Hypertrichosis symptoms

Home   ›   Hypertrichosis treatment   ›   Hypertrichosis symptoms.
The primary symptom of hypertrichosis is excessive growth of hair. There are several forms of hypertrichosis with characteristic symptoms. Hypertrichosis may be present at birth or acquired at a later stage of life.

Hypertrichosis may be localized to a few spots or extensively cover a major portion of body. Hypertrichosis may involve one of the three types of hairs, viz., terminal, vellus and lanugo hairs. The symptoms are specific to each form of excessive growth of hair.

Hypertrichosis symptoms

Congenital hypertrichosis (CH) forms are present at birth and are caused due genetic mutations. CH may be hereditary, passed on by the parents. There are instances wherein spontaneous mutation during embryonic growth causes the excessive growth. The parents and family members may be symptom-free.

Congenital hypertrichosis lanuginosa

CH lanuginosa is present at the time of birth. The characteristic symptom is the presence of lanugo hair. The newborn is covered with excessive growth of hair. In normal circumstances, the lanugo is shed by the fetus at about eighth month of pregnancy to be gradually replaced by vellus hair. The persisting lanugo hair may somewhat get thinner as the child grows.

Generalized congenital hypertrichosis

In generalized CH, the affected patient present excessive hair growth all over the body. Only the palms, soles and mucous membranes are spared.

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Congenital hypertrichosis universalis is a type of generalized CH. It is a rare familial disorder. The hairiness increased progressively. The patient's face, trunk and limbs get covered with long hair. In one case the patient's father and paternal grandfather were also noted to have the typical symptoms.

Ambras syndrome

Ambras syndrome is a type of generalized CH. The typical symptom of Ambras syndrome are silky, long, light-colored hair, prominent on face, ears and shoulders. The vellus hair uniformly covers the eyelids, nose, cheeks, ears and shoulder. Only the palms, soles and mucous membranes are spared.

In Congenital hypertrichosis universalis the differentiating symptoms from Ambras syndrome are the presence of dense hair on the nose, preauricular regions, external ears and external auditory canal. In congenital terminal hypertrichosis also, the entire body is covered with hair. The differentiating symptoms are the growth of terminal type of hair as well as the occurrence of gingival hyperplasia. The patients appear like "werewolf".

Circumscribed (localized) congenital hypertrichosis

Circumscribed CH is the characteristic symptoms of congenital melanocytic nevus, congenital Becker nevus, Hypertrichosis cubiti, smooth muscle hamartoma and nevoid hypertrichosis. Congenital melanocytic nevus is found in some newborns. The melanin filled lesions are quite often have thick black terminal hair growth. Becker's nevus is present at birth in some cases and is characterized by an overgrowth of the epidermis, melanocytes and hair follicles.

Congenital smooth muscle hamartoma is due to bundling of smooth muscles within the dermis with associated excessive growth of vellus hair at the lesion. Nevoid Hypertrichosis is a solitary patch of terminal hair. In most cases it is symptom-free. If the patch of excess hair is present in lumbosacral area it has to be diagnosed to rule out faun tail deformity and associated spina bifida occulta and diastematomyelia disorders.

In hairy elbow syndrome, both the elbows of the patient have long vellus hair. The condition may be associated with physical abnormalities such as dysmorphic facial features, microcephaly, joint hyperlaxity and mental retardation. In rare cases patients may have congenital, symmetric patches of vellus hypertrichosis on the palms or soles of the feet. Anterior cervical hypertrichosis is an autosomal recessive or X-linked chromosomal mutation with characteristic symptom of excessive hair growth on the neck.

Acquired hypertrichosis (AH) appears after birth. It is associated with side effects of drugs, porphyria cutanea tarda, cancers and eating disorders (anorexia nervosa). Some of the culprit medications are, iodine, psoralens, acetazolamide, minoxidil, phenytoin and cyclosporin. AH lanuginosa is characterized by rapid growth of lanugo hair. Generalized AH usually affects the cheeks, upper lip, and chin. Oral minoxidil treatments for hypertension is known to cause this condition. One of the symptoms of internal malignancy is the appearance of patterned hypertrichosis.

