1. The patient has a long-standing pruritic rash. Flat, slightly elevated, greasy brown papules are scattered on the chest, abdomen, and upper back, with mild surrounding erythema. The fingernails are deformed, with longitudinal red streaks and ridges and v-shaped notching of the free margin.
Reprinted with permission from Cutis. 2003;72:124-126.
Diagnosis: Darier disease (DD) or Darier-White disease is a rare genetic skin disorder caused by mutations of the ATP2A2 gene, located on the long arm of chromosome 12 at position 24,11.1,2 This genetic mutation is inherited as an autosomal dominant trait with complete penetrance. DD affects men and women equally, with progressive skin signs of interfamilial and intrafamilial variability.3 Skin manifestations occur from late childhood to early adulthood and are typical during adolescence.3 Acute flare-ups can be triggered by heat, perspiration, sunlight, ultraviolet B exposure, stress, or certain medications (in particular, lithium).2 DD is not contagious.2
1. Creamer D, Barker J, Kerdel FA. Papular and papulosquamous dermatoses. In: Acute Adult Dermatology: Diagnosis and Management (A Colour Handbook). London, UK: Manson Publishing Ltd; 2011:48.
2. Kelly EB. Darier disease (DAR). In: Encyclopedia of Human Genetics and Disease. Santa Barbara, CA: ABC-CLIO; 2013:186-187.
3. Ringpfeil F. Dermatologic disorders. In: NORD Guide to Rare Disorders. Philadelphia, PA: Lippincott Williams & Wilkins; 2003:101.
For more information on this case, see “Man, 45, With Greasy Rash and Deformed Nails.” Clin Rev. 2014;24(1):38,40-41