Cosgarea R, Cosgarea M, Turcu T
Clinique Dermatologique, Université de Médecine Cluj-Napoca, Romania.
Ann Dermatol Venereol. 1994;121(2):159-61.
We observed a case of scleromyxoedema with specific cutaneous lesions in a 62-year-old man. These lesions were associated with modifications of the central nervous system, latent thyroid failure, cardiovascular changes and myopathy, usual manifestations of this disease, but without paraproteinaemia. Unusual laryngeal manifestations were a dysphonia with chronic pseudomyxomatosis of the larynx. Urinary mucopolysaccharide excretion was increased. It is important to underscore the favourable response to Thiomucase when more toxic drugs had become ineffective.
我们观察了一名62岁男性的硬皮病样黏液水肿病例，其具有特定的皮肤损害。这些损害与中枢神经系统改变、潜在甲状腺功能减退、心血管变化及肌病相关，这些都是该疾病的常见表现，但无副蛋白血症。不常见的喉部表现为声音嘶哑伴喉部慢性黏液瘤病。尿黏多糖排泄增加。重要的是要强调，当毒性更强的药物无效时，硫黏蛋白酶有良好疗效。
