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Congenital universal hypertrichosis with deafness and dental anomalies inherited as an X-linked trait.

We report a large Mexican kindred with a variant form of congenital universal hypertrichosis that is inherited in an apparent X-linked recessive manner. In addition to the generalized hypertrichosis, the affected individuals have dental malformations and deafness. Males are more severely affected than females who exhibit only mild hypertrichosis, but not deafness or dental anomalies. Haplotype analysis in this pedigree revealed linkage to a 13-cM region on chromosome Xq24-q27.1 between markers GATA198A10 and DXS8106. Localization of the gene underlying this form of hypertrichosis is the initial step in identifying genes on the X chromosome that are involved in the control of hair growth and development.

Chromosome Mapping↗

Neonatal hypertrichosis in an infant of a diabetic mother with congenital hypothyroidism.

Hypertrichosis in a newborn girl infant of a diabetic mother with congenital hypothyroidism is reported. Both neonatal hyperinsulism and increased testosterone levels were documented. The hypertrichosis resolved after 3 months' of thyroxine replacement treatment. The possible causal association between hypothyroidism, and hypertrichosis has not been previously reported in neonatal period. Thyroid function should be evaluated in all newborn babies with hypertrichosis or abnormal distribution of body hair.

Adult↗

Hypertrichosis.

Hypertrichosis is hair growth that is abnormal for the age, sex, or race of an individual, or for a particular area of the body. Recognized forms of hypertrichosis are reviewed. Hirsutism, which is male-pattern hair growth in a female or child, is not included in this review. Hypertrichosis is categorized as congenital or acquired, and regional or generalized. Methods of managing hypertrichosis are also briefly reviewed

Humans↗

Diffuse hypertrichosis in the course of hepatitis C treatment by IFN-alpha and ribavirin.

A 32-year-old man suffered from hemophilia and hepatitis C. Treatment by interferon-alpha (IFN-alpha) and ribavirin was prescribed. After 6 months, the patient noted a diffuse hair growth, whereas he was beardless before treatment. This hypertrichosis was treated by laser therapy. No hypertrichosis is known in patients with hemophilia or viral hepatitis. Ribavirin has not been described as an inducer of hair growth. IFN-alpha is known to induce telogen effluvium, but some cases of hypertrichosis of the eyelashes or eyebrows have been reported. In this patient, the hypertrichosis is probably caused by IFN.

Adult↗

Acquired trichomegaly and hypertrichosis in metastatic adenocarcinoma.

We report the case of a non-HIV patient suffering from a metastatic renal adenocarcinoma who developed trichomegaly 3 months before death. The patient also developed generalized hypertrichosis of the terminal hair, a feature that has been previously described in malnutrition. Other cases of hypertrichosis, including ectopic hormone production, were excluded and the differential diagnosis with paraneoplastic acquired hypertrichosis lanuginosa is discussed. Although trichomegaly is usually considered as a cutaneous marker of AIDS, we suggest that both trichomegaly and hypertrichosis of the terminal hair were secondary to malnutrition.

Adenocarcinoma↗

Ambras syndrome: delineation of a unique hypertrichosis universalis congenita and association with a balanced pericentric inversion (8) (p11.2; q22)

Congenital hypertrichosis universalis is a rare autosomal dominant disease. We report the further development of a Greek girl, now aged 3 years, the first case associated with a balanced structural chromosomal aberration. She was described as a neonate by Sigalas et al. (1990). Her persistent generalized hypertrichosis is most excessive on the face, ears and shoulders. Her fine silky hair is of the vellus, not the lanugo type. The syndrome features are characterized, referring to nine further published case reports. It is distinguished from other types of congenital hypertrichoses, which have been described in the literature under different synonyms. To avoid confusion in the terminology, we propose to name this type of hypertrichosis Ambras syndrome in reference to the first documented family with congenital hypertrichosis universalis in the 16th century.

Child, Preschool↗

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.

Child↗

[Acquired paraneoplastic hypertrichosis lanuginosa associated with scleroderma].

