Kim Jun Young, Jung Hong Dae, Choe Yoon Seok, Lee Weon Ju, Lee Seok-Jong, Kim Do Won, Kim Byung Soo
Department of Dermatology, Kyungpook National University School of Medicine, Daegu, Korea.
Ann Dermatol. 2010 Aug;22(3):353-7. doi: 10.5021/ad.2010.22.3.353. Epub 2010 Aug 5.
Xanthoma disseminatum (XD) is a rare, benign non-familial mucocutaneous disorder, which is a subset of non-Langerhans cell histiocytosis. It is characterized by mucocutaneous xanthomas in a disseminated form typically involving the eyelids, trunk, face, and proximal extremities and occurs in flexures and folds such as axillae and the groin. Mucosal involvement of the respiratory or gastrointestinal tracts may lead to hoarseness or intestinal obstruction from a mechanical mass effect. This paper outlines the case of a 47-year-old female with progressive yellow-to-brown confluent nodules and plaques of various sizes on her scalp, face, oral mucosa, neck, shoulder, axillary folds, and perianal area. Xanthomas accentuating over the eyelids and eyelashes led to partial obstruction of her visual field and interfered with blinking. Further, she suffered from xerophthalmia. The presentation of histopathological features including foamy histiocytes, inflammatory cells, and Touton giant cells in conjunction with her clinical findings indicated a diagnosis of XD. Evaluations for extracutaneous involvement including the central nervous system, respiratory tract, gastrointestinal tract, and bone resulted in nonspecific findings. Although she has been treated with surgical excisions, CO(2) laser therapy, and oral prednisolone, new lesions are still emerging.
播散性黄瘤（XD）是一种罕见的、非家族性的良性黏膜皮肤疾病，是非朗格汉斯细胞组织细胞增多症的一个亚型。其特征为播散性的黏膜皮肤黄瘤，通常累及眼睑、躯干、面部及近端肢体，也见于腋窝和腹股沟等褶皱部位。呼吸道或胃肠道的黏膜受累可能导致因机械性肿块效应引起的声音嘶哑或肠梗阻。本文概述了一名47岁女性的病例，她的头皮、面部、口腔黏膜、颈部、肩部、腋窝褶皱和肛周区域出现了大小不一的、逐渐增多的黄色至棕色融合性结节和斑块。眼睑和睫毛上突出的黄瘤导致她部分视野受阻并影响眨眼。此外，她还患有干眼症。组织病理学特征包括泡沫状组织细胞、炎症细胞和杜顿巨细胞，结合她的临床症状，诊断为XD。对包括中枢神经系统、呼吸道、胃肠道和骨骼在内的皮肤外受累情况的评估结果不具有特异性。尽管她接受了手术切除、二氧化碳激光治疗和口服泼尼松龙治疗，但仍有新的病变出现。
