Darier disease is an inherited keratinisation disorder that presents with wart-like spots across the body, with the genitals frequently involved. There is a sub-version of this disease known as linear or segmental-form Darier disease, where the spots are localised to one side of the body, and not widespread like in usual Darier disease.

Symptoms

  • Wart-like spots, often yellow and hard
  • Spots can be greasy
  • Spots can smell very strong
  • Appears on scalp, forehead, upper arms, chest, back, knees, elbows, behind the ear
  • May impact mucous membranes (mouth, vagina)
  • Red and white streaks on textured nails
  • Small pits in the palms and soles of the feet

This condition typically appears in the teens, with flare-ups and calm periods coming and going. Summer usually sees more blemishes, with heat, humidity, sweat and friction. Rubbing, scratching, UV light and some medicines also make this condition worse. Mild intellectual disabilities are linked with Darier disease, which may be genetic, however there may be social problems associated with this disease, since it is physically obvious.

Keratinisation of the skin is disrupted due to a mutation of a gene on band 12q23-24.1, with incidence relatively widespread. It is found in Britain and Europe in about 1 in 100,000 and 1 in 30,000 people respectively.

How does it work?

The inner workings of muscles are affected, particularly the relaxation and contraction of a muscle.

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