Table 2 Description of AGEP and TEN
AGEP | TEN | |
|---|---|---|
Incidence | 1–5/million/year | 2–7/million/year |
Etiology | Drug (90%) viral, bacterial, or parasitic infections spider bites | Drug (60%) M. pneumoniae infections 1/3 cases no cause |
Clinical presentation | ||
Distribution pattern | Intertriginous (generalized) | Generalized |
Mucous Membrane | 20% (oral) | 100% (> 30%) |
Pustules | Yes | No |
Target lesions | No | Yes |
Nikolsky sign | Rare | Yes |
Fever | Yes | Yes |
Timing | Hours–days | Days–weeks (< 8 wks) |
Clinical course | Resolution/re-epithelialization 2-4 weeks | |
Histological features | Spongiform subcorneal and/or intraepidermal pustules edema of the dermis, necrosis of single keratinocytes, and an inflammatory infiltrate of neutrophils and eosinophils with perivascular accentuation | Keratinocyte necrosis (partial to full-thickness necrosis of all epidermis layers) perivascular, discrete lymphohistiocytic, inflammatory infiltrate (some eosinophils) in the superficial dermis, ± subepidermal bullae |
Prognosis (mortality) | Resolution 2–4 weeks | Acute phase 8–12 days Mortality 30% |
Treatment | d/c drug | d/c drug PO or IV corticosteroids, IV immunoglobulin, cyclosporin, anti-TNF |