Lestringant G G, Hadi S M, Qayed K I, Blayney B J
Department of Dermatology, Tawam Hospital, Abu Dhabi, United Arab Emirates.
Dermatology. 1992;184(1):78-82. doi: 10.1159/000247506.
Mal de Meleda (keratoderma palmoplantaris transgrediens) is a rare autosomal recessive form of palmoplantar keratoderma characterized by hyperkeratosis of the palms and soles which appears soon after birth and progressively (progrediens) involves other areas (transgrediens) of the body skin. We report the case of a patient with mal de Meleda with three unusual facultative features, i.e. prominent knuckle pads, peculiar finger-nail anomalies and pseudo-ainhum on both fifth fingers. Four other members of the family are also affected by mal de Meleda.
梅勒达病(进行性掌跖角化病)是一种罕见的常染色体隐性遗传性掌跖角化病,其特征为出生后不久手掌和足底出现角化过度,并逐渐(进行性)累及身体皮肤的其他部位(蔓延性)。我们报告了一例患有梅勒达病的患者,该患者有三个不寻常的兼性特征,即明显的指节垫、特殊的指甲异常以及双侧小指的假性断肢。该家族的其他四名成员也患有梅勒达病。