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.
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.
Authors: Katherine C MacKenzie; Rhiana Garritsen; Rajendra K Chauhan; Yunia Sribudiani; Bianca M de Graaf; Tim Rugenbrink; Rutger Brouwer; Wilfred F J van Ijcken; Ivo de Blaauw; Alice S Brooks; Cornelius E J Sloots; Conny J H M Meeuwsen; René M Wijnen; Donald F Newgreen; Alan J Burns; Robert M W Hofstra; Maria M Alves; Erwin Brosens Journal: Int J Mol Sci Date: 2021-11-16 Impact factor: 5.923
Authors: Dennis Linder; Elena Marinello; Pietro Maria Donisi; Roberto Salmaso; Edoardo Zattra; Anna Zampetti Journal: Case Rep Dermatol Date: 2016-07-12