结节性类天疱疮七例临床病理分析

目的 回顾分析结节性类天疱疮的临床特点。方法 回顾分析7例结节性类天疱疮患者的性别、发病年龄、临床表现及治疗和随访情况。结果 7例结节性类天疱疮患者中，女4例、男3例，发病年龄中位值59岁。临床表现以痒疹样皮损、结节为主，伴或不伴水疱，瘙痒症状明显，确诊前均误诊为结节性痒疹、湿疹。所有患者病理表现均有表皮增生肥厚，可见表皮下裂隙，真皮乳头胶原增生，浅层血管周围淋巴细胞、嗜酸性粒细胞浸润；直接免疫荧光检查基底膜带IgG和C3呈线状阳性，间接免疫荧光有2例阳性。7例联合使用糖皮质激素和免疫抑制剂治疗有效。结论 结节性类天疱疮临床易误诊，免疫病理检查有利于诊断，糖皮质激素和免疫抑制剂治疗有效。

Clinicopathological analysis of seven cases of pemphigoid nodularis

Chen Liuqing, Huang Meng, Chen Jinbo, Xia Yun, Chen Hongying, Ma Ling, Jiang Qian Department of Dermatology, Wuhan No.1 Hospital, Wuhan 430022, China Corresponding authors: Chen Liuqing, Email: chlq35@126.com; Chen Jinbo, Email: chen999jb@163.com 【Abstract】 Objective To analyze clinical characteristics of pemphigoid nodularis. Methods Seven cases of pemphigoid nodularis were included in this retrospective study. The clinicopathological features of pemphigoid nodularis were retrospectively analyzed, including patients′ gender, age at onset, clinical manifestations, treatment and follow?up. Results Of the 7 patients, 4 were female and 3 were male, with the median age at onset being 59 years. Clinical manifestations mainly included markedly pruritic prurigo?like lesions and nodular lesions with or without blisters. The most common misdiagnoses were nodular prurigo and eczema. Pathology showed epidermal hyperplasia and subepidermal clefts, collagen hyperplasia in the papillary dermis, and perivascular infiltration of lymphocytes and eosinophils in the superficial dermis in all the patients. Direct immunofluorescence assay showed linear deposition of IgG and C3 in the basement membrane zone, and indirect immunofluorescence was positive in 2 patients. Glucocorticoids combined with immunosuppressants were effective in all the patients. Conclusions Pemphigoid nodularis is easy to be misdiagnosed, and immunopathology is helpful for its diagnosis. Glucocorticoids combined with immunosuppressants appear to be effective for its treatment.