Linear IgA Bullous Dermatosis of Childhood

dok_zlo — 13.03.2025

A previously healthy 2-year-old boy was brought to the dermatology clinic with a 1-week history of itchy, red spots. Three days before the onset of the rash, the child had had an upper respiratory infection for which he had been given ibuprofen. On physical examination, tense vesicles and edematous pink plaques with central erosions and crust were seen on the patient’s legs (Panel A), arms, and back. Dense clustering of skin lesions was observed in the axillae and inguinal folds. No mucosal involvement was noted. Histopathological analysis of a skin-biopsy sample obtained from the right lower back showed a subepidermal blister with robust neutrophilic infiltration. Direct immunofluorescence revealed a linear band of IgA along the dermoepidermal junction (Panel B). A diagnosis of linear IgA bullous dermatosis of childhood was made. Linear IgA bullous dermatosis is a rare autoimmune blistering disorder that is characterized by the presence of IgA antibodies bound to the basement membrane zone. The condition may be drug-induced or idiopathic. In young children, the condition may take months or years to resolve. Treatment with dapsone, after glucose-6-phosphate dehydrogenase (G6PD) deficiency was ruled out, and a slowly tapering dose of prednisolone was initiated. Six months after the start of treatment, the glucocorticoid was stopped and dapsone was continued. By 7 months of follow-up, the lesions had completely resolved.
Arjun Pandya, M.D., and Mikael Horissian, M.D.
Published March 12, 2025
N Engl J Med 2025;392:1120
DOI: 10.1056/NEJMicm2413814
VOL. 392 NO. 11

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