皮肤黏蛋白病  

Papulonodular mucinosis: a case report

作　　者：季雅娟[1] 黄二顺[1] 雷鹏程[1] 钟延丰[2] 

机构地区：[1]北京大学第三医院皮肤性病科,北京100083 [2]北京大学第三医院病理科,北京100083

出　　处：《临床皮肤科杂志》2009年第1期29-30,共2页Journal of Clinical Dermatology

摘　　要：报告1例皮肤黏蛋白病。患者男,19岁。背部、右上肢结节半年余。皮肤科检查见背部有3枚直径2.5～9.5 cm 的斑块,右上臂伸侧有1枚直径5 cm 的皮肤结节,质地中等有弹性,边界欠清,无红肿及破溃,无压痛。皮损组织病理检查示:真皮胶原稀疏,血管、汗腺、毛附属器周围可见致密的单一核细胞浸润。阿新蓝染色示:真皮、皮下、毛囊周围均见染淡蓝色物质。直接免疫荧光示:免疫球蛋白 IgM、补体 C3基膜带呈线状沉积,免疫球蛋白 IgG、IgA 阴性。电镜检查示:胶原束间出现团状电子密度较高的颗粒状、纤维状物质沉积,未见血管病变。符合皮肤黏蛋白病。A case of papulonodular mucinosis is reported. A 19-year-old male presented with subcutaneous nodules on the right arm and the back for half a year. Dermatologic examination revealed three nodules on the back with a size of 2.5 cm to 9.5 cm in diameter, and one nodule on the right ann with a size of 5 cm in diameter. The lesions were somewhat elastic, without erythema, erosion and tenderness, and ill defined. Histopathological examination of skin specimens showed sparse collagen fibers in the dermis and dense infiltration of mononuclear cells around the capillaries, sweat glands and adnexal structures, and, Alcian-blue positive material around the appendages in the dermis and subcutaneous tissue. Direct immunofluoresence study disclosed a linear deposition of IgM and C3 along the dermal-epidermal junction. A granular and fibrous deposition of electron-dense material between the collagen bundles were seen under the electron microscope. These findings are consistent with the diagnosis of cutaneous mucinosis.

关 键 词：黏蛋白病 皮肤 

分 类 号：R593[医药卫生—内科学]