About dyskeratosis follicularis vegetans

What is dyskeratosis follicularis vegetans?

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. These hardened, scaly lesions are progressive and may gradually grow bigger or spread. The nails and mucous membranes are also affected in most cases. Individuals may have periods of time when signs improve (remission), but the lesions usually recur (relapse). The specific problems vary from one individual to another. Keratosis follicularis is inherited as an autosomal dominant trait.

What are the symptoms for dyskeratosis follicularis vegetans?

Skin lesions consisting thickened symptom was found in the dyskeratosis follicularis vegetans condition

The characteristic Blisters of this condition are:

Small, similar to grains of tapioca
Filled with clear fluid
In clusters between your fingers and toes or on the edges of your fingers, toes, palms, or soles
Very Itchy and sometimes painful
The Blisters usually dry and peel in about three weeks. The skin beneath them may be scaly, red, cracked, and painful.

What are the causes for dyskeratosis follicularis vegetans?

The cause of dyshidotric eczema is unknown, but more than one factor is likely involved.

It may occur along with atopic dermatitis or hay fever, flaring seasonally along with any allergies you may have.

About 40 percent of people with this skin condition are also troubled with hands or feet that perspire heavily.

Dyshidrotic eczema is more likely to occur if you:

Are under stress
Have atopic dermatitis
Have sensitive skin in general
Have allergies, including hay fever
Wash your hands frequently or your hands are often in water
Work with cement
Expose your hands to metals, such as chromium, cobalt, or nickel
Receive intravenous immunoglobulin infusions to treat another medical condition

What are the treatments for dyskeratosis follicularis vegetans?

The most effective treatment for mild or moderate dyshidotric eczema is to moisturize your hands or feet with a lotion, cream, or ointment, such a petroleum jelly.

The moisturizer you use should be free of fragrance, dyes, or alcohol.

If allergies are the cause, an over-the-counter (OTC) oral antihistamine should offer some relief.

For itching, try an OTC topical corticosteroid cream (also called a topical steroid or topical cortisone cream), cold compresses, or applications of witch hazel. When self-care doesn't resolve dyshidrotic eczema, your doctor may prescribe a topical cream, such as tacrolimus or pimecrolimus, which suppresses your immune system response.

If your skin condition is severe, your healthcare provider also may prescribe steroid pills, coal tar preparations, phototherapy, or a combination of these.

What are the risk factors for dyskeratosis follicularis vegetans?

There are a variety of factors that may dictate who develops dyshidrotic eczema.

If you are going to develop it, it’ll most likely begin between 20 and 40 years of age. Genetics may also play a role in dyshidrotic eczema. If you have one or more blood relatives with it, there’s a higher chance you could also have it.

A few other factors that may contribute to its development are:

  • you’re already living with another type of eczema
  • you’ve worked, or currently work, as a mechanic or metalworker (because of the contact to certain metals like nickel)
  • you have a history of working with cement (which can contain both cobalt and nickel)
  • you already deal with seasonal allergies
  • you’re living with asthma
  • you have occasional bouts of allergic sinusitis

Is there a cure/medications for dyskeratosis follicularis vegetans?

The treatment of dyskeratosis follicularis vegetans or Darier's disease (DD) is directed toward the specific symptoms that are apparent in each individual. Aside from symptom management and behavioral modifications to avoid triggers, currently, no validated treatments for dyskeratosis follicularis vegetans are available.

Symptomatic treatments include:

  • Sunscreen, loose clothing, moisturizing creams and avoiding excessive heat may reduce the severity of the disease for some individuals.
  • Steroids, vitamin D analogs, retinoids, photodynamic therapy, and surgical excision.
  • Oral acitretin, oral isotretinoin, systemic vitamin A, topical tretinoin, topical isotretinoin, topical adapalene gel, topical 5-fluorouracil, topical calciptriol, and topical tacalcitol (with sunscreen), Grenz ray radiation, and x-ray radiation are all considered to cause a developmental delay (DD).
  • Clinicians may think about starting a trial of certain topical or oral retinoids in patients with localized or generalized DD first based on the quality and amount of available evidence.
  • Antibiotics may be used for individuals with a secondary bacterial infection.
  • Antiviral agents such as acyclovir may be prescribed for conditions associated with infection with the herpes simplex virus.
  • Genetic counseling is important for affected individuals and their families.

Skin lesions consisting thickened, rough bumps (papules) or plaques that may be greasy or have a brown or yellow crust,Development of multiple, small, yellow-brown, flattened wart-like (verrucous) bumps (papules) on the backs of the hands or feet
Wart-like blemishes on the body,Hard to the touch or painful conditions
Synthetic derivatives of vitamin A (retinoids),Oral retinoids such as tretinoin and acitretin affect the entire body (systemic therapy),Antibiotics,Antiviral agents such as acyclovir

Video related to dyskeratosis follicularis vegetans