Liu Fang, Liu Haibo, Zhang Min, Yan Wenliang, Sang Hong
Jinling Hospital, Medical School, Nanjing University, Nanjing, CN.
An Bras Dermatol. 2015 May-Jun;90(3 Suppl 1):19-21. doi: 10.1590/abd1806-4841.20153490.
Malignant atrophic papulosisis is a rare, multisystem obliterative vasculopathy of unknown etiology, occasionally involving the cranial nerve. We describe the first case of malignant atrophic papulosisis with cranial nerve and peripheral nerve involvement in China. A 47-year-old woman presented to our hospital with atrophic porcelain white papules over the trunk and extremities, numbness in the right calf, vision decrease and impaired movement of the right eye. She was diagnosed with malignant atrophic papulosisis, based on characteristic symptoms and histopathologic examination. The patient was treated with dipyridamole and aspirin for 9 months, but later died of gastrointestinal hemorrhage. We reviewed currently available case reports on cranial nerve involvement in malignant atrophic papulosisis and emphasized the importance of skin biopsy in diagnosing this disease.
恶性萎缩性丘疹病是一种病因不明的罕见的多系统闭塞性血管病，偶尔累及脑神经。我们报道了中国首例伴有脑神经和周围神经受累的恶性萎缩性丘疹病。一名47岁女性因躯干和四肢出现萎缩性瓷白色丘疹、右小腿麻木、视力下降和右眼活动障碍就诊于我院。根据特征性症状和组织病理学检查，她被诊断为恶性萎缩性丘疹病。该患者接受双嘧达莫和阿司匹林治疗9个月，但后来死于胃肠道出血。我们回顾了目前关于恶性萎缩性丘疹病累及脑神经的病例报告，并强调了皮肤活检在诊断该病中的重要性。
