• Dermatologic clinics · Jul 2011

    Review

    Diagnosis and clinical features of epidermolysis bullosa acquisita.

    • Frédéric Caux.
    • Department of Dermatology & Reference Center on Autoimmune Bullous Diseases, University Paris 13, Avicenne Hospital, AP-HP, 125 rue de Stalingrad, 93000 Bobigny, France. frederic.caux@avc.aphp.fr
    • Dermatol Clin. 2011 Jul 1; 29 (3): 485-91, x.

    AbstractEpidermolysis bullosa acquisita (EBA) is a rare autoimmune subepidermal blistering disease characterized by immune deposits on anchoring fibrils of cutaneous and mucosal basement membrane zones. It is due to circulating antibodies directed to type VII collagen. Clinical manifestations include a classical form with skin fragility, blisters and scars on trauma-prone surfaces, an inflammatory form, and a cicatricial pemphigoid-like form. Specialized tests available in only certain laboratories are necessary to confirm a diagnosis of EBA, such as immunoelectron microscopy, immunoblotting, or ELISA using recombinant proteins. A frequent association between EBA and Crohn disease has been observed.Copyright © 2011 Elsevier Inc. All rights reserved.

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