

Bullous

Pemphigoid

Often affects

the elderly

Dermatitis Herpetiformis

Associated with gluten intolerance

Pemphigus

Affects middle-aged or elderly

Cicatricial Pemphigoid

Mucosal involvement, sometimes cutaneous

differential diagnosis cont
Differential Diagnosis, cont.

Linear IgA Disease

Itchy, ring-shaped, no internal disease

Herpes Simplex Virus

Varicella/Zoster Virus

Hand-Foot-Mouth

Disease

(Enteroviruses)

Contact Dermatitis

differential diagnosis cont1
Differential Diagnosis, cont.
  • Erythema Multiforme
  • Staphylococcal Scalded Skin Syndrome
  • Bullous Impetigo
  • Toxic Shock Syndrome
  • Paraneoplastic Pemphigus
  • Cutaneous emboli
  • Diabetic Bullae
  • Porphyria Cutanea Tarda
  • Porphyria Variegata
  • Pseudoporphyria
  • Bullous dermatosis of Hemodialysis
  • Coma Bulloae
  • Epidermolysis Bullosa Acquisita
treatment
Treatment
  • Early diagnosis - biopsy
  • Immediate discontinuation of offending agent
  • Supportive care – pay close attention to ocular complications
    • IV hydration (e.g. Parkland formula)
    • Antihistamines
    • Analgesics
    • Local v. systemic corticosteroids
    • Think about nursing requirements!
  • Possible treatment in burn unit, wound care
  • IVIg?
resources
Resources:
  • Cooper, et al. The Washington Manual of Medical Therapeutics, 32nd Edition. 2007.
  • High, et al. Stevens-Johnson syndrome and toxic epidermal necrolysis: Management, prognosis, and long-term sequelae. Up To Date. 2009.
  • Kasper, et al. Harrison’s Principles of Internal Medicine, 16th Edition. 2005.
  • Nirken, et al. Stevens-Johnson syndrome and toxic epidermal necrolysis: Clinical manifestations, pathogenesis, and diagnosis. Up To Date. 2009.