Anetoderma or Macular atrophy is a rare cutaneous disorder showing local atrophy and macular discoloration related to a local dermal defect of elastic tissiie without any other apparent change in the skin, which may show familial occurrence, called familial anetoderma. A 13-year-old female complained 3-year history of multiple asyrnptomatic atrophic macules on the trunk and both lower extremities, especially on both this. Elastic stain of skin lesion showed decrease of lastic tissue in the dermis. No other syst mic abnormalities were found on phisical examination. Her sister had same skin lesion on lower extremities and both buttocks, and had no ther systemic abnormalities. Despite infrequent occurence, the development of anetoderma among familial members possibly represents a congcnital defect of elastin. Because variou ocular, bony, cardiac, and gastrointestinal abnormalilies have been reported in familial anetoilerma, long-term follow up would be necessary.
Adolescent ; Anetoderma* ; Atrophy ; Buttocks ; Dermis ; Elastin ; Female ; Follow-Up Studies ; Humans ; Lower Extremity ; Siblings ; Skin

Trichorhinophalangeal syndiome, a rare genetic disease, is characterzed by the triad of slow growing, brittle hair with early loss, a pear-shaped nose with bulbous tip and long philtrum, and coneshaped phalangeal epiphyses wiith resultant shortening and. deformity of hands and feet. A 24-year-old female visited our department for the evaluation of lalopecia. She had had sparse, thin, and brittle hair since birth. She also complained of short fingers ar d a pear-shaped nose with bulbous tip. The X-ray findings of her hands and feet showed cone-shaped epiphyses with shortening of the phalangeal bones. There was no family history of hair, nasal, or palnkoplantar phalangeal abnormality. Chromosomal study did not reveel any abnormal finding. Shortened phalangeal bones of her index and middle fingers were slightly improved by lengthening procedure with iIlizarov apparatus.
Congenital Abnormalities ; Epiphyses ; Female ; Fingers ; Foot ; Hair ; Hand ; Humans ; Lip ; Nose ; Parturition ; Young Adult

No abstract available.
Cartilage*

A case of malignant eccrine acrospiroma of the anterior chest wall is reported. This mass had been present for 20 years and during rescent 5 years it showed frequent ulceration and bleeding suggesting malignant transformation. Microscopically, several foci malignant transformation from preexisting benign eccrine acrospiroma are seen and in immunohistochemical staining, the tumor cells both benign and malignant portion, show positive reaction to cytokeratin.

Pseudo-kaposi's sarcoma is a vasoproliferative disorder that may resemble Kaposi's sarcoma, clinically and histologically. In most cases, it has been associated with congenital or iatrogenic arteriovenous fistula and chronic venous insuffiency. We present a 36-year-old male patient with pseudo-Kaposi's sarcoma caused by a deed vein thrombosis in the absence of any detectable underlying etiologic factors.
Adult ; Arteriovenous Fistula ; Humans ; Male ; Sarcoma ; Sarcoma, Kaposi* ; Thrombosis ; Veins ; Venous Thrombosis*

Segmentalization of spinal fixation enhances correction of the coronal plane deformity and allows preservation of normal sagittal contour offering rigid fixation. Purpose of this paper was to evaluate the efficacy and safety of segmental pedicle screw fixation in the treatment of idiopathic thoracic scoliosis. From 1987 to 1991, 78 idiopathic thoracic scoliosis patients were treated with CD instrumentation. Thirty-one were treated with hooks, 23 with pedicle screws inserted in hook pattern and 24 with segmental pedicle screws. In segmental screw group, the screws were inserted for every other on the convex side. After follow up of minimum 2 years(range:24 to 52 months), the results of coronal, sagittal and rotational correction were compared. Major curve correction was 55% with hooks, 66% with hook pattern screws and 72% with segmental screws, with loss of correction of 6%, 2% and 1% respectively. Compensatory curve correction was 57% with hooks, 67% with hook pattern screws and 70% with segmental screws. In patients with hypokyphosis, all groups showed significant improvement, with best restoration in segmental screws. Rotational correction of the apical vertebra was 19% with hooks, 26% with hook pattern screws and 59% with segmental screws. Thirteen(3%) screws were malpositioned but they did not cause neurologic impairment nor adversely affect the results of treatment. This study implies that the segmental pedicle screw fixation is safe and effective method of correcting the triplanar deformity of the thoracic idiopathic scoliolis.
Congenital Abnormalities ; Follow-Up Studies ; Humans ; Methods ; Pedicle Screws ; Scoliosis ; Spine

