Hypertrichosis syndrome
- Hypertrichosis is a disease that is also called Ambras syndrome , and it is expressed in an excessive amount of hair on the surface of the human body.
- Hypertrichosis can affect a large surface of the body, including the face, legs, arms, or be localized in a separate area - all this depends on the reasons that caused the disease.
- Hypertrichosis is either inherited (it is called congenital), or acquired during life for various reasons, which will be discussed below.
- The disease can manifest itself to a severe degree - in this case, abnormal phenomena can also affect the jaws , when the child either erupts teeth late, or they never erupt at all. Fortunately, this degree of severity of the disease is extremely rare, and doctors and scientists have still not been able to figure out which genetic defects cause such mutations.
- The hair that covers the body, face or limbs, depending on where exactly it grows, has different density, color and thickness, and the degree of its hardness and the speed at which it grows vary.
Excessive hair growth
Troubleshooting methods
If you have coarse hair growing on the surface of your ears, you should get rid of this problem. A wide variety of methods will be available to you. Each of them has the right to exist, but in order to choose the optimal one, you need to consider their features, advantages and disadvantages.
- Hair trimming . If there is slight hair growth, the easiest way is to trim off any stray hairs. Scissors are usually used for this, but doing this yourself risks damaging your ear. It is better to buy a special trimmer for removing hair from the nose and ears. It is popular among men as it allows you to quickly and safely get rid of the problem. The disadvantage of this method is that the hair grows quickly, but it becomes stiff and causes discomfort.
- Removal with tweezers . Pulling out hairs from the roots has a longer lasting effect. For several hairs, this method is quite suitable. But with abundant hair growth, the procedure will take too much time. In addition, it is worth considering that such removal is very painful, especially in the ear area. Over time, the hairs become thinner and less noticeable, but this can trigger the awakening of new follicles. Another disadvantage is the risk of infection, as the skin is injured.
- Photoepilation. Using a special device, a light pulse is applied that destroys the hair. This procedure is only suitable for dark hair and requires several sessions.
- Laser hair removal . Hypertrichosis can be eliminated forever by exposing the bulbs to a laser beam. There are diode and more efficient alexandrite lasers. The procedure requires several sessions, from 3 to 12. It costs a lot, but can completely solve the problem.
- Drug treatment . Depending on the cause of hypertrichosis, hormonal medications can be used to restore balance and stop pathological hair growth. You need to be extremely careful with hormones. Oral contraceptives can affect the female body. The medicine is prescribed exclusively by a doctor and after a series of tests.
In some cases, hypertrichosis is eliminated through the use of several methods at once. This is necessary in order to influence the cause of its development and prevent the problem from occurring in the future.
Signs of hypertrichosis
- The clinical manifestations of the disease hypertrichosis are excessive hair on the body, as well as on the face, and, equally, this phenomenon can be characteristic of both representatives of the strong and weak half of humanity.
- If hypertrichosis is congenital, then hair covers the entire human body, excluding the feet and palms. Hair growth is especially pronounced in the area of the face, shoulders, and very often - hypertrichosis of the auricle. Hair can grow tens of centimeters long (this applies to the face area).
Hair growth
- With vellus hypertrichosis in infants, the covering of the body with light hairs is clearly visible, excluding the limbs. Then the hair falls out (this happens around the age of one year), but hair growth begins on the limbs.
- There is also focal hypertrichosis, in which black, coarse hair grows in certain areas of the body affected by some pathology.
- Expressed in the prepubertal period, hypertrichosis is expressed by hair growth in the temple area , from where it spreads to the forehead. The presence of thick eyebrows, long dark hair on the upper back, arms and legs - these are the signs of this type of hypertrichosis.
- Hypertrichosis can be acquired as a result of taking certain medications, and depending on the type of drug, it affects the limbs and facial area. With secondary hypertrichosis (acquired as a result of diseases such as hepatic porphyria, anorexia, celiac disease, juvenile dermatomyositis, oncology, AIDS, etc.), excessive hair growth on the face, body and limbs may also occur.
