Keratoderma of Wyna-Toast: causes, symptoms, diagnosis, treatment
Keratodermia( mutilating, Meleda, Papillon-Lefevra) are represented mainly by hereditarily transmitted lesions of the dermis.Only in isolated cases this pathology is acquired.The main feature of the disease is the corneal dermis, which usually occurs around the palms, soles.Kernodermy Unna-Toast, which we will consider in more detail, is one of such diseases.
Features of the disease
Unna-tost disease is also known among specialists, like ichthyosis of the palms, soles( congenital), Unna-Tost syndrome, congenital palmar-plantar keratoma. This disease is characterized by an autosomal dominant type of inheritance.
Most often, the defeat covers the palms, feet.Only in some cases, the cornification of the dermis is noted solely on human feet.
Development of keratodermia Unni-Toast
Causes of occurrence
Congenital ichthyosis is the result of disturbance of the normal dermis coronary process.Pathology is characterized by an autosomal dominant type of inheritance.
The disease is transmitted to
children in the event that both parents have a mutated gene.This pathology can occur in different forms:
Usually keratoderma of Wina-Toa is recorded in very premature infants.In such babies, doctors usually diagnose a lot of vices in development.
- The baby's derma looks like a shell, over which there are multiple cracks( very deep).The resulting cracks usually bleed.
- In children, the syndrome of Wyna-Toast can manifest itself in deformities of the face.Mucous membranes are very actively growing, they can even turn outward. Almost immediately after birth, a baby with this pathology dies.
Sometimes specialists can save a child with this disease.Over time, congenital disease passes into erythroderma.Rarely are recorded cases when the disease disappeared completely.
Sometimes a slight form of ichthyosis is observed.It is characterized by very minor changes in the dermis, the pathology of the internal organs is practically not observed.On the skin are visible multiple foci of redness, to which puffiness joins.Cracks characteristic of this pathology can occur in such areas:
- person( in rare cases);
In a patient with this pathology, hyperhidrosis is observed.In addition to affecting the dermis on the above parts of the body, the following structures can also be affected:
The disease can accompany anomalies in the structure of the skeleton, the pathology of some internal organs( endocrine, nervous systems).
Unna-Toa keratoderma and other forms of malaise are reviewed by a specialist in this video:
A feature of ichthyosis is that the pathology of the dermis appears in the maternal womb.At birth, the baby already has visible changes in the skin.Very rarely, pathology is manifested during the first year of development of the child.Based on such characteristics, specialists usually make an accurate diagnosis.
When examined by means of a microscope of affected tissues, the following are found:
- acanthosis( increased number of cells in the growth layer);
- granulosa( thickening of the granular layer);
- hyperkeratosis( scaling of stratum corneum).
Diffiagnosis with other diffuse types of keratoderma may be necessary.
Treatment of keratoderma Unni-Toast
It is almost impossible to cure this peculiar congenital pathology completely. The only thing a professional doctor can achieve is a stable remission. To achieve this effect, a specialist usually prescribes the intake of the following vitamins throughout the month:
After a break in a month, the vitamin therapy is repeated again for a month.It is more convenient to use an oily solution.He is prescribed 3 to 5 drops twice a day.
If the disease is very severe, the above vitamins are administered intramuscularly.In addition, the doctor can prescribe such drugs:
- Aromatic retinoid.
- Vitamins B.
- Iron glycerophosphate.
- Ascorbic acid.
A specialist can prescribe glucocorticosteroids if the case is very severe.
Prevention of disease
No preventive measures, because pathology is innate.
Due to a significant decrease in immunity, its complete absence, various infections can attach to the underlying disease.
- Very often pathology leads to the death of a newborn.Sometimes the disease can go on its own( single cases).
- 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.