Darier's disease

Darier's disease
Other namesDarier disease, Darier–White disease,[1] Dyskeratosis follicularis,[1] and Keratosis follicularis[2]: 523 [3]: 567 
SpecialtyMedical genetics Edit this on Wikidata

Darier's disease (DD) is a rare, genetic skin disorder. It is an autosomal dominant disorder, that is, if one parent has DD, there is a 50% chance than a child will inherit DD. It was first reported by French dermatologist Ferdinand-Jean Darier in 1889.

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.[4]

DD was initially studied by dermatologists. Recent research however shows DD has a whole-body effect, including cognitive (learning and intellectual) deficits, and mental health issues, particularly depression.

  1. ^ a b Rapini, Ronald P.; Bolognia, Jean L.; Jorizzo, Joseph L. (2007). Dermatology: 2-Volume Set. St. Louis, MO: Mosby. ISBN 978-1-4160-2999-1.
  2. ^ Freedberg, et al. (2003). Fitzpatrick's Dermatology in General Medicine (6th ed.). McGraw-Hill. ISBN 0-07-138076-0.
  3. ^ James, William; Berger, Timothy; Elston, Dirk (2005). Andrews' Diseases of the Skin: Clinical Dermatology (10th ed.). Saunders. ISBN 0-7216-2921-0.
  4. ^ Sehgal, V. N.; Srivastava, G. (2005). "Darier's (Darier-White) disease/keratosis follicularis". International Journal of Dermatology. 44 (3): 184–192. doi:10.1111/j.1365-4632.2004.02408.x. PMID 15807723. S2CID 45303870.