Epidermolysis bullosa acquisita in childhood

被引:49
作者
CallotMellot, C
Bodemer, C
Caux, F
BourgaultVillada, I
Fraitag, S
Goudie, G
Heller, M
deProst, Y
Prost, C
机构
[1] HOP NECKER ENFANTS MALAD, DERMATOL SERV, DEPT DERMATOL, F-75015 PARIS, FRANCE
[2] HOP NECKER ENFANTS MALAD, DEPT HISTOPATHOL, F-75015 PARIS, FRANCE
[3] HOP ST LOUIS, CTR STUDY DIAG BULLOUS DIS, PARIS, FRANCE
[4] HOP HENRI MONDOR, DERMATOL SERV, F-94010 CRETEIL, FRANCE
[5] HOP GEN, DREUX, FRANCE
关键词
D O I
10.1001/archderm.133.9.1122
中图分类号
R75 [皮肤病学与性病学];
学科分类号
100206 ;
摘要
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 II other pediatric cases of FBA 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.
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页码:1122 / 1126
页数:5
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