BACKGROUND AND OBJECTIVES: Pemphigus is an autoimmune bullous disease of the skin and mucous membranes. There are two major types of pemphigus, namely pemphigus vulgaris(PV) and pemphigus foliaceus(PF) which can be classified by the specificity of the autoantibodies against the epidermal desmosomal antigens in this disease. Like many other autoimmune diseases, pemphigus is also considered to be strongly associated with certain HLA alleles; some alleles can be detected with higher frequencies as compared with those found in ethnically matched populations. At this time, we tried to find out if there were certain HLA class II allele(s) associated significantly with Korean patients of pemphigus. PATIENTS AND METHODS: Thirty patients with pemphigus (fifteen of PV and fifteen of PF), and one hundred healthy Korean controls were enrolled in this study. For the genotyping of HLA class II alleles in DRB1 loci, genomic DNAs prepared from buccal epithelia were amplified by polymerase chain reactions with nucleotide sequence-specific primers. Each allele of thirteen different generic types belonging to the DRB1 loci were used to identify the existence of each allele in both patient and control groups on gel electrophoreses. RESULTS: In PV, there was a significantly increased frequency of HLA-DRB1*01 alleles than from the findings observed in the controls(pc=0.0013, RR:5). In patients with PF, there was a significant degree of association with HLA-DRB1*01(pc=0.00013, RR:5.5) when compared with that in normal controls. However, no allele of negative association with a significantly low frequency in the patient group was detected in both types of the disease. CONCLUSION: It can be suggested that DRB1*01 alleles may be susceptibility genes in Korean patients with PV, and DRB1*01 alleles could contribute to the autoimmune reactivity in patients with PF. This data shows different patterns in the frequency of each DRB1 allele in patient groups compared with those found in patients of other ethnic backgrounds.
Alleles* ; Autoantibodies ; Autoimmune Diseases ; DNA ; Electrophoresis ; HLA-DRB1 Chains* ; Humans ; Mucous Membrane ; Pemphigus* ; Polymerase Chain Reaction ; Sensitivity and Specificity ; Skin

We report the result of surgical removal of subfoveal choroidal neovascular membranes[CNVM] in patients older than 50 years. Eleven eyes treated consecutively by submacular surgery were included. Etiology of CNVM was age-related macular degeneration in 7 eyes, myopia in 2 eyes, and idiopathic in 2 eyes. All the eyes had vision of 20/200 or worse and had undergone surgeries within 3 months from the onset of visual symptom. After creation of posterior vitreous detachment, neovascular membranes were completely dissected and removed through 36Gsize retinotomies. No laser photocoagulation was placed to the retinotomies, and air bubble of 30% volume of vitreous cavity was left for tamponade at the end of the surgery. Within one month postoperatively, fluorescein angiogram was obtained and confirmed the absence of residual CNVM in all cases. One eye developed a recurrent CNVM 6 months following surgery and was lasered successfully. At the mean follow-up period of 6.4 months, visual acuity was improved in 6 eyes[55%], unchanged in 4 eyes[36%], and worsened in 1 eye[9%] which suffered from diffuse RPE loss during surgery. Although the visual outcome of submacular surgery is expected to be poor in older patients, surgical approach may be beneficial in carefully selected cases with CNVM for improvement or preservation of vision.
Choroid ; Fluorescein ; Follow-Up Studies ; Humans ; Light Coagulation ; Macular Degeneration ; Membranes* ; Myopia ; Visual Acuity ; Vitreous Detachment

Zika virus was first isolated in from nonhuman primate in 1947. It is in the genus Flavivirus, closely related to other flavivirus like Dengue, West Nile, Yellow fever and Japanese encephalitis virus. Since 2007 epidemic in Yap island, zika virus infections had spread to the countries in Micronesia and South Pacific. In 2015, Zika virus outbreak occurred in Brazil and now more than 40 countries in American continents reported autochthonous infection. The virus is transmitted mainly by Ae. aegypti mosquito with many other Aedes mosquito species known as vector. Recently, Zika virus infection is known to cause severe neurological complications and congenital malformation. In this paper, we will review current knowledge on Zika virus history, biology, clinical characteristics and preventive method.
Aedes ; Biology ; Brazil ; Culicidae ; Dengue ; Encephalitis Virus, Japanese ; Flavivirus ; Methods ; Microcephaly ; Micronesia ; Primates ; Yellow Fever ; Zika Virus Infection* ; Zika Virus*

