儿童头皮深在性红斑狼疮五例  

Lupus erythematosus profundus of the scalp in five children

作　　者：徐教生[1] 徐子刚[1] 孙玉娟[1] 尹瑞瑞[1] 张立新[1] 

机构地区：[1]首都医科大学附属北京儿童医院皮肤科,北京100045

出　　处：《中华皮肤科杂志》2016年第6期411-414,共4页Chinese Journal of Dermatology

摘　　要：目的探讨儿童头皮深在性红斑狼疮（LEP）临床及病理学特征。方法回顾性分析5例LEP患儿临床、组织病理特点及治疗和预后情况。结果5例儿童头皮LEP，男2例，女3例；中位发病年龄5个月（范围2—38个月）；中位病程15个月（范围4～72个月）。皮损为头部弧形或环形紫红色萎缩性斑块伴脱发，枕部及颞部最常受累。1例患儿抗核抗体（ANA）1：100，4例患儿ANA抗Ro／SSA、La／SSB抗体检查均为阴性。组织病理学改变主要为脂肪透明变性，黏蛋白沉积及脂肪小叶淋巴细胞灶状聚集。2例口服泼尼松（1．5～2）mg·kg^-1·kd^-1，1例口服羟氯喹5mg·kg^-1·d^-1，1例口服泼尼松1．5mg·kg^-1·d^-1并联合羟氯喹5mg·kg^-1·d^-1；1例患儿仅外用卤米松乳膏及0．03％他克莫司软膏。患儿皮损于治疗后2～3月均获得缓解，6个月消退，新发生长，随访1．5年未见病情反复。结论头皮LEP对泼尼松及羟氯喹治疗反应良好，患儿可选用强效糖皮质激素及钙调磷酸酶抑制剂外用治疗。Objective To investigate clinical and pathological features of lupus erythematosus profundus （LEP） of the scalp in children. Methods A retrospective study was carried out on 5 children with LEP. The clinical and histopathological features, treatment and prognosis of LEP were analyzed. Results The 5 children with LEP included 2 boys and 3 girls with a median age at onset of 5 months （range, 2 - 38 months ） and a median clinical course of 15 months （range, 4 - 72 months ）. Clinically, the patients presented with arc-shaped or circular purple atrophic plaques on the scalp complicated by alopecia. The occipitalia and tempora were the most commonly involved sites. Antinuclear antibodies （ANA） and extractable nuclear antigens （ENAs） were negative in all the patients. Main histopathologieal changes were hyaline degeneration of the fat, mucin deposition and local aggregation of lymphocytes in fat lobules. Of the 5 patients, 2 were treated with oral prednisone （1.5 - 2 mg＆g/day）, 1 with oral hydroxychloroquine （5 mg＆g/day）, 1 with oral prednisone （1.5 mg/kg/day） combined with hydroxychloroquine （5 mg＆g/day）, and another 1 with topical halometasone cream and 0.03% taerolimus ointment. Lesions were remissed after 2 - 3 months of treatment, and subsided with growth of new hairs after 6 months. No recurrence was observed during a 1.5-year follow-up. Conclusion Prednisone and hydroxyehloroquine are markedly effective for LEP, and pediatric patients with LEP may be treated by topical highpotency glueocortieoids and calcineurin inhibitors.

关 键 词：脂膜炎 红斑狼疮性 头皮皮肤病 儿童 皮肤表现 病理过程 治疗结果 

分 类 号：R758.62[医药卫生—皮肤病学与性病学]