Hypertrichosis - how is it different from hirsutism, and how to deal with it?


Table of contents

  1. Etiology and pathogenesis
  2. Clinical manifestations
  3. Principles of treatment
  4. Cosmetic procedures

Hypertrichosis (hairiness, excess hair growth, Ambras syndrome) is the growth of vellus and/or terminal hair on any area of ​​the body in an amount greater than normal for people of a given age, race and gender. Hypertrichosis is not excess hair growth under the influence of androgens, as it is called hirsutism .

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Hypertrichosis is classified:

  • by age of appearance - congenital and acquired;
  • according to the degree of distribution - generalized and focal;
  • in the affected area - hypertrichosis of the auricle, elbows, anterior or posterior surface of the neck, lumbosacral spine, etc.

Hypertrichosis can be either an isolated finding (without an obvious cause) or associated with other congenital or acquired pathologies.

Seeing a doctor

Treatment of hypertrichosis is carried out by a dermatologist. Contacting a specialist is justified if abnormal hair growth persists during the first weeks of the baby’s life. After the baby is born, the medical team conducts an initial examination, which shows the absence or presence of a connection between hypertrichosis and other diseases.

In the absence of congenital pathologies, abnormal hair growth in certain areas of the skin is treated for aesthetic reasons: with age, a child may develop complexes in the perception of his own body. Parents should take the therapy as responsibly as possible. Self-treatment or removal of abnormal hair using tweezers and razors can lead to complications. Thus, a damaged nevus degenerates into a malignant formation.

The combination of hypertrichosis with other developmental pathologies requires treatment of the underlying disease. After the child’s hormonal levels are equalized, the atypical hairline is subjected to standard treatment under the supervision of a dermatologist. Our clinic carries out a full cycle of diagnostic and therapeutic procedures for hypertrichosis in children. The pediatric department is equipped with all the necessary equipment, with the help of which dermatologists completely remove abnormally growing hair from a child’s skin.

Etiology and pathogenesis

A. Congenital hypertrichosis

  1. Congenital generalized hypertrichosis:
  • Congenital universal hypertrichosis is a rare autosomal dominant disease first described in the 16th century. Its cause is considered to be a mutation (pericentric inversion) of chromosome 8 - inv(8)(p11.2q22). Some researchers suggest X-linked inheritance of the disease, because in one Mexican family, the diseased father passed on the mutant gene to all four daughters, but to none of the nine sons.
  • Prepubertal hypertrichosis - In 1988, scientists described clinically healthy children with generalized hypertrichosis that gradually worsened from birth. This condition is common in people from Mediterranean and South Asian regions and is sometimes called "racial hirsutism." Although, unlike true hirsutism, prepubertal hypertrichosis is not associated with the action of androgens.
  • Congenital vellus hypertrichosis is a disease of unknown etiology and pathogenesis. An autosomal dominant mode of transmission has been suggested, but some cases of the disease do not support this. Any genetic pathology leading to the development of congenital vellus hypertrichosis has not been established; There are no endocrine changes in the body.
  1. Congenital focal hypertrichosis - occurs against the background of other congenital pathologies: congenital melanocytic nevus, Becker's nevus, spinal dysraphism (incomplete closure of the spinal canal of the spine), etc.

B. Acquired hypertrichosis

  1. Acquired generalized hypertrichosis:
  • Drug-induced hypertrichosis - some drugs can cause excess hair growth throughout the body (phenytoin, cyclosporine A, minoxidil, etc.).
  • Secondary hypertrichosis develops against the background of various acquired diseases: hepatic porphyria, juvenile dermatomyositis, HIV infection (AIDS), celiac disease, etc.
  • Acquired vellus hypertrichosis occurs against the background of malignant pathology. Most often this is rectal cancer, somewhat less often - cancer of the gallbladder, pancreas, bladder, uterus, ovaries, breast, bronchi, as well as lymphoma and leukemia.
  1. Acquired focal hypertrichosis - develops against the background of repeated friction or pressure in a certain area of ​​the body, as well as a chronic inflammatory process. Focal hypertrichosis is common, although it is poorly described in the literature. This is due to the fact that patients usually do not seek help because local hair growth does not bother them or is easily removed by shaving/depilation.

