Zhimin Hao, Luqi Fan, Hao Song, Zhi Xiang
Pemphigus herpetiformis (PH) is a rare variant of pemphigus. Pediatric PH is exceedingly rare, and its clinical manifestations in children overlap with those of dermatitis herpetiformis and linear IgA bullous dermatosis (LABD), often leading to clinical misdiagnosis. Here, we describe a 10-year-old girl presenting with isolated bilateral lower limb erythema, clustered tense vesicles, and severe pruritus, without mucosal involvement. The diagnosis was confirmed by elevated desmoglein 1 antibodies, subcorneous vesicles, and epidermal intercellular IgG/C3 deposition. Histopathology revealed an atypical absence of eosinophilic infiltration, deviating from typical PH lesions. The patient achieved rapid and sustained remission with only oral antihistamines and topical care. This case supports the existence of a mild, localized pediatric PH subtype that may be resolved without conventional systemic therapy, yielding a favorable prognosis.