Anti-p200 Pemphigoid: A Systematic Review.

Khalaf Kridin,A Razzaque Ahmed

doi:10.3389/fimmu.2019.02466

Khalaf Kridin, A Razzaque Ahmed

Open Access

https://doi.org/10.3389/fimmu.2019.02466

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Abstract

The many clinical aspects of anti-p200 pemphigoid are not well-characterized. We aimed to analyze and correlate known existing data on the epidemiological, clinical, histological, and immunological features of anti-p200 pemphigoid. We performed a review using Medline, Embase, and Web of Science databases (1900–2018). Case reports and series of patients were included. A total of 68 eligible studies that comprised 113 anti-p200 pemphigoid patients were included in the qualitative analysis, where there was a mean age of onset of 65.5 years. All patients presented with bullae/vesicles, and 54.3% had urticarial plaques. A similarity to bullous pemphigoid was reported in 66.1% of cases, but palmoplantar (51.4%), cephalic (40.3%), and mucosal (38.5%) involvement, besides frequent development of scars/milia (15.7%), were reported. Autoantibodies against recombinant laminin γ1 were detected in the sera of 73.1% of patients. Psoriasis was present in 28.3% of anti-p200 pemphigoid patients, particularly among Japanese patients (56.4%). The incidence of pustular psoriasis in this subgroup, was significantly greater than in the normal population. In conclusion, the diagnosis of anti-p200 pemphigoid may be suspected when a subepidermal autoimmune blistering disease develops in a younger age group, along with significant acral and cephalic distribution and mucosal involvement.

Highlights

Anti-p200 pemphigoid is a rare subepidermal autoimmune bullous disease (AIBD) initially described in 1996 [1, 2]. This novel disease, presumed to be a subset of pemphigoid, was characterized by autoantibodies targeting a 200-kDa protein localized within the lower lamina lucida of the basement membrane zone (BMZ). Their sera bound to the dermal side of salt-split skin by indirect immunofluorescence (IIF) microscopy [3]
Subsequent studies demonstrated that sera from 90% of anti-p200 pemphigoid patients recognized laminin γ1, which C-terminus was identified as an immunodominant region and utilized for immunoblotting and ELISA for diagnosis [4, 5]
All cases were defined by the authors of the respective publications as anti-p200 pemphigoid based on the following three mandatory criteria: (i) clinical profile suggestive of subepidermal AIBD; (ii) reactivity to the 200 kDa protein or to the recombinant C-terminus of laminin γ1 by immunoblot analysis; and (iii) exclusion of other subepidermal AIBDs

Summary

Introduction

Anti-p200 pemphigoid is a rare subepidermal autoimmune bullous disease (AIBD) initially described in 1996 [1, 2]. This novel disease, presumed to be a subset of pemphigoid, was characterized by autoantibodies targeting a 200-kDa protein localized within the lower lamina lucida of the basement membrane zone (BMZ). Their sera bound to the dermal side of salt-split skin by indirect immunofluorescence (IIF) microscopy [3]. The specific or precise role for laminin γ1 in pathogenesis requires further study, as ex vivo and in vivo studies did not show evidence of a direct pathogenic role of anti-laminin γ1 antibodies, leaving the true molecular identity of the pathogenic 200 kDa autoantigen yet to be

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