Barrow Neurological Institute, 350 W Thomas Road, Phoenix, AZ 85013, United States.
J Neurol Sci. 2011 Jan 15;300(1-2):157-9. doi: 10.1016/j.jns.2010.10.007. Epub 2010 Oct 29.
Degos' disease or malignant atrophic papulosis is a rare disseminated occlusive vasculopathy affecting the skin, gastrointestinal tract, central nervous system, and less often other organ systems. The exact etiology of this vasculopathy has not been established. Infections, autoimmune disease and coagulation defects have been proposed as underlying pathogenic mechanisms, but none have been confirmed. Here, we report the clinical, radiological and histopathologic features of Degos' disease in a 41-year-old man following streptococcal throat infection. Prior postulated hypothesis as post-infectious immunologic mechanism may be further supported by this case.
德戈斯病或恶性萎缩性丘疹病是一种罕见的弥漫性闭塞性血管病，影响皮肤、胃肠道、中枢神经系统，较少影响其他器官系统。这种血管病的确切病因尚未确定。感染、自身免疫性疾病和凝血缺陷已被提议作为潜在的发病机制，但均未得到证实。在此，我们报告了一例 41 岁男性在链球菌性咽炎感染后发生德戈斯病的临床、影像学和组织病理学特征。此前提出的感染后免疫机制假说可能通过该病例得到进一步支持。