Causes of hypertrichosis

Pathology can occur due to various reasons:

  • congenital mutation (due to abnormal pregnancy, infection suffered by the mother in the first trimester);
  • due to taking a number of medications;
  • due to disruption of the endocrine system.

The so-called “symptomatic hypertrichosis” is also distinguished. It may accompany certain diseases and conditions (exhaustion, anorexia nervosa, alcohol syndrome, traumatic brain injury, porphyria, etc.).

Symptoms of local hypertrichosis can be observed at the site of skin injury (eg, burn).

In addition, studies show that pathology may appear due to the awakening of hair follicles caused by various cancers.

Clinical manifestations

A. Congenital hypertrichosis

Universal congenital hypertrichosis

The entire body of such patients is covered with a continuous volume of long vellus hair, with the exception of those areas where hair usually does not grow (palms, soles). Characterized by increased hypertrichosis on the face, ears and shoulders ( Fig. 1 ). The length of hair on the forehead, eyelids, nose, cheeks and preauricular area can reach several tens of centimeters. If they are not cut, hair begins to stick out even from the external auditory canal.

In ancient times, rich courtiers found such people and exhibited them for the amusement of the public. One of these men was named Petrus Gonsalvus, and his portrait is in the Austrian castle of Ambras, near Innsbruck ( Fig. 2 ). Therefore, universal congenital hypertrichosis is also called Ambras syndrome .

Prepubertal hypertrichosis

Patients from a young age experience hair growth on the temples spreading to the forehead. Very thick eyebrows and pronounced hair growth on the upper back and proximal arms and legs are noted. Prepubertal hypertrichosis is distinguished from other congenital pathologies by the presence of dark hair of the terminal type.

Congenital vellus hypertrichosis

Immediately after birth, in such patients, the entire body, except for the arms and legs, is evenly covered with thin blond hair. During the first year of life, they begin to fall out, and gradually this process spreads to the arms and legs.

Congenital focal hypertrichosis

They manifest as small foci of excess hair growth in various parts of the body, depending on the location of another congenital pathology. For example, in Becker's nevus, coarse black hairs typically appear within the lesion and may not completely coincide with the pigmented area ( Figure 3 ).

B. Acquired hypertrichosis

Drug-induced hypertrichosis ( Fig. 4 ):

  • Phenytoin (anti-epileptic drug) - may cause hypertrichosis, acne and gingival hyperplasia after 3 months of use. The side effect is more common in girls and mainly affects the extensor side of the limbs.
  • Cyclosporine A (immunosuppressant) - hypertrichosis occurs in 60% of patients within 6 months of therapy. Additionally, hyperplasia of the sebaceous glands (10% of patients) and acne (15% of patients) develop.
  • Minoxidil (antihypertensive drug) - hypertrichosis most often occurs with oral administration, but can also occur with topical treatment. The face and limbs are mainly affected.

Secondary hypertrichosis:

  • Hepatic porphyria - In 1960, there was an epidemic of hepatic porphyria in Turkey after many children were poisoned with the insecticide hexachlorobenzene. Moreover, the victims had a characteristic appearance, for which they were called “children of monkeys.”
  • Lack of nutrients - hypertrichosis of the trunk and limbs has been recorded in young women who adhered to strict diets or suffered from anorexia nervosa. Excessive hair growth is also possible in children with celiac disease.
  • Juvenile dermatomyositis - most often in such patients local hypertrichosis is observed, but it can also be widespread, affecting the face, limbs and trunk.
  • HIV infection and AIDS - in addition to acquired trichomegaly of the eyelashes, patients with AIDS have a common form of hypertrichosis. Its pathogenesis remains unclear and appears to be related to chronic malnutrition, metabolic changes (porphyria cutanea tarda), immunological dysregulation (increased serum interferon α), and medications.

