Localized Darier disease. Implications for genetic studies.

M P O'Malley; A Haake; L Goldsmith; D Berg

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PubMed · 9301591

Localized Darier disease. Implications for genetic studies.

M P O'Malley·A Haake·L Goldsmith·D Berg

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Abstract

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.

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M P O'Malley, A Haake, L Goldsmith, D Berg. 1997. Localized Darier disease. Implications for genetic studies.. https://pubmed.ncbi.nlm.nih.gov/9301591/

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