Keratodermia encompasses a very large group of dermatoses, in which the sufferer has failures in the corneal dermis. This is the syndrome of Unna-Toast, and Papillon-Lefer's keratoderma, and a mutating type of disease. Such violations experts usually fix on the palms, soles. One of the diseases included in this group is the keratoderma of Meled. We will consider it in more detail.
Features of the disease
Meled disease is considered a very rare hereditary disease. Dry, thickened areas of the dermis slowly cover new areas of the dermis. The lesions are red, covered with scales, an abnormal thickening is noted.
The disease was first detected in 1898.It was found among the residents of Fr. Meleda( Yugoslavia).Given the data of the last 25 years, the disease was recorded already in different countries around the world.
This pathology occurs in adolescents, whose age ranges from 15 to 20 years.
Meled disease
Causes of
The rare disorder that we are considering is fixed with the same frequency in both sexes. Typically, the first symptoms of pathology doctors notice at the birth of the baby. Other signs are manifested in the second, third year of life.
The disease is inherited autosomally-recessively. Several of the abnormal genes were found in each of the patients examined. According to studies, Meled's disease provokes the mutation of the ARS gene. This gene is located on chromosome 8( 8q24.3), its long shoulder. The function of this gene is in the encoding of the protein Slurp-1.This protein, according to researchers, takes part in adhesion, cellular signaling.
On what keratodermia are, this video will tell:
Symptoms of keratoderma Meleda
The inheritance of this disease is noted to be autosomal recessive. The disease manifests itself in a special way. The first symptoms of the pathology are visible even at the birth of the baby. The first signs of the disease are in the unusual redness of the dermis of the palms, feet. Damage to the dermis is manifested symmetrically, on both sides of the body.
With the growth of the child, the disease can spread to the entire leg, arm, grab the area of the chest, abdomen. Due to excessive dryness of the dermis, the patient is concerned about pain, discomfort. Sometimes, on the upper, lower extremities of children, increased hair growth is noted.
On the dermis the disease under consideration has the appearance of a thick horny layering, which differs in a yellow-brown color. Over the resulting stratum corneum, there are deep cracks. The patient may exhibit perioral erythema( derma around the mouth turns red).
On the nails, this disease manifests itself in the form:
- coilonichia( obtaining a nail-shaped spoon);
- hookability;
- dystrophy;
- pachyonihia( the nail plate is getting fat, becoming excessively tight).
The lesion focus is delicately contoured, which has a purple-violet color. The lesion can cover different parts of the dermis:
- forearm;
- palms;
- of the lower leg;
- rear of brushes;
- stops;
- rear stop.
In ailing patients, they fix local hyperhidrosis. It is the cause of the formation of black dots, which are clearly visible. These black dots are the excretory ducts of the sweat glands, which have been clogged with a secret. There is also a thickening, deformation of the nail plates.
Patients with congenital progressive acrokeratoma suffer severe perspiration. Sometimes hyperhidrosis can be accompanied by a very unpleasant odor.
Among the additional symptoms, we indicate:
- cracks in the tongue;
- brachydactyly;
- fusion of fingers, toes.
Diagnosis
In order to make an accurate diagnosis, you need an examination by a specialist. The doctor should carefully study the patient's history. Then he identifies the features of physical manifestations. At visual inspection, abnormalities of the dermis( redness, keratinization on the palms, feet) are visible.
Treatment of
Treatment of keratoderma usually lasts a very long time, up to several years. The essence of therapy of pathology is the control over certain symptoms. Treatment is usually carried out by several specialists:
- dermatologist;
- pediatrician.
To reduce the manifestation of hyperhidrosis, a doctor may prescribe the application of such agents to affected areas:
- "Hexahydrate of aluminum chloride".
- Aluminum Acetate. "
To reduce the manifestation of the disease, you can use special lotions, exfoliating ointments. Vitaminotherapy plays a special role in the treatment. Doctors prescribe such vitamins:
- E.
- A.
- B.
- Ascorbic acid.
In the therapy of any form of keratomycosis, doctors use "Neotigazone".Dosage is calculated by a specialist, it differs in each case. The dose of the drug varies between 0.3 and 1 mg per kg. This medication can replace vitamin A. Its dosage per day should be about 100,000 - 300,000 mg.
Prevention of the disease
Special preventive measures that could prevent the onset of the disease is not, because it has an autosomal recessive nature.
Complications of
The disease can be complicated by pyococcal infection, nail dystrophy, and other nail plate lesions. The following changes can occur in the patients:
- syndactyly;
- mental retardation;
- Gothic sky;
- folded tongue.
Forecast
Even complex therapy does not have a lasting effect. Despite the fact that modern medicine uses only the latest medicines, it is impossible to achieve complete cure for pathology.
Thanks to timely therapy, a professional approach to treating the disease, doctors can greatly reduce the symptoms of dermis damage, reduce discomfort.
Melad's keratoderma is reviewed in this video: