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June 1995

Olmsted Syndrome in Twins

Author Affiliations

Center for Inherited Cutaneous Diseases Institute of Dermatological Sciences IRCCS—Ospedale Maggiore di Milano University of Milan Milan 20122, Italy

Arch Dermatol. 1995;131(6):738-739. doi:10.1001/archderm.1995.01690180118028

Olmsted syndrome is a rare disorder of keratinization appearing at birth or in early infancy that is characterized by the unusual association of palmoplantar keratoderma and periorificial hyperkeratosis, with possible involvement of other ectodermal derivatives.1-6 We report the second familial case, showing a progressive course unresponsive to conventional treatment.

Report of a Case.  The patients are monozygotic male twins, born at term to nonconsanguineous parents, aged 29 (father) and 25 (mother) years. At 4 months of age, both children developed bilateral palmoplantar keratoderma and keratotic lesions at the external acoustic meatus. Familial history was negative for any related skin disorders.Examination at age 15 months showed symmetrical, thick, sharply marginated, and yellowish areas of hyperkeratosis affecting the plantar surface of the forefeet and of the first and fifth toes. The lesions were surrounded by an erythematous border and extended onto the lateral aspects of the toes. A periodic complete

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