Chan P T, Lee K C, Chong L Y, Lo K K, Cheung Y F
Social Hygiene Service, Department of Health, Hong Kong Special Administrative Region, China.
Clin Exp Dermatol. 2006 Nov;31(6):775-7. doi: 10.1111/j.1365-2230.2006.02213.x.
A 40-year-old Chinese man presented with sensorimotor polyneuropathy, IgAlambda paraprotein, osteosclerotic bone lesions, hypertrichosis, and impotence with decreased testosterone and raised prolactin level. POEMS (polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy and skin changes) syndrome was diagnosed and he was treated with melphalan and prednisolone. After chemotherapy, other manifestations of POEMS syndrome developed, such as multiple haemangiomas over the chest and neck region, splenomegaly and generalized oedema. One haemangioma had a peculiar clinical morphology, similar to the appearance of cerebral gyri. Skin biopsy confirmed the diagnosis of glomeruloid haemangioma. Chemotherapy was then switched to cyclophosphamide and prednisolone, resulting in further improvement in muscle power and hypertrichosis. To our knowledge, this is the first report on a clinically distinctive morphology of glomeruloid haemangioma, and its recognition may increase the index of suspicion for early skin biopsy.
一名40岁的中国男性出现感觉运动性多发性神经病、IgAλ副蛋白血症、骨硬化性骨病变、多毛症以及伴有睾酮降低和催乳素水平升高的阳痿症状。诊断为POEMS(多发性神经病、器官肿大、内分泌病、单克隆丙种球蛋白病和皮肤改变)综合征,随后他接受了美法仑和泼尼松龙治疗。化疗后,POEMS综合征出现了其他表现,如胸部和颈部区域出现多个血管瘤、脾肿大和全身性水肿。其中一个血管瘤具有特殊的临床形态,类似于脑回的外观。皮肤活检确诊为肾小球样血管瘤。随后化疗方案改为环磷酰胺和泼尼松龙,肌力和多毛症进一步改善。据我们所知,这是关于肾小球样血管瘤临床独特形态的首次报告,对其的认识可能会提高早期皮肤活检的怀疑指数。