Epidermolysis bullosa acquisita in childhood.
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.
Trigo-Guzmán
Fanny X
FX
Department of Dermatology, University of São Paulo, Brazil.
Conti
Adriana
A
Aoki
Valéria
V
Maruta
Celina W
CW
Santi
Cláudia G
CG
Resende Silva
Claudia M
CM
Gontijo
Bernardo
B
Woodley
David T
DT
Rivitti
Evandro A
EA
D000284:Administration, Oral; D001707:Biopsy, Needle; D003622:Dapsone / Q000008:administration & dosage*; D004359:Drug Therapy, Combination; D016107:Epidermolysis Bullosa Acquisita / Q000175:diagnosis* / Q000188:drug therapy*; D019084:Fluorescent Antibody Technique, Indirect; D005500:Follow-Up Studies; D006801:Humans; D007223:Infant; D008297:Male; D011241:Prednisone / Q000008:administration & dosage*; D018570:Risk Assessment; D012720:Severity of Illness Index; D016896:Treatment Outcome
D003622:Dapsone; D011241:Prednisone