Access to the text (HTML) Access to the text (HTML)
PDF Access to the PDF text

Access to the full text of this article requires a subscription.
  • If you are a subscriber, please sign in 'My Account' at the top right of the screen.

  • If you want to subscribe to this journal, see our rates

Journal of the American Academy of Dermatology
Volume 39, n° 3
pages 418-421 (septembre 1998)
Doi : 10.1016/S0190-9622(98)70317-2
accepted : 14 May 1998
Epidermolytic palmoplantar keratoderma with woolly hair and dilated cardiomyopathy

Luis Carvajal-Huerta, MD
Luis Vernaza Hospital. Guayaquil, Ecuador 


Background: A new cardiocutaneous syndrome has been noted, characterized by an epidermolytic palmoplantar keratoderma and woolly hair, and associated with dilated cardiomyopathy. Objective: This describes the clinical and histopathologic characteristics of this new syndrome. Methods: 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. Results: 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. Conclusion: 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. (J Am Acad Dermatol 1998;39:418-21.)

The full text of this article is available in PDF format.

 Reprint requests: Luis Carvajal-Huerta, PO Box 5040, Luis Vernaza Hospital, Guayaquil, Ecuador.
 0190-9622/98/$5.00 + 0  16/1/91713

© 1998  American Academy of Dermatology, Inc. Published by Elsevier Masson SAS@@#104157@@
EM-CONSULTE.COM is registrered at the CNIL, déclaration n° 1286925.
As per the Law relating to information storage and personal integrity, you have the right to oppose (art 26 of that law), access (art 34 of that law) and rectify (art 36 of that law) your personal data. You may thus request that your data, should it be inaccurate, incomplete, unclear, outdated, not be used or stored, be corrected, clarified, updated or deleted.
Personal information regarding our website's visitors, including their identity, is confidential.
The owners of this website hereby guarantee to respect the legal confidentiality conditions, applicable in France, and not to disclose this data to third parties.
Article Outline
You can move this window by clicking on the headline