LAUSR

Epidermolysis bullosa acquisita (EBA) is an acquired, subepidermal blistering skin disease characterized by autoantibodies against type VII collagen, which links the epidermis to the dermis at the dermo-epidermal junction. EBA is exceptionally rare in children. The diagnosis can be challenging as it shares clinical and histopathological features with other immunobullous diseases. We report three children diagnosed with EBA. The main clinical features were blisters (n=3), milia (n=3), scarring (n=2), oral erosions (n=2) and onychodystrophy (n=1). Histological findings were non-specific, showing a subepidermal blister with various inflammatory infiltrates. On direct immunofluorescence a bright deposit of IgG was seen, and the antibodies adhered to the dermal side of the salt-split in two cases. ELISA analysis confirmed circulating autoantibodies against type VII collagen in one patient. All of them received a course of oral corticosteroids alongside steroid-sparing agents such as dapsone, azathioprine, and rituximab depending on clinical response and shared favorable prognosis.