不伴单克隆丙种球蛋白病的渐进性坏死性黄色肉芽肿导致迅速死亡(法语)  

Necrobiotic xanthogranuloma without monoclonal gammopathy and with a rapidly fatal outcome (Fren)

作　　者：Bernez A. Abdallah-Lotf M. D’Incan M. L.Machet 王琼 

机构地区：[1]Service de Dermatologie, Groupement Peau et Morphologie, Hopital Trousseau, 37044 Tours Cedex 01

出　　处：《世界核心医学期刊文摘（皮肤病学分册）》2006年第9期24-25,共2页Digest of the World Core Medical JOurnals:Dermatology

摘　　要：Background. Necrobiotic xanthogranuloma is an extremely rare form of histiocytosis that presents clinically as yellowish infiltrated plaques or nodules. Ocular involvement is seen in over 80% of cases. Histopathology reveals numerous xanthomous histiocytes and collagen necrobiosis. Benign monoclonal gammopathy associated with myeloma is found in 80% of patients, but the course is normally long, with 100% survival at 10 years. Case report. A 76-year-old man presented skin lesions that subsequently became ulcerated. The clinical appearance and histopathological examination resulted in diagnosis of necrobiotic xanthogranuloma. No monoclonal gammopathy or myeloma was seen. The disease was marked by sensitivity to corticosteroids with failure of other therapies (cyclophosphamide, alpha interferon), onset of corticosteroid dependency, iatrogenic Cushing’ s syndrome and diabetes, which were in part responsible for the infectious complications and subsequent death of the patient. Discussion. Necrobiotic xanthogranuloma is difficult to treat, even in the absence of myeloma or monoclonal gammopathy. Corticosteroids are probably the most efficacious treatment, but can give rise to multiple complications, resulting in this particular case in death of the patient.Background. Necrobiotic xanthogranuloma is an extremely rare form of histiocytosis that presents clinically as yellowish infiltrated plaques or nodules. Ocular involvement is seen in over 80% of cases. Histopathology reveals numerous xanthomous histiocytes and collagen necrobiosis. Benign monoclonal gammopathy associated with myeloma is found in 80% of patients, but the course is normally long, with 100% survival at 10 years. Case report. A 76-year-old man presented skin lesions that subsequently became ulcerated. The clinical appearance and histopathological examination resulted in diagnosis of necrobiotic xanthogranuloma. No monoclonal gammopathy or myeloma was seen. The disease was marked by sensitivity to corticosteroids with failure of other therapies （cyclophosphamide, alpha interferon）, onset of corticosteroid dependency, iatrogenic Cushing＇s syndrome and diabetes, which were in part responsible for the infectious complications and subsequent death of the patient.Discussion. Necrobiotic xanthogranuloma is difficult to treat, even in the absence of myeloma or monoclonal gammopathy. Corticosteroids are probably the most efficacious treatment, but can give rise to multiple complications, resuiting in this particular case in death of the patient.

关 键 词：单克隆丙种球蛋白病 黄色肉芽肿 渐进性坏死 坏死性 死亡 组织细胞增多症 CUSHING综合征 感染性并发症 

分 类 号：R553[医药卫生—血液循环系统疾病]