Retrospective Analysis of Clinical and Immunoserological Features in 86 Cases of Anti‐p200 Pemphigoid

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BACKGROUND: Anti-p200 pemphigoid is a rare autoimmune blistering disorder with limited data from China. This retrospective study aimed to investigate the clinical and serological characteristics of anti-p200 pemphigoid to enhance disease understanding. METHODS: We analysed 86 confirmed anti-p200 pemphigoid patients, evaluating their clinical manifestations, histopathological findings, and immunoserological profiles. RESULTS: The patients had a mean onset age of 56.92 ± 18.59 years and a male-to-female ratio of 2.31:1. The clinical presentation is highly heterogeneous, mimicking classic BP (57/86, 66.28%), and four cases (4/52, 7.69%) had concurrent psoriasis. Subepidermal blistering with variable dermal infiltrates was observed in 68 cases (68/70, 97.14%): neutrophil-predominant (24.89%), mixed neutrophilic/eosinophilic (31.43%), or eosinophil-predominant (27.14%). The positive rates of DIF, ss-IIF, IB with dermal extract, and IB with laminin γ1 C-terminal domain (LNγ1C) were 94.29% (66/70), 80.23% (69/86), 100% (86/86), and 64.29% (45/70), respectively. CONCLUSIONS: Anti-p200 pemphigoid closely resembles BP clinically but exhibits distinct immunopathological features. The partial reactivity to laminin γ1 (LNγ1) implies antigenic heterogeneity, warranting further investigation.

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