Dermatoses are included in the group of keratodermia, the basis of which is a malfunction in the process of keratinization. This is the disease of Unna-Toast, and the pathology of Meled, and the Papillon-Lefebvre syndrome).Most often there is increased keratinization, which is localized in such areas of the dermis as the palms and feet. One of such pathologies is mutilating keratoderma.
Features of the
Disease The keratoderma-curing specialists are also referred to as Fonvinkel syndrome, a hereditary keratolytic mutant. This pathology usually occurs in children in the second year of life. The pathology was first described in 1925.In some cases, there is a combination of the pathology in question with such diseases:
- ruby alopecia;
- pachyonihia;
- hearing loss;
- hypogonadism.
The peculiarity of this disease is diffuse horny layering on the dermis of the palms, the feet, which is accompanied by hyperhidrosis. After some time, the cord appears on the dermis.
Causes of
The specialists established an autosomal dominant type of inheritance of the disease under consideration. According to the research, the pathology is provoked by a mutation in the genes responsible for the keratin coding 6, 9, 16.
The development of keratoderma is largely affected by vitamin A deficiency, viral, bacterial infections, hormonal dysfunction, the presence of tumor diseases.
Symptoms of mutating keratodermia
The first signs of mutating keratoderma appear in 2 years. It is characterized by a horny layering( diffuse), which appears on the dermis of the soles, palms. In addition to affecting the dermis, hyperhidrosis is observed in this pathology.
With the progression of the pathology, the fingers show cords. There are also changes on the nail plates. These changes are manifested by the type of watch glass. Fonvinkel syndrome is characterized by the emergence of follicular keratosis in such areas:
- joints of elbows;
- surface of brushes( rear);
- knee joints.
Diagnosis
For a precise diagnosis, you may need a clinical picture, data, data from a diffodiagnosis, histological examination.
A patient with a suspicion of a keratolytic mutant is prescribed a histological examination. This diagnostic method enables specialists to detect:
- acanthosis;
- severe hyperkeratosis;
- granulosis.
A small amount of inflammatory infiltrates is found in the patient's dermis. They usually include in their composition such components:
- histiocytes;
- lymphocytes.
Also, a specialist may need to conduct a diffodiagnosis. To distinguish mutating keratoderma among other types of derma lesions, a specialist must take into account the peculiarity of mutation, which is not inherent in other forms of keratosis.
Treatment of
The modern medicine is incapable of completely curing the disease. All that experts can do is to minimize the discomfort, the appearance of keratosis. Treatment of the pathology of the dermis we are considering is done in two ways:
- is medicated;
- therapeutically.
You can also use traditional medicine.
Therapeutic way of
To alleviate the symptoms that occur with mutating keratoma( hereditary), doctors can prescribe external therapy. It involves the use of:
- ointments that contain aromatic retinoids;
- steroid medication;
- keratolytic preparations.
Medication-based
Most specialists use a medication such as "Neotigazone" for general therapy. The dosage is established by the attending physician, who takes into account the severity of the disease. The dose may be 0.3 - 1 mg / kg of the body weight.
If this drug is not available, you can apply vitamin therapy. The patient is prescribed vitamin A. The dosage is 100 to 300,000 mg per day. Take this vitamin is necessary for a long period of time.
Folk remedies
Many people use old recipes from the people to treat keratoderma. Any medicine can only soften the dermis, speed up the process of its peeling.
For the application of compresses, the following can be used:
- pulp of an aloe leaf;
- tincture of onion peel + apple cider vinegar.
You can also use a tincture of propolis, a mixture of glycerin and table vinegar. These funds must be applied to the affected area.
Disease prevention
The disease is hereditary, it arises from the mutation of the genes responsible for the keratin coding 6, 9, 16. The only preventative measure is the genetic consultation of the child's parents. If they find a defective gene, the doctor should report the possibility of a child with this syndrome.
Complications of
Complications of the disease can be an infection.
Forecast
If a person has such a pathology as mutating keratoderma, the forecast is put by the experts unfavorable. The doctors have not yet managed to get rid of the disease completely. Even prolonged complex treatment does not help, it is only able to improve the condition of the dermis.