Epidermolysis Bullosa Acquisita in Childhood

• Published in: Journal of dermatology Vol. 30; no. 3; pp. 226 - 229
• Main Authors: Trigo‐Guzmán, Fanny X., Conti, Adriana, Aoki, Valéria, Maruta, Celina W., Santi, Cláudia G., Silva, Claudia M. Resende, Gontijo, Bernardo, Woodley, David T., Rivitti, Evandro A.
• Format: Journal Article
• Language: English
• Published: England 01-03-2003
• Subjects: Administration, Oral; autoimmune blistering disease; autoimmunity; Biopsy, Needle; bullous pemphigoid; Dapsone - administration & dosage; Drug Therapy, Combination; epidermolysis bullosa acquisita; Epidermolysis Bullosa Acquisita - diagnosis; Epidermolysis Bullosa Acquisita - drug therapy; Fluorescent Antibody Technique, Indirect; Follow-Up Studies; Humans; Infant; Male; Prednisone - administration & dosage; Risk Assessment; Severity of Illness Index; Treatment Outcome
• ISSN: 0385-2407; 1346-8138
• DOI: 10.1111/j.1346-8138.2003.tb00376.x

Epidermolysis bullosa acquisita (EBA) is a subepidermal autoimmune blistering disease that is rarely reported in childhood. We describe a nine‐month‐old mulatto boy presenting with multiple, annular, widespread, tense blisters and oral lesions. The diagnosis of EBA was confirmed by histopathology, immunofluorescence, and immunoblotting analysis. The patient was successfully treated with systemic steroids (prednisone) and dapsone. After 20 months of initial treatment, clinical remission was observed, and dapsone remains as the current treatment. This case report emphasizes the rarity of EBA in childhood and the difficulties in reaching the final diagnosis.