| 摘 要：背景：渐进性坏死性黄色肉芽肿是一种非常罕见的组织细胞增多症，临床表现为淡黄色浸润性斑块或结节。80％以上的患者眼部受累。组织病理学表现为大量黄色组织细胞和胶原渐进性坏死。80％的患者可发生良性单克隆丙种球蛋白病相关性骨髓瘤，但其病程较长，10年存活率为100％。病例报道：1例76岁的男性患者出现皮损并继发为溃疡，根据其临床表现和组织病理学检查结果可诊断为渐进性坏死性黄色肉芽肿。未发现单克隆丙种球蛋白病或骨髓瘤。本病另一特点是对糖皮质激素治疗敏感，而对其他疗法无效（环磷酰胺、α干扰素），法语论文题目，糖皮质激素依赖性、医源性Cushing综合征以及糖尿病的发生可能会导致患者的感染性并发症并继发死亡。讨论：即使不伴有骨髓瘤或单克隆丙种球蛋白病，渐进性坏死性黄色肉芽肿也是一种难以治疗的疾病，糖皮质激素可能是最有效的疗法，但可引起多种并发症，法语论文题目，导致类似本特殊病例的死亡。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. |
