Carvajal-Huerta L
Luis Vernaza Hospital, Guayaquil, Ecuador.
J Am Acad Dermatol. 1998 Sep;39(3):418-21. doi: 10.1016/s0190-9622(98)70317-2.
A new cardiocutaneous syndrome has been noted, characterized by an epidermolytic palmoplantar keratoderma and woolly hair, and associated with dilated cardiomyopathy.
This describes the clinical and histopathologic characteristics of this new syndrome.
Eighteen patients were examined clinically and histologically from 1970 to 1997. Cardiologic examinations were performed in 12 patients. The cutaneous lesions were classified according to the presence of obligatory and facultative elements of the syndrome.
Patients were born with woolly hair. Around the first year palmoplantar keratoderma and the other keratotic elements appeared. The first cardiac abnormalities are exclusively electrocardiographic and occur in asymptomatic patients. In these patients, dilation of the left ventricle together with alterations in muscle contractility are observed. The dilated cardiomyopathy can lead to congestive heart failure and death.
The association of woolly hair and palmoplantar keratoderma with a histopathologic pattern of epidermolytic hyperkeratosis has not been previously described. Their frequent association with dilated cardiomyopathy defines a cardiocutaneous syndrome. Whenever woolly hair is associated with any kind of palmoplantar keratoderma, a search for possible cardiac abnormalities is recommended.
已发现一种新的心皮综合征，其特征为表皮松解性掌跖角化病和羊毛状毛发，并与扩张型心肌病相关。
描述这种新综合征的临床和组织病理学特征。
1970年至1997年对18例患者进行了临床和组织学检查。对12例患者进行了心脏检查。根据综合征的必需和可选要素的存在对皮肤病变进行分类。
患者出生时即有羊毛状毛发。在第一年左右出现掌跖角化病和其他角化性病变。最初的心脏异常仅通过心电图检测到，且发生在无症状患者中。在这些患者中，观察到左心室扩张以及肌肉收缩力改变。扩张型心肌病可导致充血性心力衰竭和死亡。
羊毛状毛发和掌跖角化病与表皮松解性角化过度的组织病理学模式之间的关联此前尚未见报道。它们与扩张型心肌病的频繁关联定义了一种心皮综合征。每当羊毛状毛发与任何类型的掌跖角化病相关时，建议排查可能存在的心脏异常。
