Marrakchi Zeineb, Marrachi Slaheddine, Meziou Taha J, Turki Hamida, Zahaf Abdelmajid
Service de Dermatologie, Etablissement Publique de Santé Hédi Chaker, Sfax, Tunisie.
Tunis Med. 2006 Jul;84(7):423-6.
Mal de Meleda is a recessive transgression palmoplantar keratoderma. We report the epidemiological, clinical and progressiveness aspects of the disease in 16 patients of a large consanguineous tunisian family. The age ranged from 3 to 90 years. Nine were male and 7 female patients. Onset in early infancy was characterized by erythema of the palms and soles, followed by a diffuse yellowish hyperkeratosis. Thickness of the dorsal aspect of the fingers started in childhood. Extension of hyperkeratosis to the dorsal of the hand were reported only in adults and usually precede the involvement of the feet Palmoplantar hyperhidrosis with fetid odor between the toes was frequently seen as well as conical distal phalanges. In some adults keratoderma gave contracture of the fingers leading to limitation of movements Mal de Meleda must be differentiated from other recessive palmoplantar keratoderma such as Papillon-Lefévre syndrome and Mal de Naxos. the remaining inherited palmoplantar keratoderma being autosomal dominant. Mal de Meleda is a rare disease but is still relatively frequent in some tunisian regions, due to the high rate of consanguinity Mal de Meleda remains an afflicting and disabling disease.
梅勒达病是一种隐性遗传性掌跖角化病。我们报告了一个大型近亲通婚的突尼斯家族中16例患者的疾病流行病学、临床及进展情况。年龄范围为3至90岁。男性9例，女性7例。婴儿早期发病表现为手掌和足底红斑，随后出现弥漫性淡黄色角化过度。手指背侧增厚始于儿童期。仅在成人中报告有角化过度扩展至手背，且通常先于足部受累。常可见掌跖多汗伴脚趾间有恶臭，以及圆锥形远端指骨。在一些成年人中，角化病导致手指挛缩，致使活动受限。梅勒达病必须与其他隐性掌跖角化病如帕皮永-勒费弗尔综合征和纳克索斯病相鉴别，其余遗传性掌跖角化病为常染色体显性遗传。梅勒达病是一种罕见疾病，但在突尼斯一些地区仍相对常见，这归因于近亲结婚率高。梅勒达病仍然是一种折磨人且使人致残的疾病。
