关键词: Scleredema cardiomyopathy myocardial biopsy skin thickening

来  源:   DOI:10.1177/23971983211013977   PDF(Sci-hub)   PDF(Pubmed)

Abstract:
Scleredema is a rare cutaneous mucinosis characterized by diffuse swelling and non-pitting induration. A 63-year-old man reported a 5-year history of skin thickening of the trunk and a 3-week history of dyspnea. Echocardiography revealed diffuse hypokinesis. Skin biopsies obtained from the waist showed thickened dermis with mucin. Myocardial biopsies showed alcian blue-stained tissue between the muscle fibers. The patient was referred to a dermatologist for phototherapy. Cardiomyopathy should be considered in patients with scleredema. Scleredema usually has a good prognosis; however, the mortality risk could be high when accompanied by cardiomyopathy.
摘要:
Scleedema是一种罕见的皮肤粘液病,其特征是弥漫性肿胀和非点蚀性硬结。一名63岁的男子报告有5年的躯干皮肤增厚史和3周的呼吸困难史。超声心动图显示弥漫性运动功能减退。从腰部获得的皮肤活检显示真皮增厚,粘蛋白。心肌活检显示肌纤维之间有阿尔辛蓝染色的组织。患者被转诊给皮肤科医生进行光疗。患有硬肿症的患者应考虑心肌病。Screedema通常预后良好;然而,当伴有心肌病时,死亡风险可能很高.
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