Behera Biswanath, Malathi Munisamy, Thappa Devinder-Mohan, Vamanshankar Hemanth, Parida Pradipta-Kumar, Gochhait Debasis
Department of Dermatology, Venereology and Leprology, JIPMER, Puducherry, India.
Department of ENT, JIPMER, Puducherry, India.
Iran J Otorhinolaryngol. 2017 Nov;29(95):365-368.
Xanthoma disseminatum (XD) is a rare, benign, non-Langerhans cell histiocytic disorder with unknown etio-pathology. It manifests with multiple, grouped, red-brown to yellow papules and nodules involving the skin, mucous membranes, and internal organs with a predilection for flexures and the face.
We report a patient who presented with disseminated xanthomatous papules and nodules involving the face, neck, trunk, axilla, groin, and oral cavity, along with hoarseness of voice. Video laryngoscopy revealed multiple yellowish nodules over the base of the tongue, vallecula, laryngeal surface of the epiglottis, ary-epiglottic folds, interarytenoid region, and subglottic region. Histopathology was suggestive of xanthoma disseminatum and the patient was treated with tablet acitretin 25mg daily for three months without any response. Following this, the patient was prescribed tablet thalidomide 100 mg daily without any significant improvement at the end of two months.
Xanthoma disseminatum is a very rare form of non-Langerhans cell histiocytosis that classically presents with cutaneous xanthomas, mucosal xanthomas, and diabetes insipidus. Hoarseness of voice due to lesions involving the larynx is a rare symptom. Because the disease has punctated, numerous relapses and causes morbidity to the patient, its multisystem manifestations have to be known. Therefore, xanthoma disseminatum has to be kept in mind as a differential diagnosis for hoarseness of voice.
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生症,病因病理不明。其表现为多发性、成群的红棕色至黄色丘疹和结节,累及皮肤、黏膜及内脏器官,好发于屈侧和面部。
我们报告一例患者,其面部、颈部、躯干、腋窝、腹股沟及口腔出现播散性黄瘤性丘疹和结节,并伴有声音嘶哑。电子喉镜检查显示舌根部、会厌谷、会厌喉面、杓会厌襞、杓间区及声门下区有多个淡黄色结节。组织病理学提示为播散性黄瘤,患者每日服用25mg阿维A胶囊三个月,无任何反应。此后,患者每日服用100mg沙利度胺片,两个月后无明显改善。
播散性黄瘤是一种非常罕见的非朗格汉斯细胞组织细胞增生症,典型表现为皮肤黄瘤、黏膜黄瘤和尿崩症。因喉部病变导致声音嘶哑是一种罕见症状。由于该病有散在、多次复发且给患者带来痛苦,必须了解其多系统表现。因此,播散性黄瘤必须作为声音嘶哑的鉴别诊断之一予以考虑。