伴手掌疼痛性皮疹的皮肌炎合并致死性急进性间质性肺病四例临床分析  

Clinical analysis of four cases of dermatomyositis with painful palmar eruptions complicated by fatal rapidly progressive interstitial lung disease

作　　者：鲁静浩 刘超凡 周行 胡东艳 辛崇美 朱鹭冰 李明 Lu Jinghao;Liu Chaofan;Zhou Xing;Hu Dongyan;Xin Chongmei;Zhu Lubing;Li Ming(Department of Dermatology,Zhongshan Hospital,Fudan University,Shanghai 200032,Chin)

机构地区：[1]复旦大学附属中山医院皮肤科,上海200032

出　　处：《中华皮肤科杂志》2018年第8期564-568,共5页Chinese Journal of Dermatology

基　　金：国家自然科学基金（30901291）

摘　　要：目的国内首报4例以手掌疼痛性皮疹为突出皮损表现的皮肌炎合并致死性急进性间质性肺病。方法2014年12月至2017年4月，复旦大学附属中山医院皮肤科诊治4例具有手掌疼痛性皮疹的皮肌炎合并致死性急进性间质性肺病患者，分析其临床与病理特征。结果患者中女3例，男1例，年龄47—59岁。4例中3例无肌肉受累。4例患者双手掌、指掌面和侧面均表现为多发红色坚实丘疹或结节，先于、晚于或与皮肌炎其他皮损同时发生。4例手掌疼痛性皮疹先于I型呼吸衰竭3周至5个月发生。早期多误诊为接触性皮炎、湿疹或多形红斑。指掌侧皮损组织病理示真皮血管周围少量淋巴细胞浸润，血管、附属器周围数量不等的黏蛋白样物质沉积。4例中3例抗黑素瘤分化相关基因5抗体阳性。虽经抗炎和免疫抑制治疗，4例均死于呼吸衰竭。结论皮肌炎患者伴发手掌疼痛性皮疹可能预示急进性间质性肺病发生，需及早对皮损活检以助识别，及时加强免疫抑制治疗，争取改善患者预后。Objective To firstly report 4 cases of dermatomyositis characterized by painful palmar eruptions complicated by fatal rapidly progressive interstitial lung disease （RP-ILD） in China. Methods Four patients with dermatomyositis with painful palmar eruptions complicated by fatal RP- ILD were enrolled from the Department of Dermatology, Zhongshan Hospital, Fudan University between December 2014 and April 2017, and their clinical and pathological features were analyzed. Results Among these patients, 3 were female and 1 was male. Their age ranged from 47 to 59 years. Of the 4 patients, 3 had no muscular involvement. All of the 4 patients had multiple solid red papules or nodules on the bilateral palms, palmar and lateral surfaces of fingers, which preceded, followed or concurred with the onset of other skin lesions of dermatomyositis. The occurrence of type I respiratory failure was preceded by 3 weeks to 5 months of painful palmar eruptions in the 4 patients. Early-stage palmar eruptions were easily misdiagnosed as contact dermatitis, eczema or erythema multiforme. Histopathological examination of the skin lesions on the finger palmar surface showed perivascular infiltration of a few lymphocytes in the dermis, and deposition of varying amounts of mucin-like substances around blood vessels and appendages. Of the 4 patients, 3 showed positive staining for anti- melanoma differentiation- associated gene 5 antibody. Although the 4 patients received anti-inflammatory and immunosuppressive therapies, they all finally died of respiratory failure. Conclusions Dermatomyositis with painful palmar eruptions may indicate the occurrence of fatal RP-ILD, and early biopsy of skin lesions is needed to help to identify the disease. Immunosuppressive treatment should be performed timely to improve the prognosis in these patients.

关 键 词：皮肌炎 肺疾病 间质性 皮肤表现 手 疼痛 糖皮质激素类 免疫抑制剂 

分 类 号：R563[医药卫生—呼吸系统] R593.26[医药卫生—内科学]