Hara M, Mizutani T, Tamura M, Ono S, Oishi M, Hanawa S
Department of Neurology, Nihon University School of Medicine.
Rinsho Shinkeigaku. 1991 Sep;31(9):1025-8.
A 41-year-old man with PPP since in 1982 was admitted in May, 1987, because of the progressive asymmetric sensory disturbance in the hands and feet over 4 months, accompanied by an exacerbation of PPP. On admission, eruptions of PPP were observed in the palms and soles. Asymmetric and mildly decreased sensations of touch and pain were present in the distal part of the four extremities as well as in his trunk, accompanied by paresthesia and dysesthesia. Mild to moderate weakness was noted in the hand muscles, and slight muscular atrophy was present in the right lower leg. A work-up for collagen vascular disease was within normal limits. T lymphocyte subset showed a decreased ratio of OKT 4/OKT 8. Left sural nerve biopsy showed axonal degeneration and moderate decrease of myelinated fibers, and the vasculitis was not found. The neurological signs and symptoms as well as the skin eruptions improved with methylprednisolone 40 mg/day. A causal relationship between the multiple mononeuropathy and PPP of our patient was indicated by the almost simultaneous onset of the neuropathy and the exacerbation of PPP as well as the improvement of these two conditions with corticosteroid therapy. Such combination of multiple mononeuropathy and PPP has not so far been reported.
一名自1982年起患有进行性色素性紫癜性皮病(PPP)的41岁男性于1987年5月入院,原因是4个月来手足部进行性不对称感觉障碍,并伴有PPP病情加重。入院时,在手掌和足底观察到PPP皮疹。四肢远端及躯干存在不对称且轻度减退的触觉和痛觉,伴有感觉异常和感觉迟钝。手部肌肉有轻度至中度无力,右小腿存在轻度肌肉萎缩。胶原血管病的相关检查结果正常。T淋巴细胞亚群显示OKT 4/OKT 8比值降低。左侧腓肠神经活检显示轴索变性和有髓纤维中度减少,未发现血管炎。给予甲泼尼龙40mg/天治疗后,神经症状和体征以及皮肤皮疹均有所改善。该患者的多发性单神经病与PPP之间的因果关系体现在神经病几乎与PPP病情加重同时出现,以及这两种情况经皮质类固醇治疗后均得到改善。迄今为止,尚未有这种多发性单神经病与PPP合并出现的报道。