Successful Treatment of Intravenous Immunoglobulins in a Patient with Intractable Epidermolysis Bullosa Acquisita with Autoantibodies to Type VII Collagen and Laminin Alpha-3
- *Corresponding Author:
- Shin-Ichi Osada
Department of Dermatology
Nippon Medical School, 1-1-5 Sendagi
Bunkyo-ku, Tokyo 113-8602, Japan
Tel: +81-3- 5814-6254
Email: [email protected]
Received date: November 04, 2013; Accepted date: December 10, 2013; Published date: December 17, 2013
Citation: Osada SI, Yoshida R, Kikuchi I, Tsuruta D, Ansai SI, et al. (2013) Successful Treatment of Intravenous Immunoglobulins in a Patient with Intractable Epidermolysis Bullosa Acquisita with Autoantibodies to Type VII Collagen and Laminin Alpha-3. J Clin Exp Dermatol Res 4:200. doi: 10.4172/2155-9554.1000200
Copyright: © 2013 Osada SI, et al. 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.
Epidermolysis bullosa acquisita (EBA) is a blistering disease caused by autoantibodies to type VII collagen, a major component of anchoring fibrils at the dermal-epidermal junction. Here, we report a case of inflammatory EBA with a unique antibody prolife showing reactivity to laminin alpha-3 as well as type VII collagen. The patient’s cutaneous lesions were refractory to dapsone, prednisolone, betamethasone, and double filtration plasmapheresis, which led to a catheter-mediated methicillin-resistant staphylococcal aureus (MRSA) sepsis. Intravenous immunoglobulins (IVIG) initially used to resolve MRSA sepsis improved the pruritus and skin manifestations of EBA, and clinical remission of EBA was achieved after only two cycles of IVIG. The mechanism for the concurrence of antibodies to type VII collagen and laminin alpha-3 and the potential mode of action of IVIG in EBA are discussed.