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Reference: 1.Trüeb RM. Causes and management of hypertrichosis. Am J Clin Dermatol. 2002;3(9):617-27. 2.Goel N, Rajaram S, Gupta B, Gupta K. Familial congenital generalized hypertrichosis. Indian J Dermatol Venereol Leprol 2013;79:849.

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Jan 13, 2015

Congenital hypertrichosis

Home   ›   What is hypertrichosis   ›   Congenital hypertrichosis.
What is congenital hypertrichosis?
Hypertrichosis is the excessive growth of hair over the body. Congenital hypertrichosis (CH) is the presence of excessive hair growth at birth.

Congenital hypertrichosis may be present as an inherited genetic disorder. Congenital hypertrichosis may also occur as a result of spontaneous mutation during fetal growth. CH may concur and coexist with many hereditary medical conditions.

Types of congenital hypertrichosis

The excessive growth of hair is classified as congenital hypotrichosis when it is present at birth. Acquired hypotrichosis is the appearance of excessive hair growth later in the life. CH may involve terminal, Vellus or lanugo hair. CH may be generalised covering the entire body or localized and circumscribed. Some of the many forms of congenital hypertrichosis are discussed below.

Generalized congenital hypertrichosis (GCH)

Various types of genetic inheritance and spontaneous mutation can cause GCH. The entire body may be covered sparing, mucous membranes, palms and soles. The excessive growth of hair may involve either terminal hair or lanugo hair.

Congenital hypertrichosis lanuginosa (CHL)

In congenital hypertrichosis lanuginosa, the entire body of the newborn is covered by the lanugo hair.

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Only the mucous membranes, palms, soles, dorsal terminal phalanges, labia minora, prepuce, and glans penis are spared. The CHL may occur as an autosomal dominant inheritance or as a sporadic mutation. It is believed that mutations of gene in 8q22 of chromosome 8 is the cause.

Ambras Syndrome

Ambras Syndrome is an extremely rare type of congenital hypertrichosis lanuginosa, characterized by the vellus-type hair covering the entire body. Abnormalities like dental anomalies, triangular, coarse face, bulbous nasal tip, delayed tooth eruption, absence of teeth, glaucoma, pyloric stenosis, photophobia are associated with Ambras syndrome.

X-linked generalized congenital hypertrichosis (X-GCH)

X-GCH occurs very rarely. The excessive terminal hair growth was mapped to chromosome Xq24-q27.1 in a Mexican family. Terminal hair covers the face, trunk and limbs, sparing mucosa, soles and palms. It may be associated with medical conditions such as, gingival hyperplasia, scoliosis, and spina bifida.

Cornelia de Lange Syndrome (CDLS)

CDLS is a genetic disorder present from birth. The disorder can occur due to mutation in genes NIPBL, SMC1A or SMC3. The disorder is characterized by thick and convergent eyebrows (synophrys), thick and long eyelashes, and low hairline. Vellus hair hypertrichosis of trunk, posterior neck, sacrum and elbows is seen. It may be associated with abnormalities such as cutis marmorata, upturned nostrils, depressed nasal bridge, low set ears, small and irregular teeth, high palate and bifid uvula. The patients may have short and abnormal arms, hands and feet. They may suffer from severe mental retardation.

Localized congenital hypertrichosis

Congenital localized hypertrichosis is a notable feature of congenital melanocytic nevus, congenital Becker nevus, Hypertrichosis cubiti, smooth muscle hamartoma and nevoid hypertrichosis. The localized abnormal hair growth may be at a single site or just a few sites.

Congenital melanocytic nevus (CMN)

Congenital melanocytic nevus (CMN) is a type of melanocytic nevus found in newborns. CMN is usually larger than the acquired melanocytic nevus. The lesion may be covered by excessive growth of terminal hair. There are three types of CMN. Small-sized congenital nevocytic nevus has a diameter less than 2 cm. Medium-sized CMN has a diameter more than 2 cm but less than 20 cm. Nevus pigmentosus et pilosus (giant nevus) is more than 20 cm in diameter with dark pigment and terminal hypertrichosis.

