结节性皮肤狼疮黏蛋白病1例并文献复习

A case of nodular cutaneous lupus mucinosis and literature review

目的报告1例结节性皮肤狼疮黏蛋白病并对相关文献进行复习,提高临床医生对该病的认识。方法收集1例结节性皮肤狼疮黏蛋白病患者的临床特点、组织病理等结果并进行分析。结果患者男,40岁。面颊部可见不规则淡红色斑片,上覆少许鳞屑,部分萎缩,胸背部、腹部及双上肢近端可见多发密集绿豆大小皮色结节。抗核抗体阳性。早期结节病理可见真皮黏蛋白沉积,后期红斑病理符合典型结节性皮肤狼疮黏蛋白病表现,可见毛囊角栓,表皮萎缩,灶性基底细胞液化变性,真皮浅层血管及附属器周围淋巴细胞浸润及较多黏蛋白沉积。直接免疫荧光示IgM、C3基底膜荧光阳性。目前无肾脏、血液等系统受累。结合临床及辅助检查诊断为结节性皮肤狼疮黏蛋白病。患者对系统应用糖皮质激素联合羟氯喹治疗应答反应好。结论结节性皮肤狼疮黏蛋白病作为一类临床少见且发病机制不明的罕见病,皮肤科医师在熟悉其典型皮疹及病理改变的情况下,仍有可能在临床上早期识别并诊断该疾病。皮肤科医师应加强对该病的认识和警惕。

Objective To report a case of nodular cutaneous lupus mucinosis and review relevant literature,in order to improve understanding of the disease.Methods We analyzed the clinical and histopathological features of a patient with nodular cutaneous lupus mucinosis.Results A 40 years old man presented with irregular erythema,with scales and partial atrophy on the cheeks,and multiple dense green bean-sized skin-colored nodules on his chest,back,abdomen,and proximal upper limbs.Antinuclear antibody was positive.Pathology of the early nodule showed dermal mucin deposition,and the pathology of later erythema was consistent with typical nodular cutaneous lupus mucinosis,with follicular keratotic plugs,epidermal atrophy,focal liquefaction of basal cells,and perivascular and peri-appendage infiltration of lymphocytes,as well as mucin deposition in the dermis.Direct immunofluorescence showed IgM and C3 positive at basement membrane.There was no renal nor hematologic involvement.The patient was diagnosed with nodular cutaneous lupus mucinosis and responded well to the treatments with systemic glucocorticoid in combination with hydroxychloroquine.Conclusions Although nodular cutaneous lupus mucinosis is a rare disease with unknown pathogenesis,dermatologists can still be able to make early diagnosis if they are familiar with its typical skin features and pathological changes.Dermatologists should strengthen their understanding of and be vigilance towards this disease.