深在性红斑狼疮21例临床及组织病理分析

A clinical and pathological analysis of 21 cases of lupus erythematosus profundus

目的:总结深在性红斑狼疮(1upus erythematosus profundus,LEP)的临床及组织病理特点,加深对LEP的认识。方法:回顾性分析北京大学第一医院于2000-2011年间诊治的21例LEP患者的临床表现、实验室检查、组织病理特点和治疗反应。结果:21例患者中男5例,女16例,男女比例为1:3.2。平均发病年龄为34.33岁(7～77岁),平均病程1.4年(2个月～5年)。21例LEP患者共有皮损位点39处,其中26处位于头面部(66.67%),6处位于上肢(15.38%),4处位于躯干(10.26%),3处位于下肢(7.69%)。21例LEP中,独立存在的14例(66.67%),并发有盘状红斑狼疮(DLE)皮损的5例(23.81%),并发系统性红斑狼疮(SLE)2例(9.52%)。组织病理检查:21例LEP共同特点是皮下脂肪小叶有明显炎症累及,主要为淋巴细胞为主的浸润,其中4例(19.05%)伴有脂肪小叶透明变性坏死。21例LEP中15例(71.43%)组织病理同时有DLE的基本改变,还有3例(14.29%)伴有真皮浅层苔藓样淋巴细胞浸润,另有3例(14.29%)真皮血管壁及其周围呈纤维蛋白样变性。11例患者予羟氯喹联合泼尼松治疗皮损消退(2例复发),3例单独予羟氯喹治疗2例效果不佳,1例皮损消退。结论:LEP女性多发,皮损主要累及头面部及上肢,少数并发SLE。羟氯喹联合泼尼松对早期皮损治疗效果好。

Objective： To study the clinical and histopathological characteristics of lupus erythematosus profundus （LEP）. Method： The clinical and histopathological data, laboratory findings and response to treatment of 21 cases of LEP diagnosed in our department from 2000 to 2011 were retrospectively analyzed. Results： Among 21 patients, 5 were males, the other were females. The ratio of male to female was 1：3.2. The mean age at onset of the skin eruption was 34.33 year-old, and the average duration before presentation was 1.4 years. Among the 39 lesions of the 21 patients, 26 on the head and face （66.67%）, 6 on the upper limbs （15.38%）, 4 on the trunk （10.26%） and three on the lower limbs （7.69%）. Among the 21 cases, 14 cases were pure LEP, 5 cases were LEP associated with DLE, and the other two were LEP with SLE. Histopathology in all cases showed inflammation of subcutaneous fat lobule, with predominant lymphocytic infiltration . Lobular hyaline degeneration was seen in 4 out of 21 cases （19.05%）, accompanied DLE manifestations was seen in 15 cases （71.43%）, lichenoid lymphocytic infiltration in the superficial dermis was seen in 3 cases, and fibrinoid degeneration involved in vessel walls and their surroundings was seen in other 3 cases. After combination therapy of oral hydroxychloroquine sulfate with prednisone, the skin lesions cleared in 11 cases （relapse occurred in 2 of them after stopping of treatment）. Single medication of oral hydroxychloroquine sulfate was given to 3 cases, the skin lesion cleared in 2 of them but no response in the other. Conclusions： LEP mainly affects females, often involves the head, face and upper limbs of the patients, and associated with SLE in a few cases. Hydroxychloroquine sulfate combined with prednisone is highly effective in the early stage of the disease.