What is Darier disease?
Keratosis follicularis, also known as Darier disease, is a rare, genetic skin disorder. Affected individuals develop skin lesions that consist of thickened, rough bumps (papules) or plaques that may also be greasy or have a brown or yellow crust.
How common is epidermodysplasia verruciformis?
About 7.5% of cases appear in infancy, 61.5% in children aged 5–11 years and 22.5% in puberty. The disease affects both males and females and people of all races. Acquired epidermodysplasia verruciformis is a result of HIV infection, organ transplantation or a lymphoma.
Is epidermodysplasia verruciformis contagious?
Over time, the infection causes skin growths, such as viral warts and pigmented, inflamed patches. In severe or extreme cases, a person may develop bark-like growths. HPV is contagious and usually transmitted through skin-to-skin contact. A person can pass it on even if they experience no symptoms.
How is Darier disease diagnosed?
Darier disease is usually diagnosed by the appearance of the skin and the family history . It may be mistaken for other skin conditions. The diagnosis may require a skin biopsy . Genetic testing to identify a mutation in the ATP2A2 gene can be used to confirm the diagnosis.
Can epidermodysplasia verruciformis be cured?
Although permanent cure of EV cannot be achieved by any therapy at present, described treatment modalities include cryotherapy, topical imiquimod and 5-fluorouracil, systemic retinoids, interferon alpha, and 5-aminolevulinic acid photodynamic therapy. Surgical excision is the treatment of choice for SCC.
Can Epidermodysplasia Verruciformis be prevented?
Epidermodysplasia verruciformis is a lifelong disease. Even though lesions can be treated or removed as they appear, the lesions develop throughout life. Currently there is no treatment to prevent new lesions from occurring.
Is there a cure for Darier’s disease?
Systemic medications Oral retinoids (eg, acitretin, isotretinoin, etretinate, alitretinoin [57, 58] ) have been the most effective medical treatment for keratosis follicularis (Darier disease), achieving some reduction of symptoms in 90% of patients. They reduce hyperkeratosis, smoothen papules, and reduce odor.
What are the symptoms of Darier’s disease?
Darier disease is a skin condition characterized by wart-like blemishes on the body. The blemishes are usually yellowish in color, hard to the touch, mildly greasy, and can emit a strong odor. The most common sites for blemishes are the scalp, forehead, upper arms, chest, back, knees, elbows, and behind the ear.
What is the treatment for Darier’s disease?
Oral retinoids have been the most effective medical treatment for Darier disease, leading to reduction of symptoms in 90% of affected people.