LOCALIZED DARIER-DISEASE - IMPLICATIONS FOR GENETIC-DISEASE

Background: Darier disease is an uncommon genodermatosis characterized by the symmetrical eruption of keratotic reddish-brown papules occurr ing in the seborrheic areas of the body. A unilateral, or localized, v ariant has been identified. We report 4 new cases of localized Darier disease and review the English-language literature. The implications o f these cases on future genetic studies are also discussed. Observatio ns: Localized Darier disease occurred with equal frequency in males an d females. The average age at onset was 27 years. The most frequent si te of involvement was the trunk (40% [16/40]). This condition was aggr avated 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 Dar ier disease suggest that it is a genetic mosaic of generalized Darier disease. Further studies of localized Darier disease may therefore pro ve to be instrumental in the search for the Darier disease gene.