Ayman T, Yerebakan O, Yilmaz E
Akdeniz University School of Medicine, Department of Dermatology, Antalya, Turkey.
J Dermatol. 2000 Oct;27(10):664-8. doi: 10.1111/j.1346-8138.2000.tb02250.x.
Keratoderma Palmoplantare Transgrediens or mal de Meleda (MDM) is a very rare, inherited disorder. Its name derives from the island of Meleda. Although, MDM has been reported from many different countries, only two Turkish patients with MDM have been reported in the international literature. Our six cases of MDM are reported, and retrospective analysis of published Turkish patients with MDM is included. We review the clinical characteristics, associated findings, consanguinity, and family history of the cases. In total, 26 Turkish cases of MDM including ours have been documented. Ten cases showed various nail abnormalities, the most common associated feature. We add 24 new cases from Turkey to the list in the literature. Our findings suggest that the MDM gene may also have originated elsewhere than in Mljet.
进行性掌跖角化病或梅莱达病(MDM)是一种非常罕见的遗传性疾病。其名称源于梅莱达岛。尽管MDM在许多不同国家都有报道,但国际文献中仅报道了两例土耳其MDM患者。我们报告了6例MDM病例,并纳入了对已发表的土耳其MDM患者的回顾性分析。我们回顾了这些病例的临床特征、相关表现、近亲结婚情况和家族史。包括我们的病例在内,土耳其共有26例MDM病例被记录在案。10例出现了各种指甲异常,这是最常见的相关特征。我们在文献中新增了24例来自土耳其的病例。我们的研究结果表明,MDM基因可能并非仅起源于姆列特岛。