What does Darier disease look like?

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 Acrokeratosis Verruciformis?

Acrokeratosis verruciformis is a rare genodermatosis with an autosomal dominant mode of inheritance. Acrokeratosis verruciformis is a disorder of keratinization characterized by multiple flat-topped, skin-colored keratotic lesions resembling plane warts typically observed on the dorsum of the hands and feet.

When does Darier’s disease start?

Darier’s disease is an incommunicable disorder that is seen in males and females equally. Symptoms typically arise between the ages of 15 and 30.

What triggers Darier’s disease?

Darier disease is caused by mutations in the ATP2A2 gene . This gene gives the body instructions to make an enzyme known as SERCA2. This enzyme helps control the level of calcium ions inside cells . Calcium ions act as signals for many cell functions needed for normal development.

Is Darier disease curable?

The disease usually persists for life and has no cure. Treatment is never complete, and the disease is characterized by a relapsing-remitting course [6]. Topical agents like retinoids, tazarotene, and 5-fluorouracil have been shown to benefit DD that is limited and not severe.

How is Darier disease treated?

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 is focal hyperkeratosis?

Focal acral hyperkeratosis (FAH) is a rare and benign cutaneous disorder characterized by clusters of discrete, small, firm, hyperkeratotic papules along the borders of the hands and feet. The exact etiology and pathogenesis are not known.

Who does Epidermodysplasia Verruciformis affect?

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.

What is the treatment for Darier’s disease?

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.

How do you get rid of Darier’s disease?

Is Darier disease contagious?

In Darier’s disease the sticky junctions that hold the skin cells together are not made properly, and the skin may be easily irritated and becomes inflamed and weeping. It is not due to an allergy and it is not contagious (catching).

What is hyperkeratosis look like?

Follicular hyperkeratosis, also known as keratosis pilaris (KP), is a skin condition characterized by excessive development of keratin in hair follicles, resulting in rough, cone-shaped, elevated papules. The openings are often closed with a white plug of encrusted sebum.