Acquired hypertrichosis lanuginosa is universally recognized as an individual disease and seldom reported as a genuine paraneoplastic manifestation. We report the case of a 30-year old woman with acquired hypertrichosis lanuginosa. Due to the finding of a cervical lymph node metastasis, she was investigated for an internal neoplasm, but the original tumour could not be found by the usual methods. A bronchogenic carcinoma was discovered at autopsy. Beside hypertrichosis, this patient had other disorders not described in the literature as associated with that disease, viz.: progressive systemic scleroderma, fissured and hyperpigmented tongue, thrombocytopenia, galactorrhoea, axillary and pubic alopecia and overcurvature of toe nails. A review of similar cases in the literature provided clinical arguments in favour of the hormonal origin of this paraneoplastic hypertrichosis.

Adult↗

Hypertrichosis, pigmentary retinopathy, and facial anomalies: a new syndrome?

We report on a 22-month-old male with congenital hypertrichosis of the face, arms, legs, shoulders, back, and buttocks, abnormal facial appearance, dolichocephaly, and pigmentary retinopathy. Symmetrical hyperpigmentation is present on the sideburn areas of his face, and hyperpigmented streaks are seen on arms and legs. Biopsy of the hyperpigmented' skin showed many separate bundles of smooth muscles in the dermis. No relative had hypertrichosis or other birth defects. To our knowledge, the syndrome of facial anomalies, pigmentary retinopathy, and congenital hypertrichosis has not been reported previously.

Abnormalities, Multiple↗

Hypertrichosis cubiti.

Hypertrichosis is an unusual but well-recognized genetic condition. Hypertrichosis may be generalized or limited to specific body areas, in which case it is usually not associated with other anomalies. Five previous cases of hypertrichosis cubiti have been reported, with short stature in 2 sibs being the only other associated abnormalities. We report on a child with hairy elbows, developmental delay, facial asymmetry, and delayed speech with normal parents. Our patient may represent severe expression of the hairy elbow syndrome or constitute a previously unrecognized syndrome.

Child, Preschool↗

Julia Pastrana, the nondescript: an example of congenital, generalized hypertrichosis terminalis with gingival hyperplasia.

Julia Pastrana (1834-1860) has gained immortality as one of the most extreme cases of generalized hypertrichosis upon record. When she was exhibited for money in the United States and Europe during the years 1855-1860, people thronged to see her, and she was several times described in the medical press of the day. After Julia Pastrana's death in childbirth, her corpse was embalmed in a very life-like manner, and exhibited all over Europe for several decades. Later, the mummy was believed to be lost, but in 1990 it was discovered at the Oslo Forensic Institute. Some writers have included Julia Pastrana among the cases of congenital hypertrichosis languinosa. However, a microscopic examination of hair samples from the mummy shows that her hairy growth is unmistakably terminal in character, and we propose that she instead was an example of congenital, generalized hypertrichosis terminalis with associated gingival hyperplasia. While many earlier writers have asserted that Julia Pastrana's dentition was abnormal, a radiographic examination of the mummy has shown that she had a complete permanent dentition.

Face↗

Mental retardation in a boy with anterior cervical hypertrichosis.

Anterior cervical hypertrichosis (ACH) is a rare form of localized hypertrichosis with 15 previously reported cases. ACH has been considered to be a dominant phenotype, either X-linked or autosomal [OMIM 600457]. ACH was associated with hereditary motor and sensory neuropathy (HMSN) in one family, in which the proband also exhibited severe chorioretinal degeneration and optic atrophy, probably as a different entity [OMIM 239840]. A Mexican boy with congenital ACH associated with moderate mental retardation, abnormal EEG, mild microcephaly, hypertrichosis on the back, and hallux valgus is presented here. An equal sex ratio found in 16 reported cases as well as the suggestion of a paternal age effect in one report appear most consistent with an autosomal dominant mode of inheritance for this trait. It remains unclear if isolated ACH, ACH-HMSN, or other associated findings reported in patients with ACH, including unusual features found in our case, are part of ACH or fortuitous associations, due to the small number of affected patients and different ascertainment biases present in previous reports.

Abnormalities, Multiple↗

[Current treatment of hypertrichosis].

Facial hypertrichosis and hirsutism may cause severe cosmetic and psychologic problems. In the following, new developments in the treatment of hypertrichosis will be presented. Permanent depilation by photothermolysis is currently the most promising treatment. A new topical treatment of hypertrichosis is eflornithine cream. It inhibits the enzyme ornithine decarboxylase which is essential for the rapidly dividing cells of the hair follicle. Furthermore, other methods of hair removal such as plucking, waxing, chemical depilation by thioglycolates, electrolysis, thermolysis and systemic therapies of hirsutism will be discussed.

