Anti-p200 pemphigoid: A systematic review

Khalaf Kridin, A. Razzaque Ahmed

Research output: Contribution to journalReview articlepeer-review

42 Scopus citations

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.

Original languageEnglish
Article number2466
JournalFrontiers in Immunology
Volume10
Issue numberOCT
DOIs
StatePublished - 2019
Externally publishedYes

Bibliographical note

Publisher Copyright:
© 2019 Kridin and Ahmed.

Funding

This study was supported in part by an unrestricted educational grant from the Dysimmune Diseases Foundation, Panama City, FL, USA.

FundersFunder number
Panama City
Dysimmune Diseases Foundation

    Keywords

    • Anti-laminin gamma-1 pemphigoid
    • Anti-p200 pemphigoid
    • BMZ autoantibodies
    • ELISA
    • IIF of salt-split skin
    • Immunoblotting
    • Mucosal disease
    • Psoriasis

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