Ehlers-Danlos syndrome is an inherited disorder of connective tissue in which joint hypermobility, hyperelasticity of skin, bleeding tendency, and scar formation are the most prominent features. It is a generalized disease with essentially universal involvement. A 3-year-old female child had velvety skin, skin hyperextensibility, joint hypermobility, subcutaneous mobile masses on the left shin, many scars on both knees, subcutaneous hematoma, and thick and very folded skin on both palms and soles, but no evidence of internal disorder. Cutaneous histopathologic findings were nonspecific without increase of dermal elastic fibers on Verhoeff stain. Diagnosis was confirmed by clinical and histopathological findings as Ehlers-DanIos syndrome.
Child ; Child, Preschool ; Cicatrix ; Connective Tissue ; Diagnosis ; Ehlers-Danlos Syndrome ; Elastic Tissue ; Female ; Hematoma ; Hemorrhage ; Humans ; Joint Instability ; Knee ; Skin

The authors studied EBV genome expression in 40 conventionally processed samples of nasopharyngeal carcinomas (NPC), using in situ hybridization for EBERs and immunohistochemistry for LMP, Bcl-2 and p53 proteins. The NPCs consisted of 6 keratinizing squamous cell carcinomas (KSCs), 13 nonkeratinizing carcinomas (NKCs) and 21 undifferentiated carcinomas (UCs). The results were summarized as follows: 1) EBERs were expressed in 80.0% of all the NPCs (32/40). As for the subtypes, they were detected in 92.3% of NKCs (12/13), in 90.5% of the UCs (19/21), and in 16.7% of the KSCs (1/6). In positive cases, the nuclei of tumor cells displayed uniformly strong staining. 2) LMP was expressed in 10.0% of all the NPCs (4/40), all of which were UC. The LMP expression in the UCs was not correlated to the expression of EBERs, Bcl-2 and p53 proteins. 3) Bcl-2 protein was detected in 85.0% of all the NPCs (34/40). As for the subtypes, they were detected in 92.3% of the NKCs (12/13), in 90.5% of the UCs (19/21), and in 50.0% of the KSCs (3/6). 4) p53 protein was detected in 75.0% of all the NPCs (30/40). As for the subtypes, they were detected in 81.0% of the UCs (17/21), in 69.2% of the NKCs (9/13), and in 66.7% of the KSCs (4/6). 5) In the NPCs the expression of EBER showed a significantly positive correlation with that of p53 or Bcl-2 protein. The above results indicate that the association of EBV with NPC is chiefly with poorly differentiated and undifferentiated carcinomas. Additionally, carcinomas commonly display widespread, strong immunoreactivity of Bcl-2 and p53 proteins over tumor cells. In conclusion, these observations indicate that the EBV-association in NPC appears to contribute to the overexpression of tumor-related genes during carcinogenesis.
Carcinogenesis ; Carcinoma ; Carcinoma, Squamous Cell ; Genome ; Herpesvirus 4, Human* ; Immunohistochemistry ; In Situ Hybridization

BACKGROUND: In eczematous skin it is expected that altered defense mechanisms are employed against an injury, which mechanisms include alterations in the stratum corneum barrier function and lipid contents. OBJECTIVE: To investigaten aspect of pathophysiology of hand eczema by comparing the water holding capacities and transepidermal water loss(TEWL) after injuries on the skin and by comparing the epidermal lipid contents between hand eczema and normal subjects. METHODS: The water holding capacities were checked by corneometer, and the TEWL were checked after epidermal barrie! destruction with acetone or adhesive tapes. The epidermal lipids were extracted with acetone, ther (1: 1 v/v) mixture solvent and analysed by HP- TLC. RESULTS: The water holdingapacities were decreased in eczema of the palm, but the recoveries of TEWL and the lipid content did not differ between hand eczema and normal subjects. CONCLUSION: The water holding capacities were decreased in the eczematous skin. But there were no significant differencies in the recovery of destructed epidermal barrier and the epidermal lipid contents.
Acetone ; Adhesives ; Defense Mechanisms ; Eczema* ; Hand* ; Skin ; Water