To examine the causative agents, clinical characteristics, management, risk factors, and neurochemical mechanism of the antidepressant-associated mania, MEDLINE searches were conducted. Mania can occur by chance during antideressant treatment or withdrawal, particularly in patients predisposed to mood disorder. Antidepressant-associated mania, especially withdrawal mania, appears to be milder and a more time-limited syndrome than a spontaneous mania and may represent a distinct clinical entity. MAOI, especially RIMA or bupropion may be associated with milder and less manic inductions than either TCA or SSRI. The possible risk factors for antidepressant-induced mania are female, mood disorder, especially bipolar type I, past and family history of mood disorder, especially bipolar type I, long-term treatment, high dose, and combined therapy in treatment-resistant depression, the possible for withdrawal mania are female, mood disorder, especially major depressive disorder, past and family history of mood disorder, especially major depressive disorder, long-term treatment, high dose, abrupt discontinuation or dose reduction, TCA or MAO(except RIMA?). Antidepressant-induced mania can result from dysfunction of mechanisms that maintain noradrenaline/acetylcholine balance associated with the antidepressant-induced activation of noradrenaline system. The mechanism of withdrawal mania with TCA is cholinergic-monoaminergic interaction theory, and with MAOI is related a hyperdopaminergic state due to loss of drug-induced subsensitivity of dopamine autoreceptors. The prevention of these side effects will require further well-designed study on risk factors.
Autoreceptors ; Bipolar Disorder* ; Bupropion ; Depression ; Depressive Disorder, Major ; Dopamine ; Female ; Humans ; Mood Disorders ; Norepinephrine ; Prevalence ; Risk Factors ; Risk Management

We report a case of infantile acute hemorrhagic edema in a 15-month-old boy. The patient showed multiple, bean to walnut-sized, red to purple-colored, indurated purpura with a cockade pattern on the face, earlobes, and extremities. Edema of the hands, feet, and earlobes was also observed. Laboratory examinations showed thrombocytosis, decreased serum CH50, and ketone in the urine. A skin biopsy taken from indurated purpura on the lower leg exhibited findings of acute leukocytoclastic vasculitis. Direct immunofluorescence examination showed deposition of IgM along the dermo-epidermal junction and C3 in the dermal vessel walls. With-out specific management except for fluid therapy to correct dehydration, the skin lesions cleared completely in 2 weeks, and recurrence has not observed.
Biopsy ; Dehydration ; Edema* ; Extremities ; Fluid Therapy ; Fluorescent Antibody Technique, Direct ; Foot ; Hand ; Humans ; Immunoglobulin M ; Infant ; Leg ; Male ; Purpura ; Recurrence ; Skin ; Thrombocytosis ; Vasculitis

A case of multiple hamartomas of the unilateral chest wall in a four month old infant is presented. There have been a few reports on the CT findings of the chest wall hamartoma in infancy. We describe bone changes of the ribs and mineralization of this rare tumor on the CT scan, and the locations of two separate masses.
Hamartoma ; Humans ; Infant ; Miners ; Ribs ; Thoracic Wall* ; Thorax* ; Tomography, X-Ray Computed

