McFarland H R, Wood W G, Drowns B V, Meneses A C
Ann Neurol. 1978 May;3(5):388-92. doi: 10.1002/ana.410030504.
Malignant atrophic papulosis usually presents as pathognomonic skin lesions followed by acute abdominal pain, bowel perforation, peritonitis, and death. Rare patients who may lack gastrointestinal symptoms present with central nervous system manifestations, including headache, paresthesias, weakness, and rapid deterioration to death. The patient reported here was a 47-year-old man whose neurological symptoms apparently preceded his cutaneous lesions. His course consisted of a disseminated neurological disease and exacerbated following a herpes zoster infection. His condition rapidly deteriorated despite corticotropin, glucocorticoids, and low-molecular-weight dextran. Necropsy revealed a disseminated occlusive vasculopathy and diffuse encephalomyelomalacia of the brain and spinal cord. A review of autopsied patients with central nervous system involvement is provided.
恶性萎缩性丘疹病通常表现为特征性皮肤损害，随后出现急性腹痛、肠穿孔、腹膜炎及死亡。少数可能无胃肠道症状的患者会出现中枢神经系统表现，包括头痛、感觉异常、无力，并迅速恶化至死亡。本文报道的患者为一名47岁男性，其神经症状明显先于皮肤损害出现。其病程为播散性神经疾病，在带状疱疹感染后加重。尽管使用了促肾上腺皮质激素、糖皮质激素及低分子右旋糖酐，其病情仍迅速恶化。尸检显示为播散性闭塞性血管病以及脑和脊髓的弥漫性脑脊髓软化。本文提供了对有中枢神经系统受累的尸检患者的回顾。
