伴多系统受累的渐进性坏死性黄色肉芽肿1例

Necrobiotic Xanthogranuloma with Multi-Systems Involvement: A Case Report

患者男,65岁,头皮、眼睑等处黄色斑块逐渐增多5年,近4个月出现声嘶。皮肤科情况:全身片状浸润性、无痛性、界限不清、质韧的黄红色斑块及盘状暗红色斑块。皮损组织病理示:真皮内渐进性坏死与肉芽肿性病变交替分布,以类上皮细胞及泡沫样组织细胞为主,可见Touton巨细胞、胆固醇裂隙和脂质空泡。免疫组织化学染色示:CD68(+)、CD1α(-)、S100(-)。颅脑CT示:颅骨及耳软骨骨质破坏;纤维喉镜示:声带增厚;淋巴结超声示:全身多部位淋巴结肿大;眼科查体示:右眼角膜上皮损害;骨髓细胞学示:骨髓增生减低。诊断:渐进性坏死性黄色肉芽肿,伴多系统损害。静脉滴注甲泼尼龙琥珀酸钠联合口服沙利度胺后,无新发皮损,原有皮损明显变平消退,但声嘶改善不明显,目前仍在随访中。

A 65-year-old male presented with progressive yellow patches on the scalp and eyelids for 5 years,accompanied by hoarseness in the last 4 months.Dermatological examination revealed infiltrating solid yellowish painless patches and plate-like dark red plaques of varying sizes distributed throughout the body.Histopathological examination showed alternating areas of necrosis and xanthogranuloma in the dermis,characterized by epithelioid histiocytes,Touton giant cells,cholesterol clefts and lipid vacuoles.Immunohistochemical staining showed the cells positive expression for CD68,negative expression for CD1a and S100.Systemic examinations revealed osteolytic destruction of the skull and auricular cartilage on cranial computed tomography,vocal cord thickening on fiber laryngoscopy,enlarged lymph nodes in multiple regions on lymph node B ultrasound imaging,corneal epithelium damage and hypomyelosis.The diagnosis of progressive necrobiotic xanthogranuloma with multi-system involvement was made.Treated with intravenous methylprednisolone sodium succinate combined with oral thalidomide,resulted in significant improvement of all skin lesions including softening and shrinkage,but no notable improvement was observed for hoarseness.The patient is under followed up currently.