A 4-year-old boy presented with blistering on his face and distal upper and lower extremities. Subepidermal blisters containing neutrophils and eosinophils visualized on histology supported the diagnosis of linear IgA bullous dermatosis of childhood (LABDC). The dermatosis presents with vesicles and tense blisters in an annular distribution, erythematous papules, and/or excoriated plaques. Histopathology shows subepidermal blisters with a neutrophilic infiltrate in the dermis, mainly concentrated at the tips of dermal papillae in the early stage of the disease, which can be mistaken for the pattern of neutrophilic infiltration as seen in dermatitis herpetiformis. Dapsone is the treatment of choice, which is started at a dosage of 0.5mg/kg/day. Linear IgA bullous dermatosis of childhood is a rare autoimmune disease that can be mistaken for other conditions with similar presentations but should always be considered in the differential diagnosis of children with blistering.
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