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Mutilating palmoplantar keratoderma with periorificial keratotic plaques (Olmsted's syndrome).

Abstract
We report the case of a boy, now aged 4 years, with a progressive, painful and disabling palmoplantar keratoderma, associated with well-defined hyperkeratotic plaques around the mouth and nostrils. His mother has an identical palmoplantar keratoderma. The palmoplantar keratoderma failed to improve with etretinate, but considerable benefit resulted from full-thickness excision of the skin of both palms, followed by skin grafting. We believe that he has a distinctive but extremely rare form of hereditary palmoplantar keratoderma, first described by Olmsted.
AuthorsD J Atherton, C Sutton, B M Jones
JournalThe British journal of dermatology (Br J Dermatol) Vol. 122 Issue 2 Pg. 245-52 (Feb 1990) ISSN: 0007-0963 [Print] England
PMID2138494 (Publication Type: Case Reports, Journal Article)
Topics
  • Child, Preschool
  • Facial Dermatoses (pathology)
  • Humans
  • Keratoderma, Palmoplantar (genetics, pathology, surgery)
  • Male
  • Skin Transplantation
  • Syndrome

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