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Epidermolysis Bullosa Acquisita in Childhood
Author(s) -
TrigoGuzmán Fanny X.,
Conti Adriana,
Aoki Valéria,
Maruta Celina W.,
Santi Cláudia G.,
Silva Claudia M. Resende,
Gontijo Bernardo,
Woodley David T.,
Rivitti Evandro A.
Publication year - 2003
Publication title -
the journal of dermatology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.9
H-Index - 65
eISSN - 1346-8138
pISSN - 0385-2407
DOI - 10.1111/j.1346-8138.2003.tb00376.x
Subject(s) - epidermolysis bullosa acquisita , dapsone , medicine , dermatology , prednisone , histopathology , epidermolysis bullosa , direct fluorescent antibody , cicatricial pemphigoid , pathology , pemphigoid , autoantibody , bullous pemphigoid , surgery , immunology , antibody
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.