Approach to inherited hypertrichosis: A brief review.

Hypertrichosis refers to the growth of hair, of an excessive amount and thickness, on any part of the body. It must be distinguished from hirsutism which is characterized by excess growth of hair in androgen-dependent areas on the upper lip, chin, chest, linea alba, thigh and axilla. Hypertrichosis may be localized or generalized, and congenital or acquired. Excess hair growth has a psychological impact on the child as well as the parents due to the cosmetic disfigurement it produces. Current treatment options are limited and not wholly satisfactory. Treatment should be customized according to the area, nature and amount of hair growth, age of the patient and personal preferences. In addition, when hypertrichosis occurs as a component of a syndrome, multidisciplinary management is required to address the associated systemic features. A detailed review of inherited generalized hypertrichosis is presented here with emphasis on clinical clues to identifying complex syndromes with multisystem involvement.

Congenital generalized hypertrichosis terminalis: a proposed classification and a plea to avoid the ambiguous term "Ambras syndrome".

Congenital generalized hypertrichosis terminalis (CGHT) is a heterogenous group of diseases with continuing excessive growth of terminal hair. "Ambras syndrome" was first coined by Baumeister in 1993 to describe a case of nonsyndromic CGHT which was erroneously analogized to the portrait paintings of Petrus Gonzales and his children, exhibited in Ambras Castle near Innsbruck, Austria. This family probably, a syndromic type with abnormal dentition, inherited as an autosomal dominant trait. CGHT associated with gingival hyperplasia is probably a particular entity typified by the historical cases of Julia Pastrana and her son. An X-linked type of CGHT has likewise been categorized as "Ambras syndrome". Moreover, some reports have mistakenly classified "Ambras syndrome" as an example of hypertrichosis lanuginosa. Potential gene loci identified so far may include 8q22, 17q24.2-q24.3 and Xq24-q27.1. The designation "Ambras syndrome" has thus been applied to various types of congenital hypertrichosis that differ to such degree that the name "Ambras" has no specific meaning, neither in the past nor in the future. Hence, this misleading term should now be jettisoned.

Alopecia in genetic diseases.

Congenital abnormalities of the hair shaft are conditions in most cases linked to chemical, biochemical, and morphological alterations, genetically determined. These alterations may be associated with a larger array of symptoms, as mentioned above, or may occur isolated. The number of genes involved and their penetration are responsible for the mode of transmission, severity, and phenotypic expression of the disease. Furthermore, in some cases, these abnormalities can be considered true pathognomonic markers of disease. Within this disorder, two groups are classically identified: with increased fragility of the hair shaft and without increased fragility. Congenital abnormalities of the hair shaft that present an increased fragility of hair include: Trichorrhexis nodosa congenita, Trichoschisis, Trichorrhexis invaginata, Trichothiodystrophy, Trichoclasia, Pili torti, Monilethrix and Loose anagen syndrome; while the alterations that are not associated with a greater fragility of the hair are: Pili annulati, Longitudinal grooves, Uncombable hair syndrome (Pili trianguli et canaliculi), Woolly hair, Woolly hair with dominant inheritance, Familial recessive woolly hair and Woolly hair nevus.

Hipertricosis cervical anterior esporádica / Sporadic Anterior Cervical Hypertrichosis

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Réplica / Response

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[Hypertrichosis]. / Hypertrichose.

Hypertrichosis denotes growth of hair on any part of the body in excess of the amount usually present in persons of the same age, race, and sex, excluding androgen-dependent hair growth. Hypertrichosis may be an isolated finding or associated with a syndrome, be associated with additional congenital anomalies or a marker for systemic disease. In order to diagnose it accurately, the age of onset, type, localization and pattern of hair growth, associated disorders, medications and perhaps associated anomalies and family history should be considered. Even though hypertrichosis usually has limited medical significance, it often causes cosmetic embarrassment, often resulting in a significant emotional burden. Treatment options are available, though limited in terms of efficacy and patient satisfaction. No single method of hair removal is appropriate for all body locations and patients, and the one adopted will depend on the type, area, and amount of excessive hair growth, as well as on the age, sex, and personal preference of the patient. Patients with hypertrichosis should be adequately advised of the treatment modalities. These include cosmetic procedures (bleaching, trimming, shaving, plucking, waxing, chemical epilatories, electrosurgical epilation), and hair removal using light sources and lasers.

Primary generalized and localized hypertrichosis in children.

