Attia A M, Bakry O A, Mohamed E E
Department of Dermatology, Andrology and Sexually Transmitted Diseases, Faculty of Medicine, Menoufiya University, Menoufiya, Egypt.
J Postgrad Med. 2014 Jan-Mar;60(1):69-71. doi: 10.4103/0022-3859.128817.
Xanthoma disseminatum (XD) is a rare, benign, non-Langerhans cell histiocytic disorder. The pathogenesis is not clear. It manifests with multiple, grouped, red-brown to yellow papules and nodules involving the skin, mucous membranes, and internal organs. We present a case of progressive XD in a 10-year-old male child. The patient presented with progressive, bilateral and symmetrical, reddish-brown, coalescent papules on the neck, around both eyes and all over his trunk and extremities. Skin lesions were accompanied by blurred vision and hoarseness of voice. Examination revealed xanthomatous infiltration of cornea, oral, pharyngeal, and laryngeal mucosae. The patient had diabetes insipidus that was diagnosed 2 years before the appearance of skin lesions. Medical treatment with corticosteroids (20 mg/day) and azathioprine (2 mg/kg/day) did not stop the disease progression.
播散性黄瘤（XD）是一种罕见的良性非朗格汉斯细胞组织细胞增多症。其发病机制尚不清楚。表现为多发、成簇的红棕色至黄色丘疹和结节，累及皮肤、黏膜及内脏器官。我们报告一例10岁男性儿童的进行性XD病例。该患者颈部、双眼周围、躯干及四肢出现进行性、双侧对称的红棕色融合丘疹。皮肤病变伴有视力模糊和声音嘶哑。检查发现角膜、口腔、咽喉黏膜有黄瘤样浸润。该患者在皮肤病变出现前2年被诊断为尿崩症。使用皮质类固醇（20毫克/天）和硫唑嘌呤（2毫克/千克/天）进行药物治疗未能阻止疾病进展。
