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September 1997

Localized Darier DiseaseImplications for Genetic Studies

Author Affiliations

From the Division of Dermatology, University of Toronto and Women's College Hospital, Toronto, Ontario (Drs O'Malley and Berg), and the Department of Dermatology, University of Rochester, Rochester, NY (Drs Haake and Goldsmith).

Arch Dermatol. 1997;133(9):1134-1138. doi:10.1001/archderm.1997.03890450084010

Background:  Darier disease is an uncommon genodermatosis characterized by the symmetrical eruption of keratotic reddish-brown papules occurring in the seborrheic areas of the body. A unilateral, or localized, variant has been identified. We report 4 new cases of localized Darier disease and review the English-language literature. The implications of these cases on future genetic studies are also discussed.

Observations:  Localized Darier disease occurred with equal frequency in males and females. The average age at onset was 27 years. The most frequent site of involvement was the trunk (40% [16/40]). This condition was aggravated by sunlight, heat, or sweating in 42% (19/40) of reported cases, and 38% (15/40) of the patients responded to treatment with topical tretinoin.

Conclusions:  Many of the clinical features of localized Darier disease suggest that it is a genetic mosaic of generalized Darier disease. Further studies of localized Darier disease may therefore prove to be instrumental in the search for the Darier disease gene.Arch Dermatol. 1997;133:1134-1138