Abstract: Objective: To summarize the clinical characteristics, diagnosis and treatment of patients with anti-p200 pemphigoid. Methods: Clinical data of 3 patients diagnosed with anti-p200 pemphigoid were collected, and a retrospective analysis was conducted on their clinical manifestations, laboratory characteristics, diagnosis and treatment regimens. Results: Among the 3 patients with anti-p200 pemphigoid, there were 1 female and 2 male cases. The clinical manifestations were bullous pemphigoid-like, linear IgA bullous dermatosis-like and eczematous lesions, respectively. All patients presented with mucosal involvement and pruritus. Histopathological examination revealed subepidermal blisters with varying degrees of infiltration by neutrophils eosinophils, and lymphocytes. Direct immunofluorescence (DIF) showed linear deposition of IgG and C3 along the basement membrane zone. Salt-split indirect immunofluorescence (ss-IIF) revealed that serum IgG antibodies bound to the dermal side of salt-split skin. The patients were treated with glucocorticoids alone, glucocorticoids combined with methotrexate and doxycycline combined with nicotinamide, respectively. All 3 patients achieved disease control at the 1-month follow-up; 2 patients achieved complete remission and discontinued treatment with no recurrence at the 1-year follow-up, and 1 patient was under follow-up with stable disease status. Conclusions: Anti-p200 pemphigoid exhibits diverse clinical manifestations. Its diagnosis requires integration of histopathology, DIF, ss-IIF and immunoblotting, and individualized treatment regimens should be adopted for clinical management.

Key words: anti-p200 pemphigoid, pemphigoid, bullous