Acute generalized exanthematous pustulosis with erythema multiforme-like lesions

Acute generalized exanthematous pustulosis (AGEP) resembles generalized pustular psoriasis, but may manifest targetoid lesions, purpura, and blisters in addition to pustules. We describe a case of AGEP with erythema multiforme (EM)-like features in a 35-year-old woman who presented with acute onset of high fever and a strikingly polymorphic eruption consisting of numerous tiny pustules on erythematous bases, marked facial edema, oral and genital erosions, targetoid vesicular and purpuric lesions, pustules in string-of-pearl configuration and ring-like vesicles. The histology revealed, in addition to subcorneal pustules, vacuolar interface dermatitis with involvement of eccrine glands, and microabscesses in pilosebaceous structures. Systemic corticorsteroid and antibiotics were initiated, resulting in rapid resolution without recurrence. Recognition of EM-like lesions on a background of generalized pustular eruption could facilitate the diagnosis of AGEP and the institution of appropriate treatment.