Hereditary palmoplantar keratoderma associated with primary (congenital) lymphedema.

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Hereditary palmoplantar keratoderma associated with primary
(congenital) lymphedema.

Lymphology. 2009 Jun

Ogunbiyi SO, Deguara J, Moss C, Burnand KG.
Department of Academic Surgery, St Thomas' Hospital, London, UK.
samuelo...@gmail.com

The palmoplantar keratodermas are a heterogenous group of hereditary
disorders of keratinization. They are characterized by epidermal
thickening and a yellow waxy appearance of the palms and soles.
Genetic studies have linked various forms of palmoplantar keratoderma
to markers on chromosomes one, twelve, and seventeen, and several
genes have been identified. Primary lymphedema is occasionally present
at birth (congenital lymphedema or Milroy's disease), but more
commonly develops at puberty (lymphedema praecox). Genetic studies
have linked various autosomal dominant forms of primary lymphedema
(Milroy's disease and lymphedema distichiasis), to genes on
chromosomes five and sixteen respectively. We report a case of
palmoplantar keratoderma in a child with congenital lymphedema. To our
knowledge, this has not been previously described and may represent a
new phenotype for future genetic study.

PubMed

http://www.ncbi.nlm.nih.gov/pubmed/19725273?ordinalpos=10&itool=EntrezSystem2.PEntrez.Pubmed.Pubmed_ResultsPanel.Pubmed_DefaultReportPanel.Pubmed_RVDocSum