Most of cataract surgeons have made a great effort to minimize or nullify corneal 'astigmatism resulted from the incision and closure, but a completely ideal wound system still eludes us. The scleral pocket incision and continuous single knotted shoelace suture of it has been known as one of the technique for reducing postoperative astigmatism. Recently, horizontal suture closure of scleral pocket incisior has been introduced because it doesn't cause suture induced wound compression. We implanted standard PMMA intraocular lenses in the bag of 400 patients through 7mm scleral pocket incision following CCC (continuous circular capsulorhexis) and bimanual phacoemulsification. The incision was closed with either the shoelace or horizontal suture. The keratometric measurement was maded at postoperative 1 day, 1 week, 1 month, 2 month, 3 month and 6 month. In shoelace suture group, the preoperative corneal astigmatism appeared -0.14 +/- 0.15D of with the rule astigmatism (WTR); at one day postoperatively -2.75 +/- 1.61 of WTR, at 3 month 0.18 +/- 1.14D of against the rule astigmatism (ATR), 6 month 0.3 +/- 1.22D of ATR. In horizontal suture group, the preoperative corneal astigmatism appeared -0.22 +/- 1.22D of WTR; at one day postoperatively -1.05 +/- 1.28 of WTR, at 2 month 0.57 +/- 1.0 of ATR, at 6 month 0.72 +/- 1.91 of ATR. The difference between two suture methods was statistically significant (p<0.01 until 3 month, p<0.05 until 3-6 month).
Astigmatism* ; Cataract ; Humans ; Lenses, Intraocular ; Phacoemulsification ; Polymethyl Methacrylate ; Sutures* ; Wounds and Injuries

Papillary eccrine adenoma is a rare sweat gland tumor with a characteristic histopathological appearance and has a benign clinical course. Five cases were reported in Korea since a report by Song et al. in 1988. We report a case of papillary eccrine adenoma in a 38-year-old male who had a firm, dark brown nodule on the medial surface of the left thigh. Microscopically, the tumor was composed of multiple dilated ducts lined by two or more layers of epithelial cells and the luminal cells showed papillary projections into the lumen in some tubules. Immunoperoxidase staining was positive for CEA, S-100, and EMA. We excised the lesion completely and no evidence of recurrence was observed for 6 months.
Adenoma* ; Adult ; Epithelial Cells ; Humans ; Korea ; Male ; Music ; Phenobarbital ; Recurrence ; Sweat Glands ; Thigh

The authors analyzed 174 consecutive shunting procedures for hydrocephalus at Severance Hospital, Yonsei University. There were a total of 65 complications (48.5%) among 134 patients. The most common complications were blockage of the catheters (26.2%) and shunt-related infections (15.4%). Among the different types of shunting technique ventriculoatrial, ventriculoperitoneal and ventriculocisternal routes were most commonly used. The ventriculoatrial route had a greater complication rate (40%) than ventriculoperitoneal shunting (21.8%). Flushing devices are critically reviewed and the disadvantages are discussed. The authors suggested a need for a standardized prospective national data collection system where Procedures and compilations may be assembled in a comparable form.
Bacterial Infections/etiology ; Catheterization/adverse effects ; Cerebrospinal Fluid Shunts*/adverse effects ; Female ; Human ; Hydrocephalus/surgery* ; Infant ; Male

We studied 25 cases of clinically diagnosed erythema dyschrornicum perstans (EDP) who were seen in our clinic within 11-year period, from 1975 through 1985, and compared light and electron microscopic findings, and direct immunoflunrescence findings. The results are summarized as follows: 1. There were 16 males(64%) and 9 females(36%). The peak age incidence was in the second decade. 2. The mean age of onset was 18 years(M: 16. 6, F: 19. 4) and the rnean duration was 1.8 years(M: 2.6, F: 1.0). 3. The annual incidence increased from 1981 and was peaked in 1985. 4. The most commonly involvement site was the trunk(92%), the least common involvement(4%) was one of the face, neck, and shoulder combined. 5. The histopathologic findings in most patients shows focal vacuolization of the basal cell layer, pigment incontinence, dermal melanophages. 6. Electron microscopic findings of five patients consist of vauoles with abundant melanosomes in the basal cells, widened intercellular spaces and dermal melanophages.
Age of Onset ; Erythema* ; Extracellular Space ; Humans ; Incidence ; Melanosomes ; Neck ; Shoulder

BACKGROUND: Neopterin is released from monocytes/macrophages specifically by stimulation with interferon-γ. Therefore the increase refers to the activation of T lymphocytes. OBJECTIVE: Our objective was to examine the potential role of neopterin in the cell mediated immune response to Behçet's disease. METHODS: We studied 67 patients of Behçet's disease classified by Shimizu and a control group of 30 normal healthy people. Serum neopterin was detected by radioimmunoassay technique. RESULTS: The serum neopterin concentration of the group with Behçet's disease was significantly elevated, compared with the control group (6.36 nmol/ml vs 3.63 nmol/ml). The increase of neopterin concentration was well correlated to the clinical severity of the diseases in increasing order. CONCLUSION: Our data suggest that the T lymphocyte derived IFN-γ which stimulates the production of neopterin has some role in the pathogenesis and clinical severity of Behçet's disease.
Humans ; Interferon-gamma ; Lymphocytes ; Neopterin* ; Radioimmunoassay ; T-Lymphocytes