- Thus, a sign of hypertrichosis is excessive hair growth in areas of the body that are characterized only by the growth of vellus hair.
Causes and risk factors
Despite the fact that hypertrichosis is not considered a very common disease, the causes of its manifestation are of interest to many people. Contrary to popular belief, this rather interesting phenomenon is not a consequence of pathology, when a very high content of male hormones is found in the blood, affecting increased hair growth in androgenic areas, but has other reasons, since it can be observed on any part of the body.
Among the known causes of the disease today are:
- genetic pathologies , including abnormalities of the central nervous system and skeletal system due to viral diseases suffered by the mother in the initial stages of pregnancy (in this case, hypertrichosis is one of the symptoms);
- spinal dysraphism is a congenital disorder that occurs due to impaired development of the fetal neural tube during intrauterine life (more often manifested by the appearance of an excessive amount of hair on the lower back);
- neurofibromatosis - characterized by the appearance of uncharacteristic long hairs in the chest area and is a serious hereditary disease;
- problems with metabolic processes and hormone imbalance , which are often associated with certain periods of a person’s life (for example, changes in the body of adolescents), a woman carrying a baby or problems with the endocrine glands (for example, hypothyroidism, hyperthyroidism, malignant neoplasms of the adrenal glands, thyroid gland, ovaries);
- neoplasms in the brain and mammary glands;
- serious mental disorders and psycho-emotional disorders (among the most common is epilepsy);
- formation of scars on the skin in places of injury and constant mechanical impact on the same area of the body (blood flow accelerates in this place, which is why stimulation of hair follicles is observed);
- premature puberty.
In addition, increased hair formation can also be affected by constant cosmetic and therapeutic procedures that help increase blood flow to the hair follicles: for example, paraffin therapy, pepper patches, mustard plasters, the use of ointments with glucocorticosteroids, long-term massages, cryotherapy, etc. You should avoid and any hair removal procedures, especially on the face.
Based on the specific cause of hypertrichosis, one can judge its form: congenital or acquired.
Important!
When assessing the condition of a person with a high level of hair growth, one should also take into account such a factor as nationality, because what would be the norm for some peoples, for representatives of others, will most likely turn out to be manifestations of hypertrichosis (for example, excessive hair formation is typical for residents Caucasus, Israel and citizens of Arab states, while for Europeans this is already a pathology).
Gene for hypertrichosis in humans
- Hypertrichosis is characteristic mainly of men , transmitted via the Y chromosome in conjunction with sex. If we talk about congenital generalized hypertrichosis, it is a fairly rare disease of autosomal dominant etiology.
- His discovery dates back to the 16th century and the cause was identified as a mutation on chromosome 8, which is also called pericentric inversion. The disease can be transmitted both to each next generation and across generations.
- There is also a version that speaks of X-linked inheritance of the disease, since medicine has described a case of a father transmitting a mutant gene only to his daughters, while none of the sons became a carrier of the disease. Also, the reasons for the manifestation of congenital vellus hypertrichosis are not fully understood, since the main version put forward by researchers speaks of an autosomal dominant pathway by which the disease is transmitted, but, as in any rule, there are exceptions: there are certain cases of the disease that contradict the main version.
- No pathologies of genetic origin were identified, as well as changes in the endocrine system. It has only been established that congenital hypertrichosis can manifest itself if either one of the parents or both are susceptible to it.
Cause of the disease
Prevention of hypertrichosis
The diagnosis is difficult to prevent; many people do not know that they are a carrier of the mutating gene, unless their immediate relatives have had signs of increased hairiness.
But, if a person knows that he is at risk, he must adhere to the following preventive measures:
- do not injure the ear;
- avoid chemical and thermal exposure;
- take hormonal drugs with caution;
- undergo medical examination in a timely manner;
- treat infections promptly.
