Toxic Epidermal Necrolysis
Kristine Scruggs, MD
July 28, 2009
Lesions: Small blisters on dusky purpuric macules or
Mucosal involvement common
Prodrome of fever and malaise common
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.
Fever (often >39) and flu-like illness 1-3 days
before mucocutaneous lesions appear
Facial edema or central facial involvement
Lesions are painful
Skin necrosis, blisters and/or epidermal detachment
Mucous membrane erosions/crusting, sore throat
Visual Impairment (secondary to ocular
Rash 1-3 weeks after exposure, or days after 2nd
SJS: age 25-47, TEN: age
Greater extent of
Certain HLA types
Polymorphisms in IL4
Concomitant viral infections
UV light, radiation therapy
Higher doses of known
Secondary to cytotoxicity and delayed hypersensitivity reaction
to the offending agent.
Antigen is either the implicated drug or a metabolite.
Granulysin (cytolytic protein produced
by cytotoxic T cells and NK cells)
Expression of HLA-DR and
intracellular adhesion molecule
CD4 cells (in dermis)
CD8 T cells (in epidermis)
Apoptosis of keratinocytes
TNF-alpha, perforin and granzyme
fas-ligand expression (cell death receptor)
Subepidermal split with cell-poor bullous.
Epidermis shows full thickness necrosis.
Differential Diagnosis for Vesicular or
Associated with gluten intolerance
Mucosal involvement, sometimes cutaneous
Affects middle-aged or elderly
Differential Diagnosis, cont.
Linear IgA Disease
Itchy, ring-shaped, no internal disease
Herpes Simplex Virus
Early diagnosis - biopsy
Immediate discontinuation of offending agent
Supportive care – pay close attention to ocular
IV hydration (e.g. Parkland formula)
Local v. systemic corticosteroids
Think about nursing requirements!
Possible treatment in burn unit, wound care
Cooper, et al. The Washington Manual of Medical
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
Nirken, et al. Stevens-Johnson syndrome and toxic
epidermal necrolysis: Clinical manifestations,
pathogenesis, and diagnosis. Up To Date. 2009.
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