What is Darier’s disease?
Darier disease is an autosomal disorder and is non-contagious. Crusty patches with pus are characteristic of this disease, it affects any part of the body. The condition becomes worse during high humidity, stress and tight clothing. Vertical striation in fingernails & fragile nails is a diagnostic feature of the disease. Sometimes a patient may develop depression and epilepsy.
Commonly affected parts of the body are the scalp, forehead, behind the ears, arms, elbows, back, chest and knee. Mucous membranes also get involved. Rashes in darier’s disease have a distinct odor. Intraoral papules can be found, and palms and soles may become thick. Darier’s disease is not a contagious disease.
Occasionally patients with Darier’s disease may develop neurological disorders like depression, epilepsy or mild intellectual disability. A minor form of the disease may remain misdiagnosed lifelong.
Who is prone to get affected by Darier’s disease?
Darier’s disease affects both males and females.
Darier’s disease commonly starts from 6-20 years of age. However, a patient aged 4 and up to 70 years are also seen as affected by the disease.
What is the prognosis of Darier’s disease?
In darier’s disease, there is increased susceptibility to cutaneous bacterial and viral infections. Misdiagnosis of darier’s disease leads to under-treatment of these infections and may prove fatal.
However, the patient with darier’s disease has a life expectancy as that of the general population.
What are the Causes of Darier’s Disease?
The cause of the development of Dariers disease is a mutation in the gene that codes for the enzyme SERCA2 (sarcoendoplasmic reticulum CA2+-ATPase). The gene ATP2A2 is localized in chromosome 12 and controls the synthesis of the SARCA2 enzyme. This enzyme pumps calcium across the cell membrane. If one of the two genes ATP2A2 is affected, the normal functionality in skin cells is not maintained, and there is impaired passage of calcium in skin cells resulting in the development of horny cells prematurely before they reach the surface of the skin.
What are the signs and symptoms of Darier’s disease?
Symptoms start with the appearance of rough papules in the outermost layer of skin, mainly around the hair follicle.
The papules then develop into a brownish rash then grow together and form a thick plaque with a moist surface. Plaques are susceptible to infections from bacteria and herpes virus.
The severity of the disease varies over time. Hot and humid climates aggravate the rashes. There is increased growth of bacteria on skin lesions that emit an unpleasant odour. Rashes are moist and bleed less often. Acute swelling of the parotid gland occurs as a result of obstruction of salivary glands.
What are the Investigations advised in Darier Disease?
Skin biopsy shows degenerated cells in the epidermis and increased keratinization.
What treatment is advised for Darier’s disease?
• Treatment aims at relieving the symptoms.
• Softening creams are used to counteract the thickening of the horny layer.
• Oral retinoids are used to normalize the keratinization process. However, the restricted use of retinoid is recommended as it has side effects. It is strictly avoided in pregnant women as it can cause birth defects.
• Surgery may be required in case of obstruction of salivary glands.
• In the case of skin infection, antibacterial treatment is required. In herpes virus infection antiviral agents are used.
How to prevent Darier Disease?
• Avoid exposure to direct sunlight.
• Loose-fitting clothes are advised to wear.
• Frequent showering proves beneficial but excessive scrubbing may cause weeping sores.
What is the Differential diagnosis of Darier Disease?
• Seborrheic dermatitis.
• Transient Acantholytic Dermatosis.
• Familial benign pemphigus.
• Acrokeratosis verruciformis of Hopf.
What are the commonly indicated Homeopathic medicines for Darier disease?
Sulphur
Mezer
Hepar sulph