It is not recommended to use products that mechanically injure the skin: scrubs, peelings, aggressive masks. But, you should know that if the patient is hereditarily prone to hypertrichosis, then even preventive measures are not always able to prevent ear hair growth.
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What are the types of hypertrichosis?
- Hypertrichosis has several types - depending on the time of appearance, a distinction is made between congenital and acquired disease.
- From the scale of hair coverage of areas of the body - focal, covering local areas, and generalized, which spreads over a large surface of the body.
- There are also differences in which area is affected - elbows, neck, ears, spine , etc.
- Another division into species depends on how the disease appeared. Either for no obvious reason, or it arose against the background of another pathological condition.
As for acquired hypertrichosis, it can also be generalized, and in turn is divided into the following types according to etiology:
- Medicinal , provoked by taking certain medications.
- Secondary , which appears as a consequence of another disease that acts as a background for the development of hypertrichosis.
- Pushkovy , the background for which is usually cancer.
- Focal , occurring in places of constant friction or compression, or in the presence of inflammation that has become chronic (as a rule, in such cases, hair can be removed quite easily by regular shaving or epilation).
- Traumatic , which manifests itself as a result of injury or burn, as well as on the face as a result of constantly plucking hairs, or due to poor shaving - as a result, hair follicles are activated, and in place of thin and light vellus hair, terminal hairs actively grow, which are much more harder and darker.
In the case of local hypertrichosis, hair growth affects certain individual areas of the body. The main types of hypertrichosis, classified according to location.
So, if hypertrichosis is localized in:
- The chest area, which is also covered with hair, like fur.
- On the lower back – here, as a rule, long black hair grows, which resembles a bun; by the way, it is called a faun’s bun.
- A nevus is a kind of “birthmark” from which dark, coarse hair grows. It can be localized on almost any part of the body.
Local
In addition, in both men and women, hypertrichosis can affect the face and ears. And one more thing, with hypertrichosis there is excessive hair growth even in ordinary places, for example, on the pubis or in the armpits, and this is typical for representatives of both sexes.
Forms of the disease
So, there are 3 forms of the disease
Congenital universal - when the child is born completely covered with long vellus hair, it can also initially manifest itself only by the connection of the eyebrows with the scalp and further progression of hair growth covering all parts of the body (excluding the soles and palms)
Congenital local - local manifestation of hair growth. Often in combination with nevus - hyperpigmentation of individual areas
Acquired - due to the influence of external and internal factors.
Clinical hypertrichosis manifests itself in the form of hair growth up to 10 cm long or vellus hair.
Hair can grow everywhere or in limited areas of the body. For example, fusion of eyebrows, excessive hair on the legs, back and face are also signs of hypertrichosis with hair unusual for the nation (southerners are hairier than northern peoples).
At the same time, it is necessary to differentiate in case of excess hair growth:
hypergonadism, when it appears at 8-10 years of age;- tuberculosis;
- diabetes;
- alcoholism;
- alcoholic or vascular encephalopathy.
The covering of the entire body of a newborn baby with fluff is also hypertrichosis. This is how hypertrichosis manifests itself in the form of atavism.
Causes of hypertrichosis
The causes of the disease are mainly associated with the types of hypertrichosis listed above, since they are the basis for classification.
Thus, hypertrichosis can be caused by such reasons as:
- A congenital mutation that developed as a result of a problematic pregnancy or an infectious disease suffered by the expectant mother during the first trimester of pregnancy.
- Taking certain medications.
- Disturbances in the functioning of the thyroid gland and the endocrine system as a whole.
- Certain conditions or diseases that cause symptomatic hypertrichosis, for example, alcoholism, nervous exhaustion, traumatic brain injury, etc.
- Damage to the skin due to injury or burn.
- Hair follicles may increase their growth as a result of the development of a malignant tumor.
