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At least 19 recordsLinked to original sources

Congenital marked hypertrichosis and Laband syndrome in a child: overlap between the gingival fibromatosis-hypertrichosis and Laband syndromes.

Gingival fibromatosis may be reported as an isolated finding or associated with a number of distinct and frequently inherited group of disorders. The characteristics of the Laband syndrome include gingival hyperplasia, dysplasia of the terminal phalanges and nails of extremities, hepatosplenomegaly and facial dysmorphism. Another well-known syndrome with gingival fibromatosis associates generalized hypertrichosis and inconstant mental retardation and epilepsy. We report a case with features of Laband syndrome and congenital marked hypertrichosis, suggesting overlap between these two genetic disorders.

Abnormalities, Multiple↗

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.

Child↗

Ipsilateral post-cast hypertrichosis and dyshidrotic dermatitis.

Post-cast hypertrichosis and dyshidrotic dermatitis are not uncommon separately. Yet, the simultaneous occurrence of both conditions in an individual is rare. A patient with both localized hypertrichosis and ipsilateral dyshidrotic dermatitis following multiple fractures and cast application is reported. The clinical presentation, management, spontaneous resolution, and/or response to therapy of post-cast hypertrichosis and dyshidrotic dermatitis that occurred concurrently in an individual were similar to that described when these conditions occur independently. The acquired conditions that may be associated with localized hypertrichosis are summarized and the postulated mechanisms of pathogenesis for dyshidrotic dermatitis are reviewed. The diagnosis of post-cast hypertrichosis in this patient was suspected based upon the distribution and temporal association of the hypertrichosis to the location and placement of his cast, and confirmed by excluding other causes of acquired localized hypertrichosis. Also, the possibility that in this individual either the trauma to his bones, the cast on his arm, or both had an etiologic role in promoting the development of dyshidrotic dermatitis is suggested.

Adrenal Cortex Hormones↗

Hypertrichosis "cubiti" with facial asymmetry.

Hypertrichosis of the elbow region may be the only abnormality in hypertrichosis cubiti (hairy elbow syndrome). Only 6 cases have been reported; 2 Amish sibs also had additional short stature and, in the most recent case report, a patient had asymmetry of the face, generalized hypotonia, ptosis, epicanthic folds, highly arched palate, and delayed growth and development. The child reported here also had asymmetry of facial growth, ptosis, delayed speech development, and hypertrichosis in a patchy distribution which included the elbow regions, face, trunk, and thighs. There was no family history of hypertrichosis, and the karyotype of cultured fibroblasts was normal in the skin of an area of hypertrichosis. These patients appear to have a distinct condition compared to other hypertrichosis syndromes.

Elbow↗

Localized hypertrichosis associated with periorbital neurofibroma: clinical findings and differential diagnosis.

BACKGROUND: Congenital localized hypertrichosis in the periorbital region is an uncommon finding. The authors report two patients with hypertrichosis and cutaneous hyperpigmentation overlying a periorbital neurofibroma. METHODS: In addition to a complete ophthalmic and systemic examination, the patients underwent computed tomography of the head and biopsy of the tumor. RESULTS: Case 1 previously had received a diagnosis of neurofibromatosis type I. On examination, hyperpigmentation, hypertrichosis, and swelling in the right supraorbital region were noted. A computed tomographic scan showed a tumor in the same region. The tumor was removed, and a plexiform neurofibroma was diagnosed. Case 2 was admitted with hyperpigmentation, hypertrichosis, and swelling of the left half of her face. Other signs of neurofibromatosis were absent. A computed tomographic scan showed a tumor, which was underlying the skin changes. Results of histologic examination of the biopsy specimen showed a plexiform neurofibroma. CONCLUSION: Neurofibroma-associated hypertrichosis should be considered in the differential diagnosis of congenital localized hypertrichosis.

Adolescent↗

Hypertrichosis terminalis, gingival hyperplasia, and a characteristic face: a new distinct entity.

Congenital generalized hypertrichosis terminalis has been described in association with other features as gingival hyperplasia, osteochondrodysplasia, and a dysmorphic face. Bondeson and Miles [1993: Am J Med Genet 47:198-212] described a woman with universal congenital hypertrichosis terminalis associated with gingival hyperplasia; the face of this patient was coarse and different from other forms of hypertrichosis described before. We present an 11-year, 6-month-old girl with universal congenital hypertrichosis terminalis, gingival hyperplasia, and a characteristic coarse face resembling the patient described by Bondeson and Miles [1993: Am J Med Genet 47:198-212]. We propose that this type of congenital generalized hypertrichosis terminalis, associated with gingival hyperplasia and a coarse face, is a distinctive new entity.

Abnormalities, Multiple↗

Clinical assessment and serum hormonal profile in prepubertal hypertrichosis.