Acquired vellus hypertrichosis

Patients with malignant neoplasms may experience excess growth of light vellus hair on the face. From the onset of hypertrichosis to detection of a tumor usually takes from several weeks to 2 years.

Rice . 1. Congenital hypertrichosis on the back (Danish national service on dermato-venereology)

https://www.danderm-pdv.is.kkh.dk/atlas/4-3.html

Rice. 2. Portrait of Petrus Gonsalvus, who suffered from congenital hypertrichosis (Wikimedia Commons)

Rice. 3. Hypertrichosis in Becker’s nevus - a noticeable discrepancy between the boundaries of hair growth and the neoplasm itself (Danish national service on dermato-venereology)

https://www.danderm-pdv.is.kkh.dk/atlas/7-152-3.html

Rice. 4. Acquired focal hypertrichosis due to topical use of a potent corticosteroid (Danish national service on dermato-venereology)

https://www.danderm-pdv.is.kkh.dk/atlas/4-8.html

Hair types with hypertrichosis

In hypertrichosis, excessive hair growth can be caused by any hair type, including lanugo, vellus, or terminal hair types.

  • Lanugo is a fine, fluffy hair seen in newborns and usually falls out within the first year of life. Tufted hair persists during childhood and increases in density after puberty.
  • The hairs of the vellus are also fine, but thicker than those of the lanugo, and are usually shorter and finer than the terminal ones.
  • Terminal hairs are thick hairs that are found in the pubic area and axillae (armpits) of both men and women. Terminal hair can also appear on the mustache or beard area, chest, legs, arms, and sometimes on the back in men.

Vellus is thin, “vellus” hair, it is short, weakly pigmented, and may have a deformed structure - “corrugated”

Principles of treatment

Treatment of hypertrichosis is based on searching for its causes and the possibilities of eliminating them. For example, early detection of a malignant tumor can solve the problem of excess hair growth. The same applies to some medications - stopping them, for the most part, helps get rid of excess hair without additional interventions. If it is impossible to identify/eliminate the cause of hypertrichosis or if the patient wishes to quickly get rid of unwanted hair, cosmetic, medicinal, hardware and other methods can be used.

Cosmetic procedures

Various methods are suitable for hair removal - shaving, plucking, waxing, chemical depilatory agents, electric and laser devices, as well as intense pulsed light (IPL).

Shaving is the simplest method, but also the shortest in terms of duration of action - hairs begin to grow back literally the next day. After plucking, they appear on average after 2 weeks, after waxing - after 2–6 weeks. Chemical peeling disrupts the protective properties of the skin, so it is only suitable for small areas of the body. The duration of its effect is about 1 week.

If the hair does not bother the patient too much, it can not be removed at all, but bleached.

Drug therapy

Unlike hirsutism, the pathogenesis of hypertrichosis does not involve the influence of androgens. Therefore, antiandrogen treatment for hypertrichosis will be ineffective. A promising method is the topical use of eflornithine, an inhibitor of the enzyme ornithine decarboxylase, which is involved in hair growth.

Hardware techniques

The most popular methods of hair removal for hypertrichosis are laser and photoepilation. The principle of operation of these methods is selective heating of the hair when it absorbs an intense light flux, followed by destruction of the hair follicle. For photoepilation, IPL devices are used; unlike laser procedures, IPL procedures are more affordable, but usually require a longer time for each procedure. Hair removal lasers are the most convenient and effective tool today for removing unwanted hair due to hypertrichosis. Among lasers, diode lasers of 800-810 nm are most widely used, since they are universal for the treatment of hypertrichosis in patients with any phototype.

Questions from our users:

  • eyelash hypertrichosis
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  • hypertrichosis causes congenital
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