September 1997

Epidermolysis Bullosa Acquisita in Childhood

Author Affiliations

From the Departments of Dermatology (Drs Callot-Mellot, Bodemer, and de Prost) and Histopathology (Dr Fraitag), Hôpital Necker-Enfants Malades, and the Center of the Study and Diagnosis of Bullous Diseases, Hôpital Saint Louis (Drs Caux, Heller, and Prost), Paris, Dermatology Services, Hôpital Henri Mondor (Dr Bourgault-Villada), Créteil, and Hôpital Général (Dr Goudié), Dreux, France.

Arch Dermatol. 1997;133(9):1122-1126. doi:10.1001/archderm.1997.03890450070008

Background:  Epidermolysis bullosa acquisita (EBA) is an autoimmune bullous disease characterized by the presence of antitype VII collagen antibodies, leading to the formation of bullae in the dermoepidermal junction. This disease is rare in childhood.

Observations:  We report 3 new cases of EBA in children. The 3 patients were similar; all 3 children were black, with a clinical phenotype resembling linear IgA bullous disease in children and typical histologic and immunologic features of EBA. In the 3 patients, diagnosis was proven using immune electron microscopy and Western blot analysis, where antitype VII collagen antibodies were demonstrated. Patients 1 and 2 were successfully treated with a combination of prednisone and dapsone. In patient 3, the lesions healed without specific therapy. We found 11 other pediatric cases of EBA in the literature and studied those cases in addition to the cases presented herein to describe the characteristics of EBA in childhood.

Conclusions:  Epidermolysis bullosa aquisita is a rare disease in childhood. Mucosal involvement is frequent and severe. Because the clinical features are misleading, the use of immune electron microscopy and Western blot analysis is essential to making a diagnosis. Treatment with a combination of prednisone and dapsone is often effective. The prognosis in children is better than it is in adult patients.Arch Dermatol. 1997;133:1122-1126