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July 1996

Olmsted SyndromeCase Report and Identification of a Keratin Abnormality

Author Affiliations

From the Departments of Dermatology (Drs Kress, Seraly, Falo, and Jegasothy) and Pathology (Dr Kim), University of Pittsburgh Medical Center, Pittsburgh, Pa, and the Department of Dermatology, The Johns Hopkins University, Baltimore, Md (Dr Cohen).

Arch Dermatol. 1996;132(7):797-800. doi:10.1001/archderm.1996.03890310083012

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.Arch Dermatol. 1996;132:797-800