Epidermolysis bullosa acquisita in childhood.

Epidermolysis bullosa acquisita in childhood. Research Abstract Details 

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Epidermolysis bullosa acquisita in childhood. Abstract Text:

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 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.

Epidermolysis bullosa acquisita in childhood. Publishing Authors By Initials

For similar integumentary system: skin research abstracts see: integumentary system: skin research

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Epidermolysis bullosa acquisita in childhood. Journal Published:

PUBLICATION TYPE: Review

Journal: Archives of dermatology

VOLUME: 133

Page Numbers: 1122-6

Journal Abbreviation: Arch Dermatol

ISSN: 0003-987X

DAY: 17

MONTH: Sep

YEAR: 1997

Epidermolysis bullosa acquisita in childhood. Information

Number of References: 25

LANGUAGE: eng

NlmUniqueID: 372433

Epidermolysis bullosa acquisita in childhood. Keywords Mesh Terms:

KEYWORDS: Skin

MESH TERMS: ultrastructure

Chemical & Substance for Abstract: Epidermolysis bullosa acquisita in childhood. Information

Substance Name: Collagen

Registry Number: 9007-34-5

Grant and Affiliation Information for Epidermolysis bullosa acquisita in childhood.

AFFILIATION: Department of Dermatology, Hôpital Necker-Enfants Malades, Paris, France.

Country: UNITED STATES

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MEDLINETA: Arch Dermatol

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