And, of course, the cause of congenital hypertrichosis is a gene mutation that affects successive generations.
Hypertrichosis in men
- The most common places for hair growth in men are the back, shoulders and arms ; hair covering the ears is also common. Experts consider such abundant hair growth to be a kind of atavism, and most often it manifests itself already at the birth of a boy.
- The causes of hypertrichosis in men are almost all of the above, and in this regard it is essentially no different from women. The only thing that affects the intensity of the disease is a greater amount of male hormones in the body, as opposed to female ones. This greatly promotes abundant hair growth and distribution throughout the body and face while preventing hair growth on the scalp.
- Also, in men, the phenomenon of fused eyebrows is much more common , which is one of the localizations of hypertrichosis, which in such a case is called limited.
- And, above all, men more often become victims of traumatic hypertrichosis due to constant shaving, which can cause skin irritations.
In men
Hypertrichosis in women
- Hypertrichosis in women, largely coinciding with men's, has a localization , namely, they begin to grow a mustache or beard, which in principle is not inherent in women, in whose body female hormones normally predominate, which promote hair growth. not on the face or body, but on the head.
- In addition, a type of hair growth characteristic of men is possible, when pubic hair does not grow in a triangle, as usual, but takes on a diamond shape. More often in this case they talk about hirsutism , but hypertrichosis can also be expressed in this way.
- The most common locations for hypertrichosis in women are the nasolabial folds and chin, arms, chest, and genitals . The disease can manifest itself at any age, both in youth and during menopause against the background of hormonal changes in the body.
- Methods used by women also contribute to hair strengthening and abundant growth: depilation, shaving, plucking individual hairs.
Happens also in women
Hypertrichosis of the auricle
The appearance of excess vegetation in the nasal cavity and ears is one of the most common types of hypertrichosis, although mainly in males.
Causes
The main reason for the development of the problem is the increased amount of androgens in the blood, and this feature may well be inherited: during pregnancy, under the influence of some external factor, a mutation occurs in the gene responsible for the strength of hair growth of the skin, and after joining the Y- chromosome, the disease will be transmitted to future boys born in this family.
In the absence of other physiological pathologies, no special treatment is needed in this case; standard cosmetic manipulations will be sufficient.
In female representatives, this pathology occurs infrequently and in most cases develops against the background of excess hair growth, manifested in the growth of a beard, mustache and hard covering in the back, abdomen and chest.
Such virilism is often diagnosed with individual endocrine and neuroendocrine diseases or as an individual feature in completely healthy women. In the latter case, the main possible reasons are increased secretion of androgens or increased sensitivity of body tissues to their effects.
Signs
The congenital form of hypertrichosis may not manifest itself in any way immediately after the baby is born. Normally, the body of a newborn is often covered with a thick layer of fluff - lanugo, which disappears on its own after a few weeks or months (no more than four).
If a boy has a hereditary predisposition to hypertrichosis of the auricles, then the first signs of pathology will be noticeable only by the age of 17, as soon as the hormonal changes in the body come to their logical conclusion and the content of hormones in the blood remains at a consistently high level.
In the case where increased hair growth in the ear area was not observed before this age, then, most likely, the first signs will appear after 35 years. Moreover, it is likely that in this case, increased hair growth will coincide with scalp baldness, which occurs due to an excess of androgens in the blood.
Hair can appear both along the edge of the ear and on the skin of the external auditory canal. Usually this feature does not cause any trouble, although there are those who consider it a significant cosmetic defect.
Hypertrichosis in a child
- The first hair that appears on a baby while still in the womb is called lanugo and is essentially the original down, which usually falls out by the 36th week of fetal development, but if the birth is premature, then the down may be present on the newborn.
- Then the body is covered with vellus hair, thin and light, practically without pigment. And especially hard, thicker than usual, and dark-colored hair is called core hair.