BACKGROUND: There has been no statistical study of dermatoses in the Ganganm area of Seoul which has been rapidly developing in the last 10 years. OBJECTIVE: We evaluated the recent distributions of dermatoses in the Gangam area of Seoul and compared them with the previous reports in Seoul and other provinces of Korea. METHODS: 47,015 new outpatients who visited the Yongdong Severance Hospital from 1983 to 1992 were analyzed statistically RESULTS: The study results are summarized as follows: 1. Among the 47,015 outpatients, the total number of number of male patients were 20,180(42.9%) and female patients 26,835(57.1%). 2. In age distribution, the most frequent age groups were the 3rd decade(21.3%), the lst decade(19.5%), and the 4th decade(19.2%) amounting to 60% of the total outpatients. 3. The most common 15 dermatoses were dermatophytosis, atopic dermatitis, urticaria, irritant contact dermatitis, acne vulgaris, allergic contact dermatitis, seborrheic dermatitis, other eczemas, warts, vitiligo, drug eruption, herpes zoster, psoriasis, bacterial infection, and syphilis. 4. Those dermatoses which showed an increasing tendency annually were seborrheic dermatitis, warts, vitiligo, and syphiiis. Dermatophytosis and bacterial infection showed a decreasing tendency. 5. Seasonal distribution showed that those diseases occurring most frequently during the summer were dermatophytosis, urticaria, allergic contact dermatitis, vitiligo, herpes zoster, and bacterial infection. The incidence of atopic dermatitis and psoriasis increased during the winter season. 6. In sexual distribution, acne, allergic contact dermatitis, and irritant contact dermatitis were more frequent in females, and the remainding common dermatoses did no show any difference. 7. In age distribution, atopic dermatitis and bacterial infection were most frequent in the lst decade. Urticaria, allergic contact dermatitis, acne, drung eruption, psoriasis, and syphilis were most frequent in the 3rd decade, with warts and vitiligo in the 2nd decade, and herpes zoster in the 6th decaded. 8. The distributions of dermotoses as disease groups were eczema(24.9%), urticaria-drug eruption (13.2%), dermatophytosis(10.7%), skin appendage disorder(10.1%), viral infection(10.1%), pigmentary disorders(4.5%), and papulosquamous diseases(4.0%) which constituted 78% of the total. 9. In patients dwelling in apartments, atopic dermatitis and molluscum contagiosumn showed a more frequent tendency in comparison to those living in house. 10. The most common 10 dermatoses among children were atopic dermatitis, urticaria, contact dermatitis, molluscum contagiosum, bacterial infection, dermatophytosis, vitiligo, other viral infections, and nevo-cellular nevi. On the other hand, the most common 10 dermatoses among the elderly were drmatophytosis, other eczemas, urticaria, herpes zoster, pruritus, contact dermatitis, drug eruption, lichen simplex chronicus, seborrheic dermatitis, and vitiligo. CONCLUSION: In contrast to earlier published report from Seoul, the infectous dermatoses, especially parasitic infestation, showed a decreasing tendency. The distribution pattern of the skin diseases did not differ fro other provinces of Korea in general.
Acne Vulgaris ; Age Distribution ; Aged ; Bacterial Infections ; Child ; Dermatitis, Allergic Contact ; Dermatitis, Atopic ; Dermatitis, Contact ; Dermatitis, Seborrheic ; Drug Eruptions ; Eczema ; Female ; Hand ; Herpes Zoster ; Humans ; Incidence ; Korea ; Male ; Molluscum Contagiosum ; Neurodermatitis ; Nevus ; Outpatients ; Pruritus ; Psoriasis ; Seasons ; Seoul* ; Skin ; Skin Diseases* ; Statistics as Topic* ; Syphilis ; Tinea ; Urticaria ; Vitiligo ; Warts

The incidence of atherosclerosis in Korea seems to be much increased due to diet change after national development. The pathogenesis of atherosclerosis is not clarified and there are many hypothesis but the most recent and reliable hypothesis is the ratio of HDL-cholesterol per total cholesterol. Under the basis of this hypothesis there have been much trials to administer the agents which has effect on lipid metabolism, so we tried pantethine on 30 patients who visited Han-Yang University Hospital Internal medicine Department, and the result as follows; 1) The mean age of study population was 50.1 years of age, mean body weight was 62kg, and mean height was 160.8cm. male was 17, and female 10. 2) The associated disease of study population was <19 with> cardiovascular disease, in 19, gastrointestinal disease 3, obesity in 1 and others in 4. 3) The undesirable effect of the drug was found on 3 patients; that is, constipation on 1 patient, dizziness on 1 patient, and skin eruption on 1 patient. 4) The mean serum lipids before and after pantethine administration(levels) are as following table. In conclusion, it seems that the effect of the drug which decreases the serum lipids is mild at initial but more increasing as the time goes by and constant, and we experience little side reaction except mild dizziness, constipation and skin eruption.
Atherosclerosis ; Body Weight ; Cardiovascular Diseases ; Cholesterol* ; Constipation ; Diet ; Dizziness ; Female ; Gastrointestinal Diseases ; Humans ; Incidence ; Internal Medicine ; Korea ; Lipid Metabolism ; Male ; Obesity ; Skin ; Triglycerides*