关键词: 皮肤疾病, 水疱大疱性, 抗p200类天疱疮, 类天疱疮, 大疱性, 皮肤表现, 荧光抗体技术, 间接, 盐裂皮肤, 印迹法, 蛋白质, 治疗

Abstract: 【Abstract】 Objective To analyze clinical, immunoserological, and therapeutic features of patients with anti-p200 pemphigoid. Methods Clinical data were collected from patients with confirmed anti-p200 pemphigoid at the Hospital of Dermatology, Chinese Academy of Medical Sciences from January 2015 to February 2024. Their clinical, immunoserological, and therapeutic characteristics were retrospectively analyzed. Results A total of 35 patients were included, with a male-to-female ratio of 2.5∶1 （25 males and 10 females） and ages of 57.74 ± 17.12 years. Two （5.71%） patients were accompanied by psoriasis. In these patients, anti-p200 pemphigoid exhibited heterogeneous clinical phenotypes, mimicking classic bullous pemphigoid （20 cases, 57.14%）, linear IgA bullous dermatosis （8 cases, 22.86%）, or eczema （4 cases, 11.43%）. The positive rates of direct immunofluorescence （DIF）, indirect immunofluorescence on salt-split skin （ss-IIF）, Western blot analysis with dermal extracts as substrates, and Western blot analysis with laminin γ1 C-terminal region （Lnγ1C） as substrates were 100% （24/24）, 82.86% （29/35）, 100% （35/35）, and 80.64% （25/31）, respectively. Among the 35 patients, treatment and follow-up information was available for analysis in 33. Six patients （18.18%） received non-glucocorticoid systemic therapy and topical glucocorticoid therapy, with a follow-up period （M ［Q1 , Q3］） of 19.50 （6.50, 69.25） months, and 1 withdrew the drugs. Sixteen patients received systemic glucocorticoids combined with traditional anti-inflammatory drugs, with a follow-up period of 13.50 （4.25, 18.00） months, the initial dose of glucocorticoids was equivalent to 0.30 - 0.50 mg·kg-1·d-1 of prednisone, and the time to disease control was 15.31 ± 5.23 days; among the 16 patients, 3 experienced fluctuations in disease condition which were alleviated by adding dapsone, and 1 discontinued glucocorticoids. Five patients （15.15%） received systemic glucocorticoids combined with immunosuppressants, with a follow-up period of 26.00 （14.00, 90.00） months, the initial dose of glucocorticoids was equivalent to 0.50 - 0.75 mg·kg-1·d-1 of prednisone, and the time to disease control was 10.20 ± 3.27 days; among the 5 patients, 2 received maintenance treatment with glucocorticoids （5 - 10 mg/d prednisone）, 2 withdrew the drugs, and 1 relapsed after discontinuing glucocorticoids. One patient （3.03%） received systemic glucocorticoids combined with rituximab therapy, with a follow-up period of 53 months, and discontinued glucocorticoids thereafter. One patient （3.03%） received systemic glucocorticoids combined with dupilumab therapy, which proved to be effective. Four patients （12.12%） received systemic glucocorticoids combined with Janus kinase inhibitors, and 3 responded well. Conclusions Anti-p200 pemphigoid presented a heterogeneous clinical profile in this series of patients, but scarring and milia were rare. Some patients showed negative results in Western blot analysis with Lnγ1C as substrates. The prognosis of anti-p200 pemphigoid was usually favorable, and most patients could achieve complete remission and ultimately discontinue medication.

Key words: Skin diseases, vesiculobullous, Anti-p200 pemphigoid, Pemphigoid, bullous, Skin manifestations, Fluorescent antibody technique, indirect, salt-split skin, Blotting, Western, Therapy