累及神经系统的恶性萎缩性丘疹病

Malignant atrophic papulosis with neurological involvement

患者女，48岁，全身丘疹、斑块6年伴右眼视物不清1年就诊。体检：躯干、四肢可见瓷白色萎缩性丘疹，外周伴红晕及毛细血管扩张。右眼右侧斜视受限，视力减退，视野缺损。左手食指、拇指麻木，右侧胫骨前麻木。腹部皮损组织病理检查：真皮层坏死伴黏液样变性，深部数个小血管周围见淋巴细胞为主的炎细胞浸润，数个小血管腔闭塞。肠镜检查：全结肠散在片状充血、糜烂、浅溃疡形成；肌电图：股四头肌源性损害；粪隐血＋＋＋。诊断：恶性萎缩性丘疹病。治疗：口服阿司匹林、双嘧达莫治疗3个月，病情无进展。

A 48-year-old female presented with a 6-year history, of papules and plaques all over the body and with 1-year history of blurred vision in the right eye. Physical examination showed porcelain-white atrophic papules with peripheral erythematous halo and telangiectasia. She also suffered from exotropia, visual deterioration, visual field defects of the right eye, as well as numbness of the left index finger, thumb and right anterior tibia. Skin biopsies of abdominal lesions revealed dermal necrosis with mucoid degeneration, inflammatory infiltration predominated by lymphocytes around several small blood vessels and occlusion of some blood vessels in deep dermis. Colonoscopy of the whole colon demonstrated scattered patches of hyperemia and erosions with the formation of shallow ulcers. Nerve electromyologram revealed damage to the nerves of fight quadriceps femoris muscles. Fecal analysis showed that occult blood was strongly positive. A diagnosis of malignant atrophic papulosis was made based on the characteristic clinical presentation, laboratory and histopathological findings. She was treated with dipyridamole and aspirin for three months, which resulted in no clinical improvement or deterioration.