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 throat. Other characteristics of this condition include: the blemishes are typically yellowish in color, hard to the touch, mildly greasy, and can emit a strong odor.
Darier disease, also known as Darier -White disease, is a rare genetic disorder characterized primarily by scaly or crusted papules. Previously, the name “keratosis folliculitis” was used to describe the condition, but this is incorrect because the scaly papules do not develop from hair follicles.
Darier disease is inherited in an autosomal dominant pattern, which means that a single gene passed from one parent causes the condition. Darier disease usually manifests as chronic skin changes, and the likelihood of a child inheriting the abnormal gene is one in two if one parent has the condition.
Causes
The SERCA enzyme or pump (Sarco Endoplasmic Reticulum Calcium ATPase), which is necessary to transport calcium within the cell, is encoded by the abnormal gene in Darier disease, identified as ATP2A2, which is located on chromosome 12q23-24.1. 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.
Symptoms
Individuals with Darier disease exhibit a wide range of symptoms and signs, from very subtle to extensive lesions that can be distressing for the patient.
The persistent, greasy, scaly papules that characterize the skin lesions are present in the following areas:
Seborrheic areas on the face, including the beard, eyebrows, ears, scalp, forehead, and regions around the nostrils and sides of the nose.
· Scalp and neck
· Central chest and back
Skin folds, including those in the groin, between the buttocks, under the breasts, and in the armpits
If several of these papules grow together, they may form larger warty lesions that can get quite smelly within skin folds. There may be a heavily crusted rash resembling seborrheic dermatitis. The papules have a firm, harsh feel like coarse sandpaper and can be skin-colored, yellow brown, or brown in color.
Darier disease frequently manifests in unusual ways.
· Flat, freckle-like lesions
· Blistered papules
· Large, raised , warty lesions
Papules have a linear pattern that corresponds to the distribution of skin-dermatomal cells during embryonic development.
For some people, Darier disease and acne conglobata, or cystic acne, are related.
Acrokeratosis verruciformis, also called keratosis folliculitis, is a condition characterized by warty papules on the dorsum of the hands.
Acrokeratosis verruciformis of Hopf is a specific variant of Darier disease characterized by papules on the hands and feet as the primary symptom.
A V-shaped nick at the free edge of the nail is another very telling sign of Darier disease, which is typically characterized by longitudinal broad stripes of white and reddish color on some or all fingernails.
Small white papules that resemble a white cobblestone pattern that affect mucous membranes are a common symptom of Darier disease, and hum overgrowth is another possibility.
Many patients with Darier disease have mild, unnoticed symptoms that appear before the age of 30. More severe cases may have a chronic relapsing-remitting pattern, and the symptoms may completely disappear.
Generally speaking, despite the severity of the disease, general health is good.
Diagnosis
The pathology is similar to Grover disease and the histology is known as focal acantholytic dyskeratosis with varying degrees of papillomatosis, skin thickening. The diagnosis of Darier disease can be made by its appearance and from the family history. A skin biopsy is also necessary.
HOMOEOPATHIC REMEDIES
When it comes to treating Darier disease, homoeopathy has a number of effective medications available, but the choice depends on the patient’s unique characteristics, taking into account the mental and emotional well-being as well as the spiritual and physical well-being. Homoeopathy is currently a rapidly expanding system that is practiced throughout the world.
In this situation, a constitutional therapy is recommended.
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