Blue rubber bleb nevus syndrome is a rare disease of cutaneous hemangioma of a variant of the carvenous type which is clinically characterized by multiple, protuberant, dark blue, generally soft, rubbery, and compressible cutaneous masses usually associated with similar lesions of the gastrointestinal tract. Most cases are sporadic, although autosomal dominant inheritance has been described. We report herein a cases of blue rubber bleb nevus syndrome in a 49-year-old, who had multiple, dark blue, slightly tender, easily compressible vascular tumors on the face, right postauricular area, tongue, neck, and right had and foot, measuring from 0.5 to 1.0 cm in diameter. Endoscopic examination also revealed several similar lesions on the upper third of the esophagus. Biopsy specimen from the lesion of the neck showed irregularly dilated vessels containing red blood cells and fibrinous material. The vascular space was lined by a single layer of flattened endothelial cells.
Biopsy ; Blister* ; Endothelial Cells ; Erythrocytes ; Esophagus ; Fibrin ; Foot ; Gastrointestinal Tract ; Hemangioma ; Humans ; Middle Aged ; Neck ; Nevus* ; Rare Diseases ; Rubber* ; Tongue ; Wills

Pars plana vitrectomy with encircling scleral buckling was performed for 30 eyes of 30 patients with complicated retinal disorders, and the changes of axial length, refractive power, and corneal curvature were analyzed. Significant axial length elongations were observed after encircling procedures. Mean elongation was 0.93+/-0.48mm after 3 months and 0.91+/-0.54mm after 6 months. These spherical equivalent of refraction showed myopic shift of -2.26+/-1.51 diopters(D) after 3 months and -2.19+/-1.39D after 6 months. These changes seem to be less prominent in the eyes with preoperative axial length of 24.50mm or longer(0.68+/-0.46mm after 3 months, 0.60+/-0.49mm after 6 months) than in the eyes with axial length of 24.49mm or shorter(1.18+/-0.35mm 1.23+/-0.38mm). Slight increase in astigmatism was observed, 0.80+/-1.35D after 3 months and 0.64+/-1.01D after 6 months. The mean keratometric readings showed no significant changes, 0.00+/-0.95D after 3 months and 0.26+/-0.95D after 6 months. The result of this study may give an important information when combined procedure of encircling scleral buckling and IOL implantation be planned.
Astigmatism ; Humans ; Reading ; Retinaldehyde ; Scleral Buckling* ; Vitrectomy

Zinc is essential for the survival of all cells. Retinal pigment epithelium(RPE)and chorioretinal complex contains unusually high concentration of zinc. Recently, zinc has received special attention of clinicians for its possible association with certain ocular diseases such as age-related macular degeneration(ARMD). However, the cellular and molecular pathological correlates of zinc deficiency in retinal cells and RPE are scanty. Thus, we examined zinc deficiency-induced RPE pathology using a cell-permeant zinc chelator, TPEN. Exposure of human RPE cultures to TPEN induced death of RPE between 1 micrometer to 2 micrometer concentrations within 48h exposure. TPEN-induced cell death was completely blocked by coaddition of zinc. Addition of a broad-spectrum caspase inhibitor, zVAD-fmk and a caspase 3 inhibitor, DEVD-fmk markedly attenuated TPEN induced RPE death.Addition of a protein synthesis inhibitor cycloheximide attenuated TPEN-induced RPE death significantly. On the other hand, EGF, IGF-1, PDGF, MK-801, or trolox did not show any protective effect. Additionally, TUNEL and Hoechst staining revealed conspicuous internucleosomal DNA fragmentation. Furthermore, ultrastructural changes supported that TPEN-induced RPE death was apoptosis. The present study using human RPE cells provides a model for zinc depletion-induced apoptosis. Considering the clinical importance of ARMD, the model may provide useful insights into the pathogenic mechanisms of zinc deficiency-related degenerative eye conditions.
Apoptosis* ; Caspase 3 ; Cell Death ; Cycloheximide ; Dizocilpine Maleate ; DNA Fragmentation ; Epidermal Growth Factor ; Epithelial Cells* ; Hand ; Humans* ; In Situ Nick-End Labeling ; Insulin-Like Growth Factor I ; Pathology ; Retinal Pigment Epithelium ; Retinaldehyde* ; Zinc*

Weber-Christian disease is an inflammatory disease of subcutneious fat tissue which is clinically characterized by relapsing, febrile, nodular, nonsuppurative pannicutilitiy. The cause is unknown, but the disease has been attributