BACKGROUND: Congenital hypotrichosis is a non-specific, descriptive term for structural abnormalities of hairs showing variable clinical features. We recently have encountered a group of eleven patients exhibiting abnormal hairs showing similar patterns. OBJECTIVES: Our purpose was to clarify the entity of this structural hair disorder. METHODS: Dermatologic examination with routine histopathology, trichograms along with scanning electron-microscopic examination and laboratory studies were undertaken. RESULTS: All cases except one were female, and hair abnormalities had developed at birth or within a year. Diffuse distribution of thin, sparse, soft and hypopigmented hairs were noticed. On hair mounts, four patients showed tapering of roots. The majority of the patients exhibited cuticular changes, as well as pitting and longitudinal axial twisting by scanning electron-microscopic examination; one case demonstrated trichorrhexis nodosa, and another, trans-verse fracture. CONCLUSION: Although our cases bear some similar points with woolly hair, some differences were noted between this type of congenital hypotrichosis and other previously described syndromes.
Female ; Hair ; Humans ; Hypotrichosis* ; Parturition

Twenty years old Korean male patient with Hallermann-Streiff syndrome who developed glaucoma is reported. The clinical features are dyscephalia with mandibulo-oculo-facial malformation, bilateral congenital cataract, dental anomalies, hypotrichosis, microphthalmia, nanism and the secondary complications. A brief review of literature is described.
Cataract ; Dwarfism ; Glaucoma ; Hallermann's Syndrome* ; Humans ; Hypotrichosis ; Male ; Microphthalmos

BACKGROUND: The incidence of hypotrichosis of pubis and atrichia pubis is relatively higher in oriental countries including Korea. OBJECTIVE: The aim of this study was to develop a treatment method to increase the density and to improve the distribution pattern of pubic hair in these patients. METHODS: Testosterone cream(5%) was applied to ten patients for six months. Then, the individual hair transplantation from the parietotemporal region of the scalp with the specially devised mini-punch was performed. Around the mons pubis, transplantation with 2-3mm punch was done. Postoperatively, the application of testosterone cream was done twice a day. RESULTS: Nine of ten patients were satisfied with the final results. Testosterone cream helped to increase the density of pubic hair. Transplanted hairs showed curling to some extent and did not grow like as it were at donor site. CONCLUSION: The advantage of this method is to make the appearance of pubic hairs more natural by controlling the direction and numbers of transplanting hairs at the time of operation and by making the hair denser with pre- and post-operative application of testosterone cream. The authors hope many dermatologic surgeons to use this new treatment approach for atrichia pubis and hypotrichosis of pubis.
Hair ; Hope ; Humans ; Hypotrichosis* ; Incidence ; Korea ; Scalp ; Testosterone* ; Tissue Donors

Basaloid follicular hamartoma is a benign adnexal tumor consisting of basaloid cells with follicular differentiation. It can present as a solitary lesion, multiple lesions, or an autosomal dominant inherited syndrome (basaloid follicular hamartoma syndrome). Basaloid follicular hamartoma syndrome is a rare adnexal tumor genodermatosis, which is characterized by autosomal dominant inheritance, histologically proven basaloid follicular hamartomas and associated findings including hypotrichosis, palmar/plantar pitting and hypohidrosis, etc. Herein, we report a case of 4-year-old girl with familial generalized basaloid follicular hamartoma syndrome.
Child, Preschool ; Female ; Hamartoma* ; Humans ; Hypohidrosis ; Hypotrichosis ; Wills

We report a family suffering from Marie Unnas hypotrichosis (8 members affected in 4 generations). At birth they had normal hairs, subsequently loss of hair on the scalp, eyebrow and eyelash. At about 3rd years, coarse twisted scalp hair regrow until puberty when progressive loss from vertex and scalp margin was noticed. On physical examination and lsboratory studies, there was no associated abnormality in the patients. The scanning electron microscopic examination of the affected hair showed cuticular peeling and twisting or angulation of hair shaft.
Adolescent ; Eyebrows ; Hair ; Humans ; Hypotrichosis* ; Parturition ; Physical Examination ; Puberty ; Scalp

'Pubic atrichosis or hypotrichosis' is defined as the agenesis or the insufficient amount of pubic hair nevertheless the presence of other secondary sexual characteristics are normal. Such females may suffer from low self-esteem and psychological problem in addition to the aesthetic problem. In past, the treatment of atrichosis or hypotrichosis was done mainly with such medical therapeutics as hormonal drug therapy, but treatment tends toward the single hair transplantation in these days. Therefore, the importance of preoperative design have been emphasized to obtain more natural appearances and satisfactory results. We have studied for the pattern, hair line, distribution, density, direction, and angle to skin surface of pubic hair in 50 adult females. In pattern of pubic hair, the most common type was the horizontal type(64%) and the acuminate, the dispersed, the few type followed in order. But, the sagittal type was not observed. In the horizontal type, the upper border of line of pubic hair was located at 3/6-4/6(53%) or 4/6-5/6(47%) point of the distance from junctional point of both inguinal ligaments to the horizontal line passing both anterior superior iliac spines. In the acuminate type, most of them(75%) were located at 4/6-5/6 point of the distance from junctional point of both inguinal ligaments. In the horizontal and the acuminate type, the most lateral points of pubic hair were located, between right and left symmetrically, at 2/6-3/6 point of distance between both anterior superior iliac spines. The density of pubic hair at mons pubis, superior and lateral region of labium major didn't have no statistical difference and the central region had higher density than peripheral region. The direction of pubic hair was equal to Langer's line, angle of hair shaft to skin surface was about 60 degrees at central region and it was more acute angle at the peripheral region. In transplantation of pubic hair, it is important to know anatomical shape of pubic hair in normal adult female. If a surgeon considers the pattern, hair line, distribution, density, direction, and angle of hair shaft to skin in preoperative design and hair transplantation, one can obtain more natural-appearing and satisfactory results.
Adult* ; Drug Therapy ; Female* ; Hair* ; Humans ; Hypotrichosis ; Ligaments ; Skin ; Spine

Hereditary hypohidrotic ectodermal dysplasia is a genodermatosis that demonstrates in its typical form a triad of hypohidrosis, hypotrichosis, and hypodontia Despite marked abnormalities of ectodermal structures, reports of cataract and corneal opacity have been conspicuously rare. This paper is a report of a case which shows the typical findings of HHED, congenital cataract, and corneal opacity.
Anodontia ; Cataract* ; Corneal Opacity* ; Ectoderm ; Ectodermal Dysplasia 1, Anhidrotic* ; Hypohidrosis ; Hypotrichosis

A case of Hallermann-Streiff syndrome characterized by dyscephalia with bird face, dental anomalies, proportionate nanism, hypotrichosis or atrichosis, atrophy of the skin, bilateral microphthalmia and presumed congenital cataract was reported. In this case, the diffuse enlargement with hyperfunction of the thyroid gland was observed and its role in the etiology of this syndrome is briefly reviewed.
Atrophy ; Birds ; Cataract ; Dwarfism ; Hallermann's Syndrome* ; Hypotrichosis ; Microphthalmos ; Skin ; Thyroid Gland

Hallermann-Streiff syndrome is a rare disease. Approximately 150 cases have been reported, including 6 cases in Korea. The authors experienced a case of Hallermann-Streiff syndrome in a 6-year-old female with aphakia. The syndrome is characterized by a bird-like face, dental abnormalities, hypotrichosis, atrophy of the skin, bilateral microphthalmia, and proportionate dwarfism. A brief review of the literature was conducted.
Aphakia ; Atrophy ; Child ; Dwarfism ; Female ; Hallermann's Syndrome ; Humans ; Hypotrichosis ; Korea ; Microphthalmos ; Rare Diseases ; Skin

We report a mild variant of hypomelia-hypotrichosis-facial hemangioma syndrome in a 3 month-old female. The patient showed major features of the previously reported cases including flexion deformities of both upper extremities, sparse light colored hair, mid-facial hemangioma and a characteristic facial appearance. Roentgenogram showed radio-humeral synostosis at both elbow joints by which shortening of upper limbs could be anticipated. However, intra- and extrauterine growth retardation were not remarkable and the lower limbs were not affected.
Congenital Abnormalities ; Elbow Joint ; Female ; Hair ; Hemangioma* ; Humans ; Hypotrichosis ; Lower Extremity ; Synostosis ; Upper Extremity