Eflornithine↗

Hypertrichosis of vellus hairs of the malar region after unilateral treatment with bimatoprost.

PURPOSE: To report hypertrichosis and increased pigmentation of vellus hairs of the malar region associated with topical bimatoprost usage. DESIGN: Interventional case report METHODS: In a female Hispanic patient with open-angle glaucoma, hypertrichosis, and increased pigmentation of the vellus hairs of the malar region developed after 8 weeks of unilateral treatment with bimatoprost. Increased growth of eyelashes was also noted. RESULTS: Bimatoprost was discontinued, with the intention of monitoring for resolution of hypertrichosis. The patient epilated the hair because of poor cosmetic appearance. Two months after epilating the hair, growth did not recur. CONCLUSIONS: Increased hair growth in the malar region can be an early side effect of topical use of ocular bimatoprost.

Amides↗

Paradoxical hypertrichosis after laser epilation.

OBJECTIVE: Hair removal using lasers or broadband intense pulsed light has become one of the most ubiquitous medical procedures. At our center a small proportion of patients have spontaneously reported what they believed to be increased hair growth at sites of previous laser epilation. We sought to retrospectively review the prevalence and features of this paradoxical effect. METHODS: This was a single center, retrospective study that included all patients who underwent laser hair removal during a 4-year period with a long-pulsed alexandrite laser. All cases of laser-induced hypertrichosis were assessed clinically by history, examination, and laboratory tests, and confirmed by review of serial clinical photographs taken during the course of the laser treatments. The clinical features of patients with postlaser hypertrichosis were compared with 50 patients randomly selected from among all those who had undergone laser hair removal at our center (n = 489). RESULTS: Of 489 patients, 3 (0.6%, 95% confidence interval: 0.01-1.9%) treated with the long-pulsed alexandrite laser (755 nm) reported increased hair after laser hair epilation. There was a trend for this adverse effect to occur in darker skin phototypes (IV) and with black hair as compared with the unaffected comparison group (n = 50). However, the small number of cases (n = 3) did not provide sufficient power to adequately test factors such as age, sex, treatment settings, and number of treatments statistically. CONCLUSION: Postlaser hair removal hypertrichosis is a real but rare occurrence in our experience.

Adult↗

[Acquired paraneoplastic hypertrichosis lanuginosa].

Acquired hypertrichosis lanuginosa, an infrequent, paraneoplastic process, consists of the rapid development of lanugo-type hair, primarily on the face. We describe a 50-year-old woman with a 4-month case of hypertrichosis on the face and upper body, and weight loss over the last 6 months, in whom an inoperable squamous cell carcinoma of the cervix was discovered. Hypertrichosis lanuginosa is an indicator of a poor prognosis. It is associated with tumors in any location, but most often with lung and colorectal tumors. This is the first case associated with squamous cell carcinoma of the cervix.

Carcinoma, Squamous Cell↗

[Hypertrichosis and hirsutism].

Facial hypertrichosis and hirsutism may cause severe cosmetic and psychologic problems. There are several therapeutic options, both on the cosmetic and medical level. Hirsutism with androgen-excess should be diagnosed and treated by gynecologists. Although frequently used, systemic antiandrogens have not yet proven their efficacy against unwanted hypertrichosis in clinical studies using modern and objective endpoints. Conventional methods of hair removal include plucking, waxing and chemical depilation by thiogycolates, as well as electrolysis and thermolysis. A new medical treatment of hypertrichosis is eflornithine cream. It inhibits the enzyme ornithine-decarboxylase which is essential for the rapidly dividing cells of the hair follicle. Permanent depilation by photothermolysis with lasers and pulsed flash-light systems is currently the most promising treatment.

Clinical Trials as Topic↗

Congenital generalized terminal hypertrichosis with gingival hyperplasia.

Congenital generalized terminal hypertrichosis is a rare disease, especially when associated with gingival hyperplasia. Congenital hypertrichosis can be a clinical feature of several syndromes, so these patients must be studied by a multidisciplinary team that should include a dermatologist, geneticist, psychologist, odontologist, and an endocrinologist. We report a 7-year-old girl with congenital generalized hypertrichosis and gingival hyperplasia, and analyze the clinical approach, differential diagnosis, and treatment.

Child↗