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https://hdl.handle.net/11499/5287
Title: | Mal de Meleda: A new geographical localization in Anatolia | Authors: | Ergin, Şeniz Şanli Erdogan, Berna Aktan, Şebnem |
Keywords: | Anatolia Hereditary palmoplantar keratoderma Mal de Meleda adolescent adult article child clinical article clinical examination clinical feature controlled study edema familial disease female finger malformation foot disease foot sole hand disease hand palm health care planning human hyperhidrosis infant interview keratosis palmoplantaris mal de meleda male pedigree prevalence priority journal Turkey (republic) Adolescent Adult Child Child, Preschool Consanguinity Female Humans Infant Keratoderma, Palmoplantar Male Middle Aged Pedigree Turkey |
Abstract: | Background: Mal de Meleda (MDM) is an autosomal recessive form of palmoplantar keratoderma first described on the Dalmatian island of Meleda. MDM has been observed in many other countries so that the origin of the MDM gene may be elsewhere than in Meleda. Objective: After identification of the first MDM patient during a medical visit, a study was planned to reveal other families with MDM in the Köprüçay region in Anatolia. Methods: The patient was interviewed with a questionnaire including a pedigree drawing. All the subsequent cases reported to be of MDM were visited for clinical examination and pedigree drawings. Results: Thirty-nine patients, 8 families and 2 additional cases with MDM were identified in a 50-km(2)mountainous region in Köprüçay canyon in Anatolia. The prevalent clinical features were nail involvement (80%), 'glove-and-sock' distribution of the keratoderma (60%), edema on the hands/feet (60%), conical tapering of the fingertips (60%) and hyperhidrosis on the palms and soles (50%). Conclusion: To our knowledge, this is the largest series reported. The relationship between the patients in Meleda and those in Anatolia awaits discovery by further researches that will be carried out with the collaboration of dermatology, genetics and medical history departments. Copyright © 2003 S. Karger AG, Basel. | URI: | https://hdl.handle.net/11499/5287 https://doi.org/10.1159/000068464 |
ISSN: | 1018-8665 |
Appears in Collections: | PubMed İndeksli Yayınlar Koleksiyonu / PubMed Indexed Publications Collection Scopus İndeksli Yayınlar Koleksiyonu / Scopus Indexed Publications Collection Tıp Fakültesi Koleksiyonu WoS İndeksli Yayınlar Koleksiyonu / WoS Indexed Publications Collection |
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