Twenty-two prepubertal girls with hypertrichosis were studied and compared to 10 prepubertal normal girls. Hypertrichosis was assessed according to a score that considers the amount and the distribution of vellus hair in androgen- and non-androgen-sensitive areas. Serum androgen profile and free androgen index (FAI) were determined in both groups. The hypertrichosis score was higher in patients than in the normal girls. Testosterone levels and FAI were increased in patients when compared to control; 3alpha-androstanediol glucuronide levels above 2 SD from the control mean were found in 10 girls and all hormonal parameters falling in the normal range were found in 4 girls. The new score designed to assess the degree of hypertrichosis was useful to differentiate between normal and pathological hair growth. Although most of the girls with prepubertal hypertrichosis showed an increased androgen bio-availability, a slight increase in peripheral 5alpha-reductase activity and a completely normal androgen profile was also associated with a pathological hair growth.

Androgens↗

Spironolactone therapy in hypertrichosis.

Although regarded as a benign condition, simple hypertrichosis may be very disturbing to a child cosmetically. An abnormality in androgen metabolism has been implied in the etiology of simple hypertrichosis recently. This study was undertaken to investigate the effect of spironolactone therapy for its antiandrogenic property in 12 prepubertal girls with hypertrichosis with no underlying etiology. The girls, with a mean age of 6.9 (1.2) years, had normal height and bone age. Basal hormone levels and adrenocorticotropin stimulation test results were in the normal ranges. Pelvic and adrenal ultrasound did not reveal pathology. Total and medullary hair width were measured from hair taken from preauricular, anterior midthigh, distal and proximal forearm areas. Spironolactone was started at an oral dose of 25 and increased to 100 mg x m(-2) x day(-1) twice daily for 1 year. Total hair width decreased significantly in the preauricular and anterior midthigh regions up to 6-9 months of treatment. Medullary hair width, which was affected by therapy to a greater extent, decreased significantly in all regions up to 6 months. Both total and medullary hair width showed a tendency to increase afterwards. No side effects were encountered. Spironolactone may be used in the treatment of simple hypertrichosis; however, more data are needed to clarify the efficacy and safety of anti-androgen therapy in hypertrichosis.

Administration, Oral↗

Causes and management of hypertrichosis.

Hypertrichosis is the term used for the 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-induced hair growth. In its generalized and circumscribed forms, hypertrichosis may either be an isolated finding, or be associated with other abnormalities. Therefore, accurate classification of hypertrichosis is mandatory. Excessive hair may cause cosmetic embarrassment, resulting in a significant emotional burden, particularly if extensive. Treatment options are limited, and the results of therapy not always satisfactory. Patients should, therefore, be adequately advised of the available treatment modalities for temporary or permanent hair removal. No single method of hair removal is appropriate for all body locations or patients, and the one adopted will depend on the character, area, and amount of hair growth, as well as on the age of the patient, and their personal preference. The currently available treatment methods include cosmetic procedures (bleaching, trimming, shaving, plucking, waxing, chemical depilatories, and electrosurgical epilation), and hair removal using light sources and lasers. Laser-assisted hair removal is the most efficient method of long-term hair removal currently available. The lack of comparative data make it difficult to choose the most effective system, however, although the color contrast between epidermis and the hair shaft will determine the type of laser to favor. A novel treatment for slowing excessive hair growth is topical eflornithine, an inhibitor of the enzyme ornithine decarboxylase present in hair follicles that is important in hair growth. In general, treatment of hypertrichosis is more satisfactory for patients with localized involvement, than for those with generalized hypertrichosis.

Androgen Antagonists↗

Acquired hypertrichosis languiginosa. Report of two new cases and a review of the literature.

Hypertrichosis lanuginosa is a pathologic state characterized by an excessive, new growth of fine, fetal hair. Two cases of hypertrichosis languinosa with malignancy (lymphoma and uterine cancer) are presented and added to the 9 in the literature. Lymphoma and uterine cancer are previously unreported as associated with hypertrichosis osis lanuginosa. Review of the 11 cases of hypertrichosis lanuginosa revealed the following characteristics: females were predominant; none was below the 4th decade; all had advanced neoplastic disease; all malignancies except one were of epithelial origin; and there were no demonstrable endocrine abnormalities. Despite an attempt to find etiologic factors in our patients and in the literature, none could be elicited.

Adenocarcinoma↗

Hypertrichosis and increased pigmentation of eyelashes and adjacent hair in the region of the ipsilateral eyelids of patients treated with unilateral topical latanoprost.

PURPOSE: To describe hypertrichosis and increased pigmentation of eyelashes associated with topical latanoprost usage. METHODS: Patients using unilateral topical latanoprost for glaucoma were examined for evidence of hypertrichosis and pigmentation of eyelashes and adjacent hair in the latanoprost-treated eye compared with the nontreated control eye. RESULTS: In 43 patients receiving unilateral topical latanoprost, hypertrichosis involved ipsilateral terminal eyelashes and regional intermediate hairs of the upper and lower eyelid as well as vellus hair of the lower eyelid skin. Differences in hair appearance between the latanoprost-treated eye and the nontreated control eye included increased number, length, thickness, curvature, and pigmentation. CONCLUSIONS: Latanoprost-treated eyes develop hypertrichosis and increased pigmentation in the region of treatment. Patients may benefit from being made aware of this side effect.

Administration, Topical↗

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↗

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↗