Olmsted Syndrome—Palmoplantar and Periorificial Keratodermas: Association with Malignant Melanoma

  • Josee Dessureault
  • Yves Poulin
  • Marc Bourcier
  • Eric Gagne
Case Report

Abstract

Background: Olmsted syndrome is a rare congenital disorder with mutilating palmoplantar keratoderma, periorificial keratotic plaques, and other variable features. Objective: We describe a 65-year-old woman with Olmsted syndrome complicated by the occurrence of a malignant melanoma inside the plantar keratoderma. To our knowledge, this is the first reported case of such an occurrence in Olmsted syndrome. The published cases of this rare disorder are reviewed. Conclusion: An association between malignant epithelial tumors and Olmsted syndrome has already been reported. The association of malignant melanoma with other types of palmoplantar keratodermas has been reported. This may suggest a predisposition to melanocytic as well as squamous cell malignancies in congenital keratodermas. Oral retinoids appear to be the most promising treatment for Olmsted syndrome and for other symptomatic keratodermas.

Antécédents: Le syndrome d’Olmsted est un trouble congénital rare présentant une kératodermie palmoplantaire mutilante, des papules kératosiques autour des orifices naturels et autres diverses caractéristiques. Objectifs: Nous décrivons le cas d’une femme de 65 ans atteinte du syndrome d’Olmsted qui s’est compliqué en raison d’un mélanome dans la kératodermie plantaire. À notre connaissance, il s’agit du premier cas rapporté d’une telle occurrence en présence du syndrome d’Olmsted. Les cas publiés de ce trouble rare sont passés en revue. Conclusion: Une association entre des tumeurs malignes épithéliales et le syndrome d’Olmsted a déjà été rapportée, de même qu’une association entre des tumeurs malignes épithéliales et d’autres formes de kératodermie palmoplantaire. Cela peut suggérer une prédisposition à développer des cellules mélanocytaires ou squameuses en présence de ké’ratodermies congénitales. Il semble que les rétinoïdes oraux sont le traitement le plus prometteur du syndrome d’Olmsted et d’autres kératodermies symptomatiques.

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Copyright information

© Canadian Dermatology Association 2003

Authors and Affiliations

  • Josee Dessureault
    • 1
  • Yves Poulin
    • 1
  • Marc Bourcier
    • 2
  • Eric Gagne
    • 3
  1. 1.Department of DermatologyCentre Hospitalier Universitaire de Québec, pavilion Hotel-Dieu de Québec, Québec, QuébecCanada
  2. 2.Department of DermatologyHopital Docteur Georges-L-Dumont, Moncton, New BrunswickCanada
  3. 3.Department of PathologyCentre Hospitalier Universitaire de Québec, pavilion Hotel-Dieu de Québec, Québec, QuébecCanada

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