Definition:
Darier's disease is defined as a genetic disease or inherited dermatitis characterized by ‘dark crusty patches’ on skin, which sometimes contain pus. These patches are known as keratosis follicularis or keratotic papules. Although complications are uncommon, the disease can cause bacterial sepsis, as well as Kaposi’s varicelliform eruption.
Diagnosis:
Darier's disease is diagnosed through medical history, physical examination, procedures, and other tests. Gene sequencing is also used, as well as skin biopsy for confirming the diagnosis.
Treatment:
The preferred treatment for severe Darier's disease is ‘oral retinoids’. In cases of flares, oral or topical antibiotics can be administered. Topical corticosteroids and cyclosporin have been employed for acute flares. A number of affected individuals are able to avert flares using oral vitamin C and topical sunscreens. Besides medical care, surgical treatment may also be done, such as electrosurgery, dermabrasion, and others.
Symptoms and Signs:
The most common symptoms include hyperkeratotic samples in the seborrheic regions, changes in mucous membrane, and nail abnormalities. Other symptoms include foul odor, pruritus, , keratotic rash, and hyperkeratotic lesions.
Causes:
The disease is caused by a mutation in a gene called ATP2A2. The mutation results to disrupted signaling within the cells and causes the skin condition.
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