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Anti-p200 Pemphigoid: A Systematic Review
Khalaf Kridin1, A Razzaque Ahmed2
1Department of Dermatology, Rambam Health Care Campus, Haifa, Israel.
Frontiers in Immunology
|November 8, 2019
Summary
Anti-p200 pemphigoid is an autoimmune blistering disease with varied clinical presentations. Diagnosis may be suspected in younger patients with acral, cephalic, and mucosal involvement.
Area of Science:
- Dermatology
- Autoimmune Diseases
- Immunology
Background:
- Anti-p200 pemphigoid's clinical features are not well-understood.
- Existing data on epidemiological, clinical, histological, and immunological aspects require synthesis.
Purpose of the Study:
- To analyze and correlate known data on anti-p200 pemphigoid.
- To characterize the epidemiological, clinical, histological, and immunological features of this condition.
Main Methods:
- Systematic review of case reports and series.
- Searches conducted in Medline, Embase, and Web of Science databases (1900-2018).
- Qualitative analysis of 113 patients from 68 eligible studies.
Main Results:
- Mean age of onset was 65.5 years.
- All patients had bullae/vesicles; 54.3% had urticarial plaques.
- Common features included palmoplantar (51.4%), cephalic (40.3%), and mucosal (38.5%) involvement, with frequent scarring (15.7%).
- Autoantibodies against laminin γ1 detected in 73.1% of patients.
- Psoriasis co-occurred in 28.3%, notably in Japanese patients (56.4%), with increased pustular psoriasis incidence.
Conclusions:
- Anti-p200 pemphigoid diagnosis should be considered in younger individuals presenting with subepidermal autoimmune blistering disease.
- Key diagnostic indicators include significant acral, cephalic, and mucosal distribution.
- Co-occurrence with psoriasis, particularly pustular psoriasis, is notable in certain populations.
