Clinical and immunological features and outcome of anti-p200 pemphigoid.

Background Anti p200 pemphigoid is a rare autoimmune blistering disease (AIBD) of the dermoepidermal junction, characterized by autoantibodies to laminin gamma 1. The clinical course of anti p200 pemphigoid in patients remains poorly investigated. Objectives We aimed to describe the clinical and immunological features and the course of a series of patients with anti-p200 pemphigoid. Methods We conducted a retrospective study by immunoblotting detection of sera on 200-kDa dermal protein extracts from the register of the French reference centre for AIBD. We recorded the clinical and immunological features and the course of patients. Results A total of 14 patients with a mean age 81.6 +/- 6.5 years were included. Only one patient had an associated neurological condition and one had psoriasis. Twelve patients had atypical clinical presentation, including ectematous, urticarial, prurigo like, dyshydrosis-like and rosette like skin lesions. Eight patients (57%) had mucosal involvement. Immunoblot analysis of sera on dermal and epidermal extracts showed a 200 kDa band in 14 and 10 cases, respectively. All eight of the sera tested by enzyme linked immunosorbent assay detected recombinant human laminin gamma 1. Disease control was obtained in six of nine patients treated with topical corticosteroids, and four of five patients who received systemic treatment. Seven patients relapsed (50%) and five patients (36%) died during the median follow up time of 12.6 months. At the end of the study, only one of the nine living patients was in complete remission off therapy. Conclusions Many patients with anti-p200 pemphigoid had heterogeneous clinical presentation and a more severe prognosis than previously suspected.