An extremely rare syndrome characterised by a symmetrical telangiectasia of the extremities with hyperkeratoris and hyperhidrosis. Both sexes affected, female less severe manifestations; onset in infancy and gradual progression. Asymptomatic or painful fissure on palms and soles. Signs are keratosis of palms and soles extending to all hands and feet and eventually to patches of arms and legs. Aetiology unknown. Autosomal dominant inheritance.
Meleda is an island off the coast of Dalmatia, Croatia.
We thank Andre Trombeta for information submitted.
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Keratosis extremitatum hereditaria progrediens mit dominantem Erbgang.
Der Hautarzt, 1952, 3: 198-203.
- V. P. Sybert, B. A. Dale, K. A. Holbrook:
Palmar-plantar keratoderma: A clinical, ultrastructural, and biochemical study.
Journal of the American Academy of Dermatology, St. Louis, 1988, 18: 75.
- T. Gedde-Dahl, Jr.; S. Rogde, P. Helsing, T. Gabrielsen, B. Olaisen:
Greither's disease and erythrokeratodermia variabilis (EKV) caused by the same mutation on chromosome 1. Abstract.
Human Genome Mapping Workshop 93 1 only, 1993.
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Palmoplantar keratoderma of Sybert.
Dermatology Online Journal, October 2003, 9 (4): 30.