OBJECTIVE: To review the causes, presentation, and therapy of primary generalized and localized symmetrical hypertrichosis in children. DESIGN: Retrospective medical record review. SETTING: Academic specialty referral clinic for pediatric dermatological disorders. PATIENTS: Case series of 11 prepubertal male and female patients who had idiopathic hypertrichosis between July 1, 1990, and November 30, 1999. INTERVENTIONS: None. MAIN OUTCOME MEASURES: Clinical distribution of increased hair growth and types of hair removal methods used. RESULTS: Seven girls and 4 boys, ranging in age from 4 months to 11 years, were evaluated. Four patients showed generalized hypertrichosis. The other 7 patients had localized symmetrical hypertrichosis, representing the subsets of hypertrichosis cubiti, anterior cervical hypertrichosis, posterior cervical hypertrichosis, and faun tail deformity. All patients with generalized hypertrichosis manifested the condition at birth; the age of onset in children with localized symmetrical primary hypertrichosis ranged from birth to 4 years. One girl with generalized hypertrichosis had gingival hyperplasia and the girl with faun tail deformity had bony diastematomyelia with spina bifida occulta. The medical histories and physical examination findings of all of the children were otherwise unremarkable. All patients were referred for diagnostic and therapeutic considerations. CONCLUSIONS: Primary hypertrichotic conditions, whether localized or generalized, are rare in pediatric patients and of unknown origin. Although otherwise benign, these disorders may result in cosmetic disfigurement and psychosocial trauma for patients and families. Patients and their families should be adequately advised of the available treatment methods for both temporary and permanent hair removal.

The prevalence of upper lip hair in black and white girls during puberty: a new standard.

In order to develop standards for upper lip hair in adolescent girls, 4693 observations in 856 black and white subjects were made over 9 years. Up to 2 years after menarche, 90% of girls had no upper lip hair. More than 2 years after menarche, 48.8% of black girls and 9.0% of white girls had small amounts of upper lip hair. This may be more significant in adolescent girls than in older women.

[Trichomegaly in HIV infection]. / Trichomegalie bei HIV-Infektion.

Hypertrichosis of the eyelashes was first described as "trichomegaly" in the setting of rare congenital syndromes. Recently numerous cases of acquired trichomegaly of the eyelashes have been described, especially in patients with advanced human immunodeficiency virus type I infection. The pathomechanisms leading to acquired trichomegaly remain largely unknown. Direct effects of the virus on the hair follicle, immune dysregulation, and a multifactorial pathogenesis are discussed. We report a case of acquired trichomegaly in advanced HIV-I infection in order to present this unusual clinical finding and other hair problems in AIDS patients. It is suggested that acquired trichomegaly of the eyelashes may act as a useful clinical marker for assessment of severity of HIV-I infection.

Hypertrichosis universalis congenita: a separate entity, or the same disease as gingival fibromatosis?

Hypertrichosis universalis congenita is an extremely rare disorder characterized by generalized hypertrichosis. It is generally accepted as being inherited as an autosomal dominant trait with varying expression. Many aspects of this disease are still unknown. Several reports associating hypertrichosis and gingival fibromatosis raise the question of whether they are separate entities or the same disease with different expressions of the underlying process. Hypertrichosis universalis congenita occurred in a 6-year-old girl without known family history. Her facial features were simian-like and her gingiva was moderately hyperplastic. We pose the question of whether or not these phenomena are related.

The management of severe hypertension with Minoxidil in a once-a-day treatment regimen

We evaluated the antihypertensive efficacy of "once-a-day" minoxidil, given in conjunction with a diuretic and sympatholytic, and the effect of this simple regimen on patient compliance. Twenty-one severely hypertensive patients and their existing antihypertensive regimens changed to a single daily dose of chlorthalidone (50-100mg) and either nadolol (160 mg) or reserpine (.25mg) for a 3-week period. After stabilization on these two drugs, a single daily dose of minoxidil (2.5 mg) was added to each patient's regimen. Doses were titrated as necessary to achieve diastolic blood pressures of <90 mmHg. After 3 and 6 months of maintenance therapy, blood pressures were measured 24 hours after the previous day's dosing to evaluate the persistence of the antihypertensive effect. Twenty-four-hour blood pressure control was achieved on 76 percent of these occasions, and on at least one occasion in 90 percent of the patients. In addition, compliance was excellent (AU)