Epidermolysis bullosa acquisita in childhood.
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.


METHODS
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 acquisita 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.
Callot-Mellot
C
C
Department of Dermatology, Hôpital Necker-Enfants Malades, Paris, France.
Bodemer
C
C
Caux
F
F
Bourgault-Villada
I
I
Fraitag
S
S
Goudié
G
G
Heller
M
M
de Prost
Y
Y
Prost
C
C
D000293:Adolescent; D001323:Autoantibodies / Q000032:analysis; D001327:Autoimmune Diseases / Q000175:diagnosis* / Q000473:pathology; D015153:Blotting, Western; D002648:Child; D002675:Child, Preschool; D003094:Collagen / Q000276:immunology; D016107:Epidermolysis Bullosa Acquisita / Q000175:diagnosis* / Q000473:pathology; D005260:Female; D019085:Fluorescent Antibody Technique, Direct; D006801:Humans; D008297:Male; D016253:Microscopy, Immunoelectron; D008970:Molecular Weight; D012867:Skin / Q000276:immunology / Q000648:ultrastructure
D001323:Autoantibodies; D003094:Collagen