Darier Disease

Other names: Darier-White disease; keratosis follicularis

OMIM: 124200

Inheritance: autosomal dominant

Incidence: 1:55,000

Key findings:

Associated findings:

Age at first appearance: usually second decade, sometimes later

Long-term course: spontaneous relapses and remissions are common, but generally tends to worsen over time; heat and sun exposure are known causes of exacerbations; superficial bacterial infections are common and herpes simplex virus infections can become widespread

Diagnostic tests: skin biopsy shows characteristic changes; analysis of cellular DNA

Abnormal gene: calcium transporter gene ATP2A2


Learn more about FIRST's Regional Support Network -  connecting affected individuals and families with each other.

Clinicians seeking diagnosis should visit FIRST's TeleIchthyosis site to submit a case to experts in ichthyosis

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