IMPORTANCE Immunobullous diseases mediated by IgA are often difficult to manage, but to date no mechanism has been proposed. Rituximab is an anti-CD20 monoclonal antibody that has demonstrated good efficacy in the treatment of refractory mucous membrane pemphigoid. However, not all cases of mucous membrane pemphigoid respond to rituximab. Herein we present a case of treatment-refractory mucous ...

OBJECTIVES To identify and analyze trends in bullous disease mortality from 1979 through 2002 in the United States. DESIGN Retrospective population-based analysis. SETTING Mortality records from the Centers for Disease Control and Prevention mortality database. PARTICIPANTS Mortality records from 1979 through 2002 for persons who died of bullous disease. MAIN OUTCOME MEASURES Age-adjust...

We have recently isolated a 1.0-kilobase (kb) cDNA encoding 180-kDa bullous pemphigoid antigen (BPAG2), an autoantigen in blistering skin disease, bullous pemphigoid (Giudice, G. J., Squiquera, H. L., Elias, P. M., and Diaz, L. (1991) J. Clin. Invest. 87, 734-738). The deduced amino acid sequence identified two collagenous domains characterized by Gly-X-Y repeats. In this study we have elucidat...

High-dose intravenous immunoglobulin (hdIVIg) is being used increasingly for dermatological indications. Its mode of action is via a number of proposed mechanisms and it is not associated with the many side-effects of steroids and other immunosuppressive agents. The evidence for using hdIVIg in the treatment of autoimmune bullous disorders is based on uncontrolled trials and case reports. Howev...

Anti-p200 pemphigoid has been characterized by autoantibodies to an unidentified 200-kDa protein (p200) of the dermal-epidermal junction. The objective of this study was to identify p200. We performed 2D gel electrophoresis of dermal extracts and immunoblotting with patients' sera, followed by MS analysis of a unique protein band. The protein band corresponded to laminin gamma1. Anti-laminin ga...

Bullous pemphigoid (BP) and mucous membrane pemphigoid (MMP) are autoimmune disorders involving IgG antibodies to basement membrane zone antigens [1]. The natural course of these diseases is that of exacerbations and remissions [2]. Older adults over the age of 60 and men are more likely to be affected [3]. BP manifests as tense, 1-3 cm fluid filled bullae on the skin [2]. The bullae may be on ...