布加综合征和单克隆丙种球蛋白病患者的弥漫性扁平黄瘤病

Koçak Mukadder, Keleş Hatice, Yakaryilmaz Fahri, Bozdoğan Onder, Güliter Sefa

Department of Dermatology, University of Kirikkale, School of Medicine, Ankara, Turkey.

Indian J Dermatol. 2009;54(4):369-71. doi: 10.4103/0019-5154.57616.

Diffuse plane xanthomas are characterized by the presence of yellowish plaques on the eyelids, neck, upper trunk, buttocks, and flexural folds. Histology shows foamy histiocytes in the dermis. Approximately half of the cases are associated with lymphoproliferative disorders. Budd-Chiari syndrome is an uncommon condition induced by thrombotic or nonthrombotic obstruction of hepatic venous outflow. We present a case of diffuse plane xanthoma in a 62-year-old man who developed normolipemic plane xanthomas coinciding with Budd-Chiari syndrome and monoclonal gammopathy. We review the English-language literature regarding the rare association of xanthomas and Budd-Chiari syndrome.

弥漫性扁平黄瘤的特征是在眼睑、颈部、上躯干、臀部和屈侧褶皱处出现淡黄色斑块。组织学显示真皮中有泡沫状组织细胞。大约一半的病例与淋巴增殖性疾病有关。布加综合征是一种由肝静脉流出道血栓形成或非血栓形成性阻塞引起的罕见病症。我们报告一例62岁男性的弥漫性扁平黄瘤病例，该患者出现了血脂正常的扁平黄瘤，同时伴有布加综合征和单克隆丙种球蛋白病。我们回顾了关于黄瘤与布加综合征罕见关联的英文文献。