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坏死性黄色肉芽肿

Necrobiotic xanthogranuloma.

作者信息

Fernández-Herrera Jesús, Pedraz Javier

机构信息

Department of Dermatology, Hospital Universitario de la Princesa, Madrid, Spain.

出版信息

Semin Cutan Med Surg. 2007 Jun;26(2):108-13. doi: 10.1016/j.sder.2007.02.008.

Abstract

Necrobiotic xanthogranuloma (NXG) is an uncommon granulomatous disease involving the skin and extracutaneous tissues. In its typical clinical presentation, it consists of multiple indurate yellow-brownish plaques or nodules, usually located on the periorbital area. The lesions often are bilateral and symmetric and frequently show tendency to atrophy and ulceration. Most cases are asymptomatic, and oral mucosal erosions are sometimes present. Ophthalmologic complications are observed in many patients, and NXG may involve other extracutaneous sites. Paraproteinemia is closely associated with NXG, demonstrating a monoclonal gammapathy, and other hematologic or lymphoproliferative disorders also may appear in association with NXG. Large areas of degenerated collagen alternating with granulomatous inflammation, multinucleate giant cells, cholesterol clefts, and lymphoid follicles are the most outstanding histopathologic characteristics of NXG. The prognosis for NXG is good, but no curative treatment is available. Chemotherapy with alkylating agents is the most frequently administered treatment, but oral and intralesional corticosteroids, interferon alpha, plasmapheresis, surgical excision, and radiation therapy also have been used for treatment of this disorder, with variable results.

摘要

坏死性黄色肉芽肿(NXG)是一种累及皮肤和皮肤外组织的罕见肉芽肿性疾病。其典型临床表现为多个硬结性黄棕色斑块或结节,通常位于眶周区域。病变常为双侧对称,且常表现出萎缩和溃疡倾向。多数病例无症状,有时可见口腔黏膜糜烂。许多患者会出现眼科并发症,NXG还可能累及其他皮肤外部位。副蛋白血症与NXG密切相关,表现为单克隆丙种球蛋白病,其他血液系统或淋巴增殖性疾病也可能与NXG相关出现。大片变性胶原与肉芽肿性炎症、多核巨细胞、胆固醇裂隙和淋巴滤泡交替出现是NXG最突出的组织病理学特征。NXG的预后良好,但尚无治愈性治疗方法。烷化剂化疗是最常用的治疗方法,但口服和病灶内注射皮质类固醇、干扰素α、血浆置换、手术切除和放射治疗也已用于治疗该疾病,效果不一。

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