In about 2% to 45% of patients with giant melanocytic nevi at birth, neurocutaneous melanosis is involved. Neurocutaneous melanosis (NCM) is characterized by melanocytic nevi on the skin and melanocytic tumors in the leptomeninges of the central nervous system. Nearly half of the patients with neurocutaneous melanosis may develop malignancy in the form of leptomeningeal melanoma. Early embryonic, postzygotic somatic mutations in the NRAS gene are implicated in the pathogenesis of NCM.

Hypertrichosis cubiti

Hypertrichosis cubiti, also known as hairy elbows syndrome, is a rare symmetric, localized, congenital, circumscribed hypertrichosis with long vellus hair occurring on the elbow (extensor surfaces). Both familial and sporadic forms have been reported. Partial or complete resolution during puberty is reported. A very high percentage of hair in the elbow appear to be in the anagen phase.

The hairy elbows syndrome may in some cases have associated abnormalities. Short stature or other physical abnormalities such as dysmorphic facial features, microcephaly, joint hyperlaxity, thin-long-webbed neck and mental retardation have been associated with hypertrichosis cubiti. Autosomal dominant as well as autosomal recessive inheritance is postulated in the pathogenesis.

Congenital smooth muscle hamartoma

Smooth muscle hamartomas are caused by the benign proliferation of smooth muscle bundle within the dermis. It is an uncommon malformation of the pilar smooth muscle, often involving the back and lower limbs. These lesions typically appear as a skin colored or lightly pigmented patch or plaque with hypertrichosis of vellus hair.

Becker's nevus

Becker's nevus is due to an overgrowth of the epidermis, melanocytes and hair follicles. Though most often it is seen as an acquired disorder with terminal hypertrichosis, it may also be present at birth. The pathophysiology of Becker's nevus remains unclear. A case was reported wherein the disorder had genetic association. A 16 month boy had a lesion on his right shoulder and a similar lesion existed on the right shoulder of his father.

Nevoid Hypertrichosis

Nevoid Hypertrichosis usually appears as a solitary patch of terminal hair without other abnormalities anywhere in the body. If present in lumbosacral area, differential diagnosis is required to rule out faun tail deformity conditions like, spina bifida occulta and diastematomyelia.

Lumbosacral hypertrichosis

Lumbosacral hypertrichosis is also known as faun tail deformity. This malformation is associated with cutaneous anomalies such as sacral dimple, lipoma, port-wine stain, hypertrichosis or dermoid cyst. It is also associated with bone and spinal cord defects such as spina bifida occulta, diastematomyelia, myelomeningocele, and vertebral abnormalities.

Hairy palms and soles

Hairy palms and soles is a bilaterally symmetric, hereditary hypertrichosis affecting the palms and soles. It is an autosomal dominant inheritance. The hair follicles are normal and the children and women have vellus hair. The excessive hair growth is androgen-sensitive and in boys the vellus hair becomes terminal at puberty.

Anterior cervical hypertrichosis

The cause of this congenital disorder can be autosomal dominant, autosomal recessive or X-linked chromosomal mutations. Excessive hair growth is observed on the neck of the affected individuals. In the autosomal recessive mutation, peripheral neuropathy is observed.

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Reference: 1.Trüeb RM. Causes and management of hypertrichosis. Am J Clin Dermatol. 2002;3(9):617-27. 2.Goel N, Rajaram S, Gupta B, Gupta K. Familial congenital generalized hypertrichosis. Indian J Dermatol Venereol Leprol 2013;79:849.

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Current topic in natural skin care: Congenital hypertrichosis.

Dec 26, 2014

Hypertrichosis causes

Home   ›   What is hypertrichosis   ›   Hypertrichosis causes.
Hypertrichosis, excessive growth of hair, is caused by several factors. Hypertrichosis may be congenital, being present at birth. It may also be acquired later in the life.

Hypertrichosis presence at birth is caused by congenital syndromes, autosomal mutations and hereditary diseases. Acquired hypertrichosis is induced by malignancies, endocrine anomalies and certain therapeutic medications.

