Burrow J N, Blumbergs P C, Iyer P V, Hallpike J F
Royal Adelaide Hospital, SA.
Aust N Z J Med. 1991 Feb;21(1):49-51. doi: 10.1111/j.1445-5994.1991.tb03002.x.
Kohlmeier-Degos disease, or malignant atrophic papulosis, is a rare and clinically distinctive vasculopathy characterised by cutaneous features with frequent gastrointestinal and neurological involvement and, almost invariably, with fatal outcome. The case is reported of a 22-year-old male who died of progressive cerebral infarction nine months from the time of development of pathognomonic skin lesions. Treatments with anti-platelet agents and plasma exchange were ineffectual in altering the progressive course of the disease. At autopsy, there was an extensive small vessel occlusive vasculopathy with similar features in skin, brain and bowel wall. The aetiology of Kohlmeier-Degos disease remains unknown. Diagnosis is on clinico-pathological grounds. Awareness of the disease is important, particularly in the differential diagnosis of cerebrovascular-arteritic-syndromes presenting in young adults.
科尔迈尔-德戈斯病，即恶性萎缩性丘疹病，是一种罕见的、具有临床特征性的血管病，其特点是皮肤表现常伴有胃肠道和神经系统受累，且几乎无一例外会导致致命后果。报告了一例22岁男性病例，该患者自出现特征性皮肤病变起9个月后死于进行性脑梗死。使用抗血小板药物和血浆置换治疗未能改变疾病的进展过程。尸检发现广泛的小血管闭塞性血管病，皮肤、脑和肠壁具有相似特征。科尔迈尔-德戈斯病的病因仍不明。诊断基于临床病理依据。认识这种疾病很重要，尤其是在对年轻成人出现的脑血管动脉炎性综合征进行鉴别诊断时。
