伴IgA-κ型副球蛋白血症的渐进性坏死性黄色肉芽肿1例

A Case of Necrobiotic Xanthogranuloma with Immunoglobulin A Kappa Paraproteinemia

患者男,57岁,全身多发皮下结节伴体重减轻4个月。皮肤科情况:头面部、躯干、四肢泛发大小不等黄红色、紫红色结节。血清免疫固定电泳:单克隆Ig A,Kappa轻链。组织病理:真皮内大量组织细胞、泡沫细胞及Touton巨细胞浸润,未见明显胶原渐进性坏死。诊断:伴IgA-κ型副球蛋白血症的渐进性坏死性黄色肉芽肿。

A 57-year-old male suffered subcutaneous nodules in the whole body with weight losing for 4 months. Physical examination showed multipleyellow-red or purple-red nodules at different sizes covering the head, face, trunk and limbs. A monoclonal IgA kappa light chains were detected by serum immunofixation electrophoresis. Histopathological examination revealedmuhiple histiocytes, foam cellsand Touton giant cellsinfiltratewithout necrobiotic collagen. A diagnosis ofnecrobiotic xanthogranuloma with immunoglobulin akappa paraproteinemia was made.