The excess hair growth may be generalized, affecting the whole of the body or localized to form well defined patches. Hypertrichosis may involve all the three types of hairs; vellus hair, lanugo hair or terminal hair. The various types of excess hair growth and their causes are discussed below.

Common causes of hypertrichosis

Common health conditions like hormonal, endocrine disturbances, systemic illness and certain diseases can induce excessive growth of hair.
Some of the common factors inducing acquired hypertrichosis are:

• hypothyroidism,
• hyperthyroidism,
• anorexia nervosa,
• porphyria cutanea tarda,
• pituitary abnormalities,
• hypothalamic abnormalities,
• HIV/AIDS,
• Becker’s nevus,
• cancers,
• head injury,
• injury,
• friction and
• inflammation.

Congenital hypertrichosis (CH)

Congenital syndromes, autosomal mutations and hereditary diseases cause CH.

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• Congenital erythropoietic porphyria (Gunther disease) is a rare, autosomal recessive metabolic disorder affecting heme, caused by deficiency of the enzyme uroporphyrinogen cosynthetase. Exposure to long-wave ultraviolet light causes vesiculobullous lesions, hyperpigmentation and excessive growth of hair.
• CH lanuginosa is considered to be due to autosomal dominant mutation on chromosome 8q.
• Generalized CH is considered to be due to autosomal dominant mutation on chromosome x24-q27.1.
• CH generalized terminalis is due to a mutation in MAP2K6 on chromosome 17.
• Localized CH may be associated with nevus, especially melanocytic naevus.
• Abnormal localized lumbosacral hypertrichosis or faun-tail nevus is usually a marker for spinal dysraphism like spina bifida occulta or diastematomyelia.

Drug induced hypertrichosis

Many medications have been found to induce excessive hair growth. Some of the medications and chemicals inducing excessive hair growth are listed below:

• Topical minoxidil,
• Glucocorticoids,
• Anticonvulsants,
• Cyclosporine,
• Diazoxide,
• Psoralen,
• Streptomycin,
• Latanoprost,
• Acetazolamide and
• Phenytoin.

Treatment options

There is no treatment for congenital forms of excessive hair growth. Acquired forms resolve when the factors responsible excessive hair growth are removed or discontinued. Hair removal methods like shaving, waxing, laser hair removal and electrolysis are the treatment options available for congenital excessive hair growth.

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Reference: 1.Trüeb RM. Causes and management of hypertrichosis. Am J Clin Dermatol. 2002;3(9):617-27. 2.Goel N, Rajaram S, Gupta B, Gupta K. Familial congenital generalized hypertrichosis. Indian J Dermatol Venereol Leprol 2013;79:849.

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Dec 21, 2014

Hypertrichosis treatment - Hypertrichosis management

Home › Hypertrichosis definition › Hypotrichosis treatment › Hypertrichosis treatment - Hypertrichosis management.

Hypertrichosis is excessive growth of hair on any part of the body. Hypertrichosis excludes androgen-induced excessive hair growth, known as hirsutism. Acquired hypertrichosis can be treated by removing the underlying cause.

For congenital hypertrichosis there is no cure. Hair removal treatments are the best options for improving the appearance. In patients with localized hypertrichosis, satisfactory results can be realized with the treatment. In extensive and generalized forms, hair removal treatment is time consuming and likely to be unsatisfactory.

In acquired form of hypertrichosis cases, the treatment of the underlying causative factor can normalize the hair growth. However alleviating or completely resolving the medical cause may take considerable time. Till such time temporary cosmetic treatment to remove the unwanted hair is necessary for addressing the associated emotional problems of the patient. However it is to be noted that, in some cases the hair removal methods may cause, dermatitis, hives or scarring.

Hypertrichosis treatment and management

Extensive hypertrichosis may cause cosmetic embarrassment and is psychologically devastating to the patient. Generalized hypertrichosis may involve all three types of hairs, namely, lanugo, vellus and terminal hairs. In localized hypertrichosis, the local vellus hairs transform into terminal hairs.

