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Table 2 Description of AGEP and TEN

From: Acute generalized exanthematous pustulosis simulating toxic epidermal necrolysis: case presentation and literature review

 AGEPTEN
Incidence1–5/million/year2–7/million/year
EtiologyDrug (90%)
viral, bacterial, or parasitic infections
spider bites
Drug (60%)
M. pneumoniae infections
1/3 cases no cause
Clinical presentation
 Distribution patternIntertriginous (generalized)Generalized
 Mucous Membrane20% (oral)100% (> 30%)
 PustulesYesNo
 Target lesionsNoYes
 Nikolsky signRareYes
 FeverYesYes
 TimingHours–daysDays–weeks (< 8 wks)
 Clinical courseResolution/re-epithelialization 2-4 weeks
 Histological featuresSpongiform 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 weeksAcute phase 8–12 days
Mortality 30%
 Treatmentd/c drugd/c drug
PO or IV corticosteroids, IV immunoglobulin, cyclosporin, anti-TNF