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December 20, 2016 00:05

Gipomelanoz - Symptoms and Treatment gipomelanoza

Gipomelanoz is a pathology formation of pigmentation of the skin on the background of any disease.At the heart of gipomelanoza is a violation of melanin by melanocytes, which is located in the skin.This pathological condition may manifest itself in the form of leucoderma, reduced the amount of melanin as well as its complete absence.

starting moment in the development of gipomelanoza is damage to one or more parts of the development and transformation of melanin.This may be the absence of melanocytes in the skin, forming a complete violation of melanosomes and their transportation to the keratinocytes.

main clinical manifestation of disease are considered blind spots arising from this illness with dyschromia surface of the skin.Often it noted gipomelanoz among kids that occurs soon after an illness.

For the correct diagnosis is often used histological examination.After all, in its absence, you can skip gipomelanoz that leads to childhood developmental delay.Therapeutic goals in pathology focused o

n peeling treatments and the use of retinoids.

reasons gipomelanoza

appearance of white spots can occur in the first days of the baby's life, because gipomelanoza reasons laid at the genetic level.So, there is melanin synthesis of crashing - a special pigment responsible for coloring skin.

melanin production begins by the action of special enzyme - tyrosinase, and then run a lot of chain reactions on the molecular level.This complex process is regulated by a special combination of genes, among which damage occurs.

For reasons gipomelanoza be found in the genetic apparatus.In addition the transfer of diseases characterized by recessive type, especially among consanguineous marriages.gene carrier may be unmasked by the presence of gray hair area with clear boundaries, freckles and white spots on the skin.

Because the exact cause of the gipomelanoza not been clarified, and genetic damage is impossible to have any effect and therefore there is no pathogenetic treatment.Through research we have found ways and drugs that can partially normalize the synthesis of melanin.

Symptoms gipomelanoza

In view of the fact that the pathological condition is genetic causes of disorders of melanin, the first clinical manifestation gipomelanoza can be seen already from the birth of the baby.

Symptoms gipomelanoza expressed formation of white skin area with clear boundaries, which differs from the other color of the skin.The number and size of these spots can vary and increase over time.

If the baby is pale or white skin, then the symptoms gipomelanoza not immediately possible to notice.For a more accurate visualization using Wood's lamp is required for research plot without pigmentation in a dark room.

With this lamp is enhanced contrast between normal coloring of the skin and gipomelanozom.In the case of gipomelanoza Ito possible except cutaneous manifestations of nervous system pathology with neurological disorders as mental disorders and increased convulsive readiness, as well as anomalies of the skeletal system.

Teardrop gipomelanoz

This form of disease can often be observed in representatives of the female half of the population aged 35-55 years.Most susceptible gipomelanozu women with lighter skin tone, as well as conducting a long time in direct sunlight.

As a result, parts of the defeat marked decrease in the number of melanocytes is almost 2 times.In addition there is the view that the teardrop-shaped gipomelanoz associated with HLA-DR8.

Genetic factors play an important role in the development of the disease, especially if it is seen in close relatives.

gipomelanoza clinical manifestations characterized by the appearance of spots on the skin of white color and round shape.The diameter of these altered areas reaches up to 1 cm.

Teardrop gipomelanoz first appears on the lower leg (extensor surfaces), and then spread to the forearm, upper back and chest.For this is not a pathological condition characterized by loss of skin facial coverings.

Progression process occurs with age, as well as an excess stay under direct sunlight.

Gipomelanoz Ito

pathology observed in both men and particularly women and the prevalence is second only to Neurofibromatosis and tuberous sclerosis.Gipomelanoz ito refers to sporadic disease, but it is possible to inherit the recessive and dominant type.

Underlying pathology is a failure of cell migration from the neural tube in utero, resulting in marked anomalous location of gray matter in the brain, as well as the insufficient number of melanocytes in the skin thicker.

melanoblast migration occurs during the second or third trimester of pregnancy.At the same time there is movement of the neurons, resulting gipomelanoz ito includes clinical symptoms and pathology of pigmentation disorders of the brain.

Cutaneous symptoms are expressed in areas of hypopigmentation of irregular shape (curls, zigzags, waves).In most cases the data are located near the centers of Blashko lines and their appearance can be observed in the first days or months of life the baby, but until adolescence, they may become less noticeable or even disappear.

neurological symptoms characterized by intellectual disability, seizures, which are characterized by their resistance to anticonvulsant often suffer from autism, muscular hypotonia and motor disinhibition.In a quarter of cases there macrocephaly.

addition frequently observed pathology of other organs, such as heart disease, abnormality of the structure of genitals, face, deformation of the spine, feet, eye symptoms, as well as violation of the structure and growth of teeth and hair.

Idiopathic gipomelanoz

At the heart of the development is a violation of gipomelanoza stages due to lack of melanin synthesis of melanocytes, a failure in the formation of melanosomes and their full migration.

Melanocytes come from ectomesenchyme.Their differentiation has 4 stages.The first - the emergence of melanocyte precursors in the neural crest, the second - the migration of melanocytes in the dermis is thicker in the direction of the basement membrane of the epidermis.Their movement is further noted in the epidermis, and finally the step of forming processes (dendritic) when the cell takes its position in the epidermis.Idiopathic

gipomelanoz developing in case of failure in one of these steps, resulting in melanocyte may reside in an unusual location for it, which is why a certain area of ​​the skin remains "colorless" as pigment synthesis is missing.