Temporary hair removal for hypertrichosis

There are many methods of temporary removal of hair. In these methods as the hair follicle is not destroyed, new strands grow after some time. Skin sensitivity, hives, scarring, ingrown hair (pseudofolliculitis), folliculitis, dermatitis, hyperpigmentation and hypopigmentation are the possible problems associated with these methods. It is cumbersome and time consuming for treating large areas of hypertrichosis.

Superficial removal

Abrasives like pumice stones are rubbed on the skin to break and remove hair. Quite often this method damages the skin. Shaving cuts and removes the hair at the skin level. The hair may become apparent after two or three days of growth. Chemical depilatories are available in gel and cream forms. They contain calcium thioglycolate or potassium thioglycolate.

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These chemicals react with the cystine and breaks down the disulfide bonds in keratin. The weakened hair can be just scraped off. In all these superficial methods removal of hypertrichosis condition is highly temporary and chemicals may sensitize the skin.

Depilation

In epilation, hair is completely removed from the hair shaft. It is quite an effective method for temporary removal of hypertrichosis. Each hair may be pulled out individually with a tweezer. By threading also a small group of hairs can be removed at a time. Waxing treatment is done to remove large numbers in one go. As an alternative to waxing, sugaring used. Sugar and lemon juice are heated in water and made into paste. The paste is applied to the skin and a cotton strip is placed over it. The cotton strip is pulled back sharply to pull out the strands stuck on it from the follicle.

Hair growth inhibition in hypertrichosis

Eflornithine cream (Vaniqa cream 13.9%) is successfully used for reducing the unwanted hair. Eflornithine interferes with ornithine decarboxylase. Ornithine decarboxylase is needed in the follicles for hair growth. Eflornithine irreversibly binds to Ornithine decarboxylase and prevents the natural substrate ornithine from accessing the active site of the enzyme. The treatment effects become apparent after 4-8 weeks of application. However hypertrichosis condition returns in eight weeks when the application is stopped.

Permanent hair removal for hypertrichosis

For permanent reduction in hair use of electrolysis and laser technology are the best solutions. Skilled persons are required for carrying out these procedures. Many sittings may be required to reduce hair growth and get satisfactory results. The results of hypertrichosis treatment depend upon the skill of the technician and and use of correct intensity and duration of current.

Electrolysis for hypertrichosis

Electrolysis is aimed to destroy the hair follicle and bulb for successful hair removal. A fine needle is inserted into the hair follicle and a weak electrical current is applied to destroy bulb. There are two types of electrolysis. In galvanic electrolysis direct current is used to destroy the hair bulb. In thermolysis a high frequency alternating current is used to produce heat and cauterize the hair bulb. Hypertrichosis treatments are also offered combining the two procedures.

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Each electrolysis session may extend up to 30 minutes and a number of sittings may be required. Post inflammatory hypopigmentation and hyperpigmentation are common with electrolysis treatment for hypertrichosis. There may be erythema, folliculitis and angioedema at the site of electrolysis. Up to 50% regrowth may be observed after the procedure requiring further sessions.

Laser-assisted hair reduction in hypertrichosis

In laser hair removal, exposure to pulses of laser light destroys the hair follicle by selective photothermolysis. The Food and Drug Administration (FDA) has approved hair removal lasers for "permanent hair reduction". The laser procedure is effective on pigmented terminal hair. Hypertrichosis with lanugo or vellus hairs may not respond to the treatment. In laser-assisted hair reduction side effects like erythema and perifollicular edema are common. In some cases there may be crusting, blistering, hyperpigmentation and hypopigmentation. These side effects are mostly temporary and resolve with skin care measures.

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Reference:
1.Trueb RM. Causes and management of hypertrichosis. Am J Clin Dermatol. 2002;3(9):617-27.
2.Trueb RM. Hypertrichosis. Hautarzt. 2008 Apr;59(4):325-37; quiz 338.
3.Goel N, Rajaram S, Gupta B, Gupta K. Familial congenital generalized hypertrichosis. Indian J Dermatol Venereol Leprol 2013;79:849.

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Current topic in dynamic skin care: Hypertrichosis treatment.

