Pilomatricoma is a benign tumor derived from the hair matrix. Pilomatricoma-like changes have been described in epidermal cysts in Gardner's syndrome. Apart from this association, a cyst can arise from various portions of a hair follicle, and combine to form a hybrid cyst. Follicular hybrid cyst is an interesting pathologic phenomenon, and needs to be explained for their pathogenesis. We present a case in which a pilomatricoma was situated in the immediate vicinity of an epidermal cyst. Histopathologic findings showed that one part of the cyst had a feature of an epidermal cyst, and the other part had a feature of a pilomatricoma.
Chimera ; Epidermal Cyst ; Gardner Syndrome ; Hair ; Hair Follicle ; Pilomatrixoma

Tumor of the follicular infundibulum(TFI) is a rare benign adnexal tumor originating from the infundibular portion of hair follicle. Majority of the lesions have been solitary and not recognized clinically before biopsy. TFI can be classified as follows according to the clinical presentation of the tumor (1) solitary tumor, (2) eruptive tumors, (3) TFI associated with other lesions of Cowden's disease, (4) TFI arising in nevus sebaceus, and (5) TFI-like changes in various tumors. We present clinicopathologic features of 2 cases of TFI. One was a solitary form and the other was associated with nevus sebaceus.
Biopsy ; Hair Follicle ; Hamartoma Syndrome, Multiple ; Nevus

No abstract available.
Hair ; Humans ; Klinefelter Syndrome ; Male ; Young Adult

A case of adrenogenital syndrome which occurred in a girl of the age of 5 years, having the chief complaint of the abnormal external genitalia such as enlarged clitoris, abnormal urethral meatus and pubic hairs, was herein presented with brief review of literatures.
Adrenogenital Syndrome* ; Clitoris ; Female ; Genitalia ; Hair ; Humans

Humans ; Infant ; Male ; Menkes Kinky Hair Syndrome

A 37-year old primigravid with HAIR-AN syndrome on Metformin therapy achieved spontaneous pregnancy after fifteen years of infertility.
Human ; Female ; Adult ; HAIR-AN SYNDROME ; HYPERINSULINISM ; HYPERANDROGENISM

Menkes disease, so-called kinky hair disease or steely hair disease, is a rare X-linked recessive disorder of intracellular copper transport protein ATP7A defect, due to mutation of ATP7A gene, resulting in copper deficiency. It is characterized by seizure, retarded neurological development, kinky hair, skeletal abnormality, recurrent infection and subnormal body temperature. In addition, gastroesophageal reflux with the risk of aspiration is another important feature. This article is the first report of anesthetic management in a patient with Menkes disease who underwent gastrostomy and bladder diverticulectomy in Korea.
Body Temperature ; Copper ; Gastroesophageal Reflux ; Gastrostomy ; Hair ; Humans ; Korea ; Menkes Kinky Hair Syndrome* ; Seizures ; Urinary Bladder

A 24-year Korean woman presented with bizarre pigmentary skin changes. Eruptive vellus hair cysts (EVHC) were observed in conjunction with Hypomelanosis of Ito (HI). Also, the karyotype of 45XO with typical clinical symptoms of Turner's syndrome was detected in this patient, which has been rarely reported in HI as a chromosomal defect. EVHC may manifest as a rare skin manifestation of Hl.
Female ; Hair* ; Humans ; Hypopigmentation* ; Karyotype ; Skin ; Skin Manifestations ; Turner Syndrome*

Trichoptilosis is a longitudinal splitting or fraying of the distal end of the hair caused by cumulative physical and/or chemical effects on the hair shaft. We present three cases of trichoptilosis patients complaining of alopecia, two cases with the scalp hairs and one case with the pubic hairs. One of the two scalp hair cases showed the hair shaft disorder as a part of a Tourette's syndrome and the other case was caused by scalp pruritus. The patient with pubic hair involvement showed hypochondriasis on his genital area. The cases were thought to be a good example showing the stereotypic change of the hair shaft in various different underlying diseases of the affected patients.
Alopecia ; Hair ; Humans ; Hypochondriasis ; Pruritus ; Scalp ; Tourette Syndrome

OBJECTIVES: The purpose of this study was to examine the association of exposures to heavy metals with positive diagnosis for attention-deficit hyperactivity disorder (ADHD) and Tourette's syndrome (TS). METHODS: Study participants included 27 children diagnosed with ADHD (9.9+/-2.9 years of age), 21 diagnosed with Tourette's disorder (10.7+/-2.2 years of age), and 45 normal control children (9.6+/-0.5 years of age). A Perkin-Elmer mass spectrometer was used to measure the concentrations of 5 heavy metals (Pb, Cd, U, Be, Hg) in hair samples obtained from each participant. Each heavy metal concentration was compared among the groups by use of a Kruskal-Wallis test. RESULTS: The levels of lead (p=.006) and cadmium (p=.037) observed in the hair of children diagnosed with ADHD were significantly higher than those found in the control subjects. There were no significant differences observed for heavy metal levels when comparing TS and control subjects. CONCLUSION: We confirmed that lead exposure is a risk factor for ADHD. We also identified that cadmium may be a new candidate risk factor for manifestation of ADHD. We did not find an association between heavy metals and manifestation of TS.
Cadmium ; Child ; Hair ; Humans ; Metals, Heavy ; Risk Factors ; Tourette Syndrome