HOMOEOPATHIC REMEDIES FOR DARIER 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 year. The mucous membranes can also be affected , with blemishes on the roof of the mouth, tongue, inside of the cheek, gums and the throat. Other features  of this disease include nail abnormalities, such as red and white streaks in the nails with an irregular texture, and small pits in the palms of the hands and soles of the feet.

Darier disease is a rare genetic disorder that is manifested predominantly by scaly or crusted papules. It is also known as Darier –White disease . Darier disease was previously called ‘keratosis folliculitis ‘ , but this in correct because the scalypapules do not arise from hair follicles.

Onset of skin changes usually in adolescence and the disease is usually chronic . Darier disease is inherited in an autosomal dominant pattern , which means that a single gene passed from one parent causes the condition. The chance of a child inheriting the abnormal gene if one parent is affected is 1 in 2, but not all people with the abnormal gene will develop symptoms of the disease.

Causes

The abnormal gene in Darier disease has been identified as ATP2A2, found on chromosome 12q23-24.1. This gene codes for the SERCA enzyme or pump ( Sarco Endoplasmic Reticulum Calcium ATPase ) that is required to transport calcium within the cell. The exact mechanism by which this abnormal gene causes the disease is still under investigation but it appears that the way in which skin cells join together may be disrupted. The skin cells-keratinocytes, stick together via structures called desmosomes and it seems the desmosomes  do not assemble properly if there is insufficient calcium.

Symptoms

The symptoms and signs of Darier disease vary markedly between individuals. Some have very subtle signs that are asymptomatic and found only on careful inspection.  Others have extensive lesions which can cause considerable distress to the affected individual. In an affected person the severity of the disease can fluctuate over time.

The skin lesions are characterized by persistent , greasy, scaly papules. Affected site include:

·         Seborrhoeic areas of the face –scalp margins, forehead, ears, around the nostrils and sides of nose , eyebrows, and beard areas

·         Scalp and neck

·         Central chest and back

·         Skin folds , such as armpits, groin, under the breasts and between the buttocks

The papules have a firm , harsh feel like coarse sandpaper and may be skin colored , yellow brown or brown in color. If several of these papules grow together they may form larger warty lesions which can become quite smelly within skin folds. There may be heavily crusted rash similar to seborrhoeic dermatitis.

Atypical presentations of Darier disease are common

·         Flat, freckle-like lesions

·         Blistered papules

·         Large, raised , warty lesions

·         Linear pattern, with papules following the lines of embryonal development of the skin-dermatomal distribution.

Acne conglobata –cystic acne, is associated with Darier disease in some individuals

Acrokeratosis verruciformis—Warty papules on the dorsum of hands are known as acrokeratosis verruciformis or keratosis folliculitis. Other signs may include: Small pits on  the palms and soles. Bleeding under the skin.

A localized variant of Darier disease, in which papules on hands and feet are the main feature is called as acrokeratosis verruciformis of Hopf

Most people with Darier disease will have longitudinal broad stripes of white and reddish color on some or all fingernails. A V –shaped nick at the free edge of the nail is also very suggestive of Darier disease.

Patients with Darier disease may commonly have a white cobblestone pattern of small papules affecting mucous membranes. Overgrowth of the hums may also occur.

Most affected patients develop signs of Darier disease before the age of 30 years. Many have mild and unnoticed Darier disease . More severe disease may have a chronic relapsing –remitting pattern. The signs may clear up altogether. It may be flare up by—exposure to sunlight or heat, topical corticosterioids, bacterial infection, herpes simplex infection including severe eczema herpeticum etc.

In most cases , general health remains good regardless of the severity of the disease .

Diagnosis

The diagnosis of Darier disease  can be done by its appearance and from the family history. Also require a skin biopsy. The histology is characteristic , known as focal acantholytic dyskeratosis with varying degrees of papillomatosis, skin thickening. The pathology is similar to Grover disease.

HOMOEOPATHIC REMEDIES

Homoeopathy today is a rapidly growing system and is being practiced all over the world. Its strength lies in its evident effectiveness as it takes a holistic approach towards the sick individual through promotion of inner balance at mental , emotional , spiritual and physical levels. When Darier disease   is concerned there are many effective medicines are available in Homoeopathy, but the selection depends upon the individuality of the patient , considering the mental and physical symptoms.

A constitutional treatment is advisable in this case