What is hypertrichosis - Hypertrichosis definition

Home › Congenital hypotrichosis › What is hypertrichosis - Definition of hypertrichosis.

What is hypertrichosis?

Hypertrichosis is defined as excessive growth of hair. Hypertrichosis disorders may be present at birth, or may be acquired later in life.

The excessive hair disorders present at birth include congenital syndromes, autosomal mutations and hereditary diseases. The mutation may be spontaneous genetic mutation in autosomal chromosomes or in x-y chromosomes. These excessive hair disorders may also be acquired later in life, triggered by cancers, hormonal anomalies and certain therapeutic drugs. The excess hair growth may be generalized, affecting the whole of the body or localized to form well defined patches.

Extensive excess growth of hair is also known as Ambras syndrome or werewolf syndrome. These disorders affect both the genders equally. Hypertrichosis is basically different from hirsutism in that it is androgen-independent. Hirsutism is the excessive male-pattern hair growth in women. Hirsutism is an androgen-dependent condition. The excessive growth may be of vellus hair, lanugo hair or terminal hair, depending upon the type of syndrome. Generally the palms of the hands, soles of the feet and mucosal surfaces are not affected.

Web definition of hypertrichosis

Definition by merriam-webster.com: "excessive growth of hair."

Definition by reference.md: "Excessive hair growth at inappropriate locations, such as on the extremities, the head, and the back. It is caused by genetic or acquired factors, and is an androgen-independent process."

Definition by thefreedictionary.com: "An extremely rare (less 100 cases reported worldwide) excess of hair on the body, which can be generalised or localised, congenital or acquired."

What are the types of hypertrichosis

Considering the time of appearance, excessive hair growth can be categorized into congenital (present at birth) or acquired (acquired later in life) types. Further these disorders, depending upon the extent, may categorized as generalized or localized types.

Congenital hypertrichosis (CH)
Considering the type of hair (vellus, lanugo or terminal) and extent of excess hair, CH can again be categorized into subtypes.

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CH lanuginosa patient has generalized cover of lanugo hair over the body. It is considered to be an autosomal dominant mutation on chromosome 8q.

Generalized CH is noticed in men as excessive hair on the face and upper body, whereas women have less severe asymmetrical hair distribution. It is considered to be an autosomal dominant mutation on chromosome x24-q27.1.

Terminal CH is characterized by whole body being covered by terminal hair. It is considered to be due to a mutation in MAP2K6 on chromosome 17. Further, circumscribed, localized and nevoid forms of CH have been reported.

Acquired hypertrichosis (AH)

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AH is associated with side effects of medication, presence of cancers and alopecia and glaucoma treatment. AH lanuginosa is characterized by growth of lanugo hair, especially on the face. In some cases it is associated with malignancy. Generalized AH is usually associated with minoxidil treatments. Patterned AH shows hair growth in a pattern formation and is associated with malignancy. Localized AH may appear as well defined excess hair when there is irritation or trauma to the skin.

What are the causes of hypertrichosis

Acquired hypertrichosis is caused by the presence of cancer, alopecia medication, hypertension medication, metabolic disorders or hormone imbalances.

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Topical applications like iodine, psoralens, topical minoxidil or topical steroids may also cause acquired hypertrichosis. Congenital hypertrichosis is caused by inherited genetic abnormalities or spontaneous genetic mutations in autosomal or x-y chromosomes.

What are the hypertrichosis treatment options

There is no treatment for congenital forms of hypertrichosis. Acquired forms resolve when the causative factors are removed or discontinued. Hair removal methods like shaving, waxing, laser hair removal and electrolysis are the treatment options available for congenital hypertrichosis.

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Reference: 1.Trüeb RM. Causes and management of hypertrichosis. Am J Clin Dermatol. 2002;3(9):617-27. 2.Trüeb RM. Hypertrichosis. Hautarzt. 2008 Apr;59(4):325-37; quiz 338. 3.Goel N, Rajaram S, Gupta B, Gupta K. Familial congenital generalized hypertrichosis. Indian J Dermatol Venereol Leprol 2013;79:849.

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