Darier's disease

Darier's disease is a rare, genetic skin disorder. It is an autosomal dominant disorder, that means, if one parent has Darier’s, 50% chance if the parent is heterozygous or, very rarely, a 100% chance that a child will inherit Darier’s if that parent is homozygous. Darier's disease was initially studied by dermatologists, but recent research shows that Darier’s has a whole-body effect, including cognitive (learning and intellectual) deficits, and mental health issues, particularly depression.

Mild forms of the disease are the most common, consisting of skin rashes that flare up under conditions such as high humidity, high stress, or tight-fitting clothes. Short stature, combined with poorly-formed fingernails that contain vertical striations, is diagnostic even for mild forms. Symptoms usually appear in late childhood or early adulthood between the ages of about 15 and 30 years and will vary over the lifespan in an intermittent pattern of relapse (flareups) and remit.

More severe cases are characterized by dark crusty patches on the skin that are mildly greasy and that can emit a strong odor. These patches, also known as keratotic papules, keratosis follicularis, or dyskeratosis follicularis, most often appear on the arms, chest, back and legs.