Leslie T A, Goldsmith P C, Thompson A J, Dowd P M
Department of Dermatology, University College and Middlesex School of Medicine, London, UK.
Clin Exp Dermatol. 1993 Jul;18(4):344-6. doi: 10.1111/j.1365-2230.1993.tb02214.x.
Malignant atrophic papulosis (Degos disease) is a rare disorder characterized by a vasculopathy of unknown origin. The cutaneous manifestations comprise erythematous papules, which heal to leave scars with a pathognomonic central porcelain-white atrophic area and a peripheral telangiectatic rim. There is usually involvement of the gastrointestinal tract but other organ systems can also be affected, the central nervous system being involved in 20% of cases. It is frequently fatal within 2 or 3 years from onset of systemic involvement, the cause of death usually being intestinal perforation. Our patient is of interest as she has survived an unusually long time despite florid cutaneous and neurological manifestations.
恶性萎缩性丘疹病（德戈斯病）是一种罕见的疾病，其特征为病因不明的血管病变。皮肤表现包括红斑丘疹，愈合后留下疤痕，中央有特征性的瓷白色萎缩区及周边的毛细血管扩张边缘。通常累及胃肠道，但其他器官系统也可受累，20%的病例会累及中枢神经系统。从全身受累开始起病，常在2至3年内死亡，死因通常是肠穿孔。我们的患者很有意思，因为尽管有明显的皮肤和神经表现，但她存活了异常长的时间。
