Epidermolysis Bullosa Acquisita in Children: Case Series

Abstract

Background. Epidermolysis bullosa acquisita (EBA) is chronic disease accompanied with subepidermal blistering on skin and mucous membranes as a result of autoimmune aggression to type VII collagen. EBA diagnostics in children is complicated due to similarity of clinical presentation with other bullous dermatosis in children.Clinical Case Description. The description of three clinical cases of EBA in children is provided. It is shown that for establishing the diagnosis it is necessary to estimate clinical evidence and to define the depth of blisters according to the results of histological examination of skin biopsy sample. Determination of IgG deposition positions relatively to the skin basal membrane due to performed indirect immunofluorescence test helps us to establish final diagnosis and specify patient management. Medical drug Dapsone was used in children with EBA, it has shown to be effective and safe to use as the first-line drug in management of such patients.Conclusion. The algorithm for EBA differential diagnosis with other bullous dermatosis in children is provided. Successful results of medical treatment are described.