Which clinical history findings are characteristic of keratosis follicularis (Darier disease)?

Updated: Jul 31, 2018
  • Author: Pui-Yan Kwok, MD, PhD; Chief Editor: Dirk M Elston, MD  more...
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Answer

Most patients with keratosis follicularis (Darier disease) have a family history of the disease. The pattern of inheritance is autosomal dominant. However, some patients, up to 47% in one series, have no clear family history. These cases may represent sporadic mutations, or these patients may have family members with mild disease that was not recognized.

The first skin lesions typically occur in the teenage years and are frequently associated with pruritus.

Heat, sweat, humidity, sunlight, UVB exposure, [38] lithium, oral corticosteroids, and mechanical trauma have been reported to exacerbate keratosis follicularis (Darier disease). Some females report flares around menstruation.

Even though the severity of keratosis follicularis (Darier disease) fluctuates over time, keratosis follicularis (Darier disease) is a chronic, unremitting condition. In one study, one third of patients noted improvement of the condition with age; however, another one third of patients showed worsening of the disease with age.


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