HEREDITARY PALMOPLANTAR KERATODERMA ASSOCIATED WITH PRIMARY (CONGENITAL) LYMPHEDEMA

Abstract

The palmoplantar keratodermas are aheterogenous group of hereditary disordersof keratinization. They are characterizedby epidermal thickening and a yellow waxyappearance of the palms and soles. Geneticstudies have linked various forms ofpalmoplantar keratoderma to markers onchromosomes one, twelve, and seventeen, andseveral genes have been identified. Primarylymphedema is occasionally present at birth(congenital lymphedema or Milroy’s disease),but more commonly develops at puberty(lymphedema praecox). Genetic studies havelinked various autosomal dominant formsof primary lymphedema (Milroy’s diseaseand lymphedema distichiasis), to genes onchromosomes five and sixteen respectively.We report a case of palmoplantar keratodermain a child with congenital lymphedema.To our knowledge, this has not been previouslydescribed and may represent a newphenotype for future genetic study.