Idiopathic gipomelanoz can manifest in children or older.Also under the influence of ultraviolet rays possible progression of the disease.

Idiopathic guttate gipomelanoz

main cause of the disease is quite difficult to identify, since almost 100% of cases - it is a genetic breakdown.Idiopathic guttate gipomelanoz may occur immediately after birth or during adolescence.The most common type of pathology has hronicheky flow with periodic relapses.

Clinical manifestations of the disease are lesions gipopigmentazii different localization (lower leg, forearm, back) and a diameter of up to 1 cm. Sites are located separately from each other and are not able to merge.

most often idiopathic guttate gipomelanoz observed in women with lighter shade of skin, especially those living in areas with increased exposure to sunlight.Also at the first appearance of lesions on the shin, then under the influence of insolation areas of depigmentation are increasing.

pathogenetic therapy aimed at removing the causative factor does not exist, in connection with which the symptomatic treatment used to reduce the intensity of the manifestations of disease.

Gipomelanoz the child

Lack of pigment produced in children may indicate the presence of the syndrome Vardeburga that is transmitted genetically dominant type.Its main clinical manifestations are considered white strands of hair, lots of hypopigmentation on the skin, a different color of the iris and the eye level, as well as a broad bridge of the nose and congenital deafness.

addition gipomelanoz the child is observed for tumoroznom sclerosis, which is characterized by the appearance of white spots of up to 3 cm and the localization on the trunk and knots - on the forehead, hands and feet.In addition to skin manifestations observed mental retardation, epilepsy, phakomatoses retina cystiform formation in the kidneys, lungs, bones and heart rhabdomyomas.

Gipomelanoz the child seen with gipomelanoze Ito with the advent of hypopigmented areas of the skin of various shapes in the form of waves and strips.These symptoms may disappear with age alone.

Vitiligo is also defective synthesis of the pigment, which is characterized by the appearance of white skin with a clear outline.Localization is possible on the face, genitals, feet, hands, at the joints.

Diagnostics gipomelanoza

Violation pigmentation processes may manifest various forms.For verification of pathology must be used in addition to visual inspection study using Wood's lamp.Especially often used in the presence of her light skin and clearly manifested pathology.

gipomelanoza Diagnosis is based on the identification of clear boundaries hypopigmented hearth in the glow of a lamp in a darkened room.Thanks to her, there is an opportunity to discover the site and verify it.

Diagnostics gipomelanoza Ito further includes performing computed tomography of the brain, which found an increase in the third and lateral ventricles, blurred boundaries between the substance of the brain, as well as atrophy of the frontal lobes.

Histological examination barks possible to identify an insufficient number of melanocytes in the area of ​​hypopigmentation.Also at gipomelanoze Ito possible presence of other features in the outbreak, for example, vascular nevi, blemishes cocoa color nevus Ott Mongoloid or blue spots.

Treatment gipomelanoza

This pathological process is distinguished by its proliferation at the genetic level, and therefore the pathogenetic treatment does not exist yet.Used symptomatic therapy, the main direction of which is to stop the generalization of pathology and reduce its clinical manifestations.

gipomelanoza Treatment involves the use of corticosteroid agents, the introduction of which is held inside the chamber.In addition, various studies confirming the effectiveness of the use of topical retinoids, pimecrolimus (Elidel) and cryomassage lesions.

gipomelanoza treatment also may be performed by photochemotherapy, whereby marked activation of melanin pigment cells.In addition, we recommend carrying out substitution therapy with Melagenin.Its action is aimed at the stimulation of melanocytes to the synthesis of the pigment.

As for bio-resonance therapy, it is directed at restoring the normal functioning of the nervous system, as well as increase the level of immune forces of the body.

People's treatment is also possible with this type of pathology, but before its use, be sure to consult a doctor.

Prevention gipomelanoza

specific prevention gipomelanoza does not exist, since the pathology is genetic inheritance.However, still there are techniques that can reduce the risk of occurrence gipomelanoza or its recurrence.

main precipitating factor of generalization and the progression of the process is considered to be excessive sun exposure.As a result, it is necessary to inform the public about its negative impact, not only in respect of gipomelanoza, but also the likelihood of developing cancer.

gipomelanoza prevention is to avoid exposure to direct sunlight on unprotected skin, especially in the period from 11.00 to 16.00.This also applies to fans of indoor tanning.To protect the skin a special cream to use, a hat and clothes that cover areas gipomelanoza.

gipomelanoza Prevention includes the use of sunscreen cosmetics in hot weather, as the UV radiation can be reflected from the surrounding objects and the earth, pass through clouds and is recommended to be on the street during the day when there is no urgent need Consequently, no.

forecast gipomelanoza

themselves areas of hypopigmentation in the form of white spots do not pose a health hazard, but when the first clinical symptoms should see a specialist for further diagnosis and therapy appointments.The sooner the pathology is detected, the more likely to stop the process and prevent relapse.

forecast gipomelanoza favorable, but in the excessive influence of sunlight, it may be spread to healthy tissue as well as excessive sun exposure can reduce the number of melanosomes and pigment.

We can not prevent the possibility of the carcinogenic process under the influence of sunlight.This is due to a malignant degeneration of cells as a result of gene expression violations.In addition, each person has birthmarks, which are also able to change under the influence of the sun.

So gipomelanoz - not terrible pathology, but nevertheless calls for a special approach and compliance of specific prevention of occurrence and recurrence of the chronic course.