Dutta Abhijit, Ghosh Sudip Kumar, Majumder Biswajit, Majumdar Rahul
Department of Dermatology, Venereology, & Leprosy, R G Kar Medical College, Kolkata.
Dermatol Online J. 2016 Sep 15;22(9):13030/qt86r7s857.
Woolly hair may occur as an isolated problem of cosmetic concern or can be a part of a systemic disease (woolly hair syndrome) with underlying fatal cardiomyopathy. Two characteristic associations of woolly hair syndrome are Naxos disease and Carvajal syndrome. Naxos disease is characterized by woolly hair, palmoplantar keratoderma, and arrythmogenic right ventricular cardiomyopathy.In this report we describe a case of a young girl who presented with heart failure and was subsequently diagnosed as a case of generalized woolly hair with biventricular arrythmogenic cardiomyopathy.Our case represented a rare variant of Naxos disease in the advanced stage of arrythmogenic right ventricular cardiomyopathy; biventricular failure may occur with involvement of the interventricular septum and left ventricle causing congestive heart failure.
羊毛状发可能作为一个仅涉及美观问题的孤立情况出现，也可能是伴有潜在致命性心肌病的全身性疾病（羊毛状发综合征）的一部分。羊毛状发综合征有两个典型关联疾病，即纳克索斯病和卡瓦哈尔综合征。纳克索斯病的特征为羊毛状发、掌跖角化病和致心律失常性右室心肌病。在本报告中，我们描述了一名年轻女孩的病例，她出现心力衰竭，随后被诊断为患有双心室致心律失常性心肌病的全身性羊毛状发。我们的病例代表了纳克索斯病在致心律失常性右室心肌病晚期的一种罕见变异型；当室间隔和左心室受累导致充血性心力衰竭时，可能会出现双心室衰竭。