Systemic lupus erythematosus (SLE) is a multisystemic disease that can involve the gastrointestinal tract, liver, and biliary system. Symptomatic pancreatic involvement, however, has rarely been reported. It may be part of the primary disease process, such as vasculitic or autoimmune etiology, or associated with drug therapy, in particular corticosteroid. We report here a lupus patient who developed severe pancreatitis within 30 hours of initiation of corticosteroid therapy; we also discuss the relation between pancreatitis and systemic lupus erythematosus.
Biliary Tract ; Drug Therapy ; Gastrointestinal Tract ; Humans ; Liver ; Lupus Erythematosus, Systemic* ; Pancreatitis*

No abstract available.
Gonadotropin-Releasing Hormone* ; Humans ; Hypogonadism* ; Male*

PURPOSE: Cervical neoplasias are known to be preventable. But the outcome of our efforts for early detection in Korea is disappointing. This study was undertaken to determine the level of screening participation in Korean women with cervical cancer and how the early detection of cervical cancer might be improved. MATERIALS AND METHODS: Two hundred and forty-nine women with cervical cancer diag- nosed and treated at Seoul National University Hospital from September 1996 to February 1998 were subjected to this study. Self-reported cervical cancer screening histories, med- ical records obtainable were reviewed and analysed. RESULTS: One hundred forty-seven women (147/249; 59.0%) hadn't got through the screen- ing at proper intervals. Ninety-nine women (39.9%) had never been screened and remain- ing 48 (19.3%) hadn't had their last Pap test 3 years before their diagnosis of nvasive cancer. Of the 150 women (60.1%) who had ever had a Pap test, only 47 (18.9%) had had annual Pap test during recent 5 years and 55 (22.1%) had had routine Pap test with interval between 1-3 years. Among 102 women who had at least one Pap test during recent 3 year, 73 (71.6%) had had a normal Pap report within three years of diagnosis, including 36 (35.3%) whose last normal Pap report was within a year of diagnosis. This results suggest the possibilities of smear-taking and/or reading errors. Women who had had routine Pap with interval less than 3 years had significantfy less chance of advanced tumor (FIGO stage Ib < ) than unscreened population. There was a statistically significant trend for the more younger and educated groups to be participated at the screening program with more shortened interval. All the other factors had failed to show significant correlation with adequacy or regularity of screening interval. CONCLUSIONS: Despite widespread chance of opportunistic cervical cancer screening, non-participants form the main reason for the failure of cervical cancer screening in Korea. So, far much efforts should be aimed at participating more women in cervical cytologic screening program, especially in the old-aged and less-educated groups.
Diagnosis ; Female ; Humans ; Korea ; Mass Screening* ; Nose ; Seoul ; Uterine Cervical Neoplasms*

Ocular complications of HIV-related cryptococcal meningitis are reasonably common, but complete binocular blindness as the first manifestation of HIV is extremely rare. A 58-year-old man presented with binocular blindness. He experienced blurred vision for 3 days before the blindness. Mild pleocytosis was present in the cerebrospinal fluid, from which Cryptococcus neoformans was cultured. Serology revealed positivity for HIV antibody. He was treated with antifungal and antiretroviral therapy. This case indicates that HIV-related cryptococcal meningitis should be taken into consideration when determining the cause of unexpected sudden binocular blindness.
Blindness* ; Cerebrospinal Fluid ; Cryptococcus neoformans ; HIV ; Humans ; Leukocytosis ; Meningitis, Cryptococcal* ; Middle Aged ; Telescopes*