Teh Sue-Ann, Kandiah David A
Department of Health Western Australia, Bunbury Hospital, Bunbury.
School of Psychiatry and Clinical Neurosciences, Faculty of Medicine, Dentistry and Health Sciences, University of Western Australia, Crawley, WA, Australia.
Int Med Case Rep J. 2016 Sep 19;9:295-299. doi: 10.2147/IMCRJ.S115315. eCollection 2016.
Scleromyxedema is part of a group of cutaneous mucinoses, characterized by a generalized papular eruption, dermal mucin deposition, and an increase in dermal collagen. This condition can be localized as discrete papular lichen myxedematous skin or as a systemic condition usually associated with paraproteinaemia. To date, there is no unifying treatment and is limited by rarity, small number of case reports, and the lack of randomized controlled trials. We describe the case of a 56-year-old gentleman with features of scleromyxedema who had cutaneous and cardiac involvement, and significant mediastinal lymphadenopathy without monoclonal gammopathy.
硬化性黏液水肿是一组皮肤黏液病的一部分，其特征为全身性丘疹性皮疹、真皮黏液沉积和真皮胶原增加。这种情况可局限为离散的丘疹性黏液水肿性苔藓样皮肤，或为通常与副蛋白血症相关的全身性疾病。迄今为止，尚无统一的治疗方法，且因该病罕见、病例报告数量少以及缺乏随机对照试验而受到限制。我们描述了一名56岁男性硬化性黏液水肿患者的病例，该患者有皮肤和心脏受累，且有明显的纵隔淋巴结肿大但无单克隆丙种球蛋白病。
