Olmsted syndrome: Case report and identification of a keratin abnormality

Douglas W. Kress, Mark P. Seraly, Louis Falo, Bong Kim, Brian V. Jegasothy, Bernard Cohen

Research output: Contribution to journalArticle

Abstract

Background: Olmsted syndrome is a rare disorder characterized by a mutilating palmoplantar keratoderma and periorificial keratotic plaques. It begins in early childhood and is complicated by the development of painful flexion contractures, constrictions, and autoamputations of the digits. Only 11 cases of Olmsted syndrome have been reported to date. However, no biochemical abnormalities in the skin were reported in any of these cases. Observations: We report the 12th case of Olmsted syndrome. In addition, we describe a keratin abnormality found in a skin specimen obtained from our patient. The specimen showed a suprabasilar staining pattern with AE1, an antibody that shows only basilar staining in normal skin. Conclusion: We report the 12th case of Olmsted syndrome, review the literature, and describe a keratin abnormality that was found in our patient's skin specimen.

Original languageEnglish (US)
Pages (from-to)797-800
Number of pages4
JournalArchives of dermatology
Volume132
Issue number7
DOIs
StatePublished - Jul 30 1996

ASJC Scopus subject areas

  • Dermatology

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