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Darier's disease

Diseases of the Skin and Subcutaneous Tissue

Darier's disease is a rare genetic skin disorder caused by mutations in the ATP2A2 gene, which encodes a calcium pump that normally helps skin cells stick together. When the pump fails, skin cell adhesion breaks down and the epidermis develops a pattern resembling nutritional vitamin A deficiency. The condition follows autosomal dominant inheritance, so a child of an affected parent has a 50 percent chance of inheriting it, and symptoms usually first appear between ages 15 and 30. Typical signs include fragile or ridged fingernails with vertical striations, greasy or scaly skin rashes that worsen with heat and humidity, itching, and dark, crusty, mildly greasy patches on the chest, back, arms and legs. Research has also linked the disease to higher rates of depression and suicidal ideation, and symptoms tend to fluctuate in severity over a person's life.

Facts
Classification
Body System
Integumentary System 1
ICD-10 Chapter
XII: Diseases of the Skin and Subcutaneous Tissue 1
Communicable or Non-communicable
Non-Communicable 1
Causative Agent
Causative Agent (category)
Genetic / Hereditary 1
Sources
1. Darier's disease (Wikipedia)
  • lead sentence 1
    Darier's disease is a rare, genetic skin disorder.
  • Lead: disease category
    Darier's disease is a rare, genetic skin disorder.
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