Chen Hao, Xue Yanning, Jiang Yiqun, Zeng Xuesi, Sun Jian-fang
Institute of Dermatology, Chinese Academy of Medical Sciences and Peking Union Medical College, Nanjing, China.
Am J Dermatopathol. 2012 Jul;34(5):553-6. doi: 10.1097/DAD.0b013e3181f498e2.
Cutaneous and systemic plasmacytosis (CSP) is a rare disorder characterized by disseminated reddish brown plaques and polyclonal hypergammaglobulinemia. The lesions of CSP are histologically characterized by an infiltration of mature polyclonal plasma cells, which display similar pathological features to the plasma cell-type Castleman disease (CD). The relationship between CSP and CD is controversial. Herein, we described a 43-year-old man from China with disseminated reddish brown plaques and nodules on the cheek and temple. The serum level of immunoglobulin G and immunoglobulin A were higher than normal. In addition to mature plasma cell perivascular infiltrate in the dermis, the biopsy of the lesions showed small to medium-sized germinal follicles with hyalinized vessels and a concentrically arranged mantle zone. The patient had clinical features of CSP, but the biopsy revealed changes resembling mixed-type CD. To the best of our knowledge, this is the first case of CSP with the pathological features of mixed-type CD reported from China.
皮肤和系统性浆细胞增多症(CSP)是一种罕见的疾病,其特征为散在的红棕色斑块和多克隆高球蛋白血症。CSP的皮损在组织学上的特征是成熟多克隆浆细胞浸润,其显示出与浆细胞型Castleman病(CD)相似的病理特征。CSP与CD之间的关系存在争议。在此,我们描述了一名来自中国的43岁男性,其面颊和颞部有散在的红棕色斑块和结节。血清免疫球蛋白G和免疫球蛋白A水平高于正常。除了真皮中成熟浆细胞血管周围浸润外,病变活检显示有小到中等大小的生发滤泡,伴有玻璃样变的血管和同心排列的套区。该患者具有CSP的临床特征,但活检显示出类似混合型CD的改变。据我们所知,这是中国报道的首例具有混合型CD病理特征的CSP病例。