- So, if a child is born covered with lanugo hair, then, as a rule, they fall out over time, but their patchy growth due to certain malformations, and then careful examination and treatment are required.
- A child who is developing and growing rapidly may develop hair on the forearms and lower legs , which is not a pathology. Representatives of some nationalities may actively grow shaft hair (for example, Gypsies, Jews or Caucasians) - this is also considered the norm.
- It is important to determine the cause of premature hair growth in girls in those places that are characteristic of the action of sex hormones, such as the pubic area, inner thighs, armpits, abdomen and chest .
- Then a parallel examination by both an endocrinologist and a gynecologist is necessary to determine whether this fact is the result of hypertrichosis, or caused by disorders in the endocrine system.
- And, of course, it is important to determine whether hair growth is a consequence of taking glucocorticoids and other drugs. If the cause of hypertrichosis in children is not determined by external factors, doctors talk about idiopathic hair growth , which is determined by the Ferrimon-Gallwey scale, which evaluates hair growth in a particular area of the face or body using a four-point system.
- If the number of points exceeds 7, they speak of hormonal disorders.
Hypertrichosis in children
Acquired form of the disease
If it turns out that the appearance of hypertrichosis is not associated with heredity, diagnostics of the patient’s body is required in order to prescribe adequate treatment.
For example, the acquired cannon form of the disease is very often the first symptom of the appearance of malignant tumors of various organs: these can be the lungs, mammary glands, bladder, uterus, gall bladder or large intestine.
A timely diagnosis will help identify a developing dangerous disease, and, after the necessary treatment is carried out, hair growth will also return to normal.
In some cases, the appearance of hypertrichosis can be observed after injuries, scars and other damage to skin areas.
This is especially likely in cases where the skin is subjected to constant traumatic effects with the help of ointments, patches, depilation, etc.
This leads to damage to peripheral nerves, which in turn can become a catalyst for increased hair growth.
Often limited hypertrichosis appears in those places that are exposed to aggressive medicinal ointments containing glucocorticosteroids.
These drugs include, for example, psoriasin, minoxidil, danazol, etc.
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Very often, the development of hypertrichosis indicates some other disease: for example, in children, hair in the area of the shoulder blades may appear due to tuberculosis.
In adults, increased hair growth in different areas of the body can signal a brain tumor or diabetes.
In men, this disease sometimes develops together with severe forms of alcoholism and signals the presence of alcoholic encephalopathy.
Hypertrichosis of the ears
- This localization of the disease is typical for both women and men. It can also be hereditary or acquired under the influence of external factors or other pathologies. The most common causes of ear hypertrichosis, in addition to a hereditary factor determined by a gene mutation, is the transformation of epithelial tissue into cells that contain hair follicles.
- In addition, there are a number of other common causes for hypertrichosis - injuries, surgical interventions, malignant neoplasms, pathological conditions during pregnancy, taking medications, mechanical.
- Usually, if there is little hair, more thorough ear hygiene is sufficient, but if there is too much hair and it continues to grow rapidly, a doctor’s intervention is necessary.
It also happens in the ears.
Signs of incipient hypertrichosis of the auricle are the following:
- The hair in the ear area becomes thicker.
- Coarse hairs appear in the middle of the ear.
- The hair growth area increases.
- The person begins to experience discomfort.
- It is also necessary to carefully monitor if the baby was born with small stubble on the ear, whether it fell out during the first few months after birth. If not, you need to see a doctor.
- In addition, age also plays an important role - the older the person, the greater the risk of ear growth . To determine the reason for the activation of the follicles, it is necessary to examine not only the hairy areas, but also the condition of the body in general.
- Among the methods that can be used to combat the problem, if it is minor, is cutting the hairs and removing them with tweezers . These are not the most reliable methods, since they can lead to both mechanical damage to the ear itself and transformation of the hairs into harder ones. Photo and laser hair removal methods do a better job of removing hair
- The first is carried out exclusively on dark hair and is quite long-lasting, the second affects the hair follicles and can solve the problem radically. Treatment with hormonal drugs is also possible which is carried out exclusively with a doctor’s prescription and under his constant supervision.
Treatment
There is no universal treatment regimen for hypertrichosis. Therapeutic tactics are formed taking into account the cause of the development of the pathology. If it is the result of disorders of the endocrine glands, the underlying disease is treated. This solution allows you to achieve a reduction in increased body hair.
If the abnormality is caused by gene mutations, there is little that doctors can do. They advise such patients to undergo permanent hair removal. There are several ways to do this, the choice of method is made taking into account the place where thick hair grows. Electric hair removal is often prescribed for adults. During this procedure, a needle is inserted into each follicle at a right angle, then an electric current is supplied to the hair follicle. It destroys the root system. Dead hairs are removed with tweezers after the procedure. One session lasts on average half an hour. During this time, the specialist manages to treat a small area of the body, so the number of sessions directly depends on the degree of hair growth.
Children cannot radically remove vegetation until the end of puberty, so doctors recommend that they bleach their vegetation with hydrogen peroxide.
Treatment of hypertrichosis
- Like any treatment, the fight against hypertrichosis is aimed primarily at eliminating the cause of the disease. Therefore, treatment methods vary.
- So, if hypertrichosis is caused by disturbances in the functioning of the endocrine system, then the underlying disease is treated first. If the treatment is carried out on the basis of taking hormonal drugs, then in the case of hypertrichosis developing under their influence, the drug is replaced by its analogue.
- Before starting treatment, consultation with specialists such as an endocrinologist and dermatologist , and women should also be required to visit a gynecologist.
- laser and photoepilation , the use of chemical depilatory agents, and exposure to intense pulsed light remain some of the most effective methods for treating hypertrichosis Among medications, doctors highlight eflornithine, which is involved in the process of hair growth.
Limited
Diseases associated with excess hair growth include hirsutism, which is often confused with hypertrichosis. You should know that the first disease is characteristic only of women, and it is manifested by hair growth characteristic of the male type. The second disease, hypertrichosis, can occur in both men and women, and at any age.
Diagnostics
Such a disease requires an integrated approach, where therapy should begin with a visit to a dermatologist, endocrinologist and gynecologist - these specialists give their opinions. After this, it is necessary to perform a biochemical blood test, which will determine the patient’s hormonal background, as well as the stage of the main pathological process. If congenital hypertrichosis dominates, the photo of which is shown below, then from laboratory analysis it is possible to draw a conclusion about the predominant disease of the endocrine system.
Additionally, the doctor must perform a visual examination and find out in detail the history of the characteristic disease. The patient's complaints indicate a major disease in the body. Special instrumental examinations are not needed. After establishing the correct diagnosis, therapy should begin as scheduled.
Family with hypertrichosis
- The best known familial case of hypertrichosis are members of the Gonsalvus family , which lived in the 16th century. His father, Petrus Gonsalvus, was perceived as a monkey-man from childhood and it was in this capacity that he was taken as a servant to the French king Henry II at the age of 10. He married an ordinary woman who was not susceptible to hypertrichosis, but of the seven children born in this marriage, four also inherited the disease.
- Their portraits were painted by artists of that time; the best minds of France and Italy argued about the causes of the disease and its heredity.
- Another family is the Aceves, who currently live in Mexico. The most famous is Jesus, commonly called Chewie. He, like his sister and cousins, were born with hypertrichosis. Chui himself became the father of three daughters, who were born to different women, and at the same time, all the girls inherited their father's disease.
Family
- This chain began with Jesus’ great-grandmother, and during this time, 30 members of the Aceves family were born and live with a similar diagnosis. A film was made about the family - and this is not surprising, because out of 50 people who have had hypertrichosis recorded in the entire history of registration of cases of the disease, 30 are members of the Aceves family.
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