alexa Chronic Bullous Disease or Linear IgA Dermatosis of Childhood -Revisited
ISSN: 2157-7412

Journal of Genetic Syndromes & Gene Therapy
Open Access

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Review Article

Chronic Bullous Disease or Linear IgA Dermatosis of Childhood -Revisited

Patsatsi A*

2nd Dermatology Department, Aristotle University School of Medicine, Papageorgiou General Hospital, Thessaloniki, Greece

*Corresponding Author:
Aikaterini Patsatsi
Dermatology Department, Aristotle University School of Medicine
Papageorgiou General Hospital, Greece
Tel: +302310991583
Fax: +302310991473
E-mail: [email protected], [email protected]

Received date: May 31, 2013; Accepted date:June 19, 2013; Published date: June 22, 2013

Citation: Patsatsi A (2013) Chronic Bullous Disease or Linear IgA Dermatosis of Childhood -Revisited. J Genet Syndr Gene Ther 4:151. doi:10.4172/2157-7412.1000151

Copyright: © 2013 Patsatsi A. This is an open-access article distributed under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited.

 

Abstract

Linear IgA dermatosis or chronic bullous disease of childhood (CBDC) is a nonhereditary, autoimmune
subepidermal bullous disease, characterized by the presence of continuous linear deposits of IgA autoantibodies along the basement membrane zone. Antigens mainly involved in the pathogenesis of CBDC are a 97-kDa and a 120 –kDa protein, which represent fragments of the extracellular domain of collagen XVII (BP180), a transmembrane protein playing a critical role in maintaining the linkage between the intracellular and the extracellular structural elements involved in epidermal adhesion. Humoral and cellular response contributes to the pathogenetic mechanism leading to blister formation. CBDC is characterized by a clinical polymorphism in each patient, with the “cluster of jewels” pattern being the most typical clinical lesion. For the establishment of CBDC diagnosis, combination of
histology and immunofluorescence studies are of utmost importance. The majority of patients respond to dapsone, sulfapiridine or systemic steroids in cases of widespread disease. CBDC tends to resolve spontaneously within several months to 5 years after its onset. In this review article, the key features of this rare autoimmune bullous disease are revisited.

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