Darier disease

Darier disease is an inherited keratinisation disorder that presents with wart-like spots across the body, with the genitals frequently involved.​1,2​

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 of Darier disease

  • 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.​3​

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.​4​

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.​5​

The inner workings of muscles are affected, particularly the relaxation and contraction of a muscle.​6​

References

  1. 1.
    Sehgal VN, Srivastava G. Darier’s (Darier–White) disease/keratosis follicularis. Int J Dermatology. Published online June 10, 2004:184-192. doi:10.1111/j.1365-4632.2004.02408.x
  2. 2.
    Alsharif SH, Alesa D, Baabdullah A. Type 1 Segmental Darier Disease: Case Report and Discussion of the Treatment Options. Case Rep Dermatol. Published online September 11, 2020:159-167. doi:10.1159/000509177
  3. 3.
    Hanna N, Lam M, Fleming P, Lynde CW. Therapeutic Options for the Treatment of Darier’s Disease: A Comprehensive Review of the Literature. J Cutan Med Surg. Published online November 28, 2021:280-290. doi:10.1177/12034754211058405
  4. 4.
    Curman P, Jebril W, Larsson H, Bachar-Wikstrom E, Cederlöf M, Wikstrom JD. Darier disease is associated with neurodegenerative disorders and epilepsy. Sci Rep. Published online March 26, 2024. doi:10.1038/s41598-024-57779-4
  5. 5.
    MUNRO CS. The phenotype of Darier’s disease: penetrance and expressivity in adults and children. Br J Dermatol. Published online August 1992:126-130. doi:10.1111/j.1365-2133.1992.tb08044.x
  6. 6.
    Gharaeinejad K, Eftekhari H, rafiei rana, Darjani A, Alizadeh N. Inflammatory myopathies in a patient with Darier’s disease, a possible association. Caspian J Intern Med. Published online January 2018. doi:10.22088/cjim.9.2.201


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