| Dental Dictionary: Darier’s disease |
An apparently genetic dermatologic disease that also involves mucous membranes. The oral lesions are whitish papules of the gingiva, tongue, or palate. Darier’s disease is characterized histologically by the presence of corps ronds.
| 5min Related Video: Darier's disease |
| Medical Dictionary: keratosis fol·lic·u·lar·is |
| WordNet: keratosis follicularis |
The noun has one meaning:
Meaning #1:
a rare hereditary condition marked by dark crusted patches (sometimes containing pus)
Synonym: Darier's disease
| Wikipedia: Darier's disease |
| Darier's disease | |
|---|---|
| Classification and external resources | |
| ICD-10 | Q82.8 (ILDS Q82.868) |
| ICD-9 | 757.39 |
| OMIM | 124200 |
| DiseasesDB | 3467 |
| eMedicine | derm/209 |
| MeSH | [1] |
Darier's disease (also known as "Darier-White disease," and "Keratosis follicularis"[1]:523[2]:567) is a genetic disorder discovered by French dermatologist Ferdinand-Jean Darier. Darier's is characterized by dark crusty patches on the skin, sometimes containing pus. The crusty patches are also known as keratotic papules, keratosis follicularis or dyskeratosis follicularis.[3]
Contents |
Darier's is an autosomally dominant inherited mutation in the gene ATP2A2.[4] Family members with confirmed identical ATP2A2 mutations can exhibit differences in the clinical severity of disease, suggesting that other genes or environmental factors affect the expression of Darier's disease. With the discovery of the ATP2A2 gene, performing genetic tests to confirm the diagnosis of Darier's disease is now possible.
It affects both group c2 men and women and is not contagious. The disease often starts during or later than the teenage years, typically by the third decade. The symptoms of the disease are thought to be caused by an abnormality in the desmosome-keratin filament complex leading to a breakdown in cell adhesion.
It most commonly affects the chest, neck, back, ears, forehead, and groin, but may involve other body areas. The rash associated with Darier's disease often has a distinct odor. The rash can be aggravated by heat, humidity, and exposure to sunlight.
Treatment of choice for severe cases is oral retinoids. During flares, topical or oral antibiotics may be administered. Ciclosporin and topical corticosteroids have been used during acute flares. Some patients are able to prevent flares with use of topical sunscreens and oral vitamin C.[5]
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 | Keratosis follicularis |
| Longitudinal erythronychia |
| ATP2A2 |
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Copyrights:
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 | Dental Dictionary. Mosby's Dental Dictionary. Copyright © 2004 by Elsevier, Inc. All rights reserved. Read more |
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| Medical Dictionary. The American Heritage® Stedman's Medical Dictionary Copyright © 2002, 2001, 1995 by Houghton Mifflin Company. Read more |
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| WordNet. WordNet 1.7.1 Copyright © 2001 by Princeton University. All rights reserved. Read more |
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| Wikipedia. This article is licensed under the Creative Commons Attribution/Share-Alike License. It uses material from the Wikipedia article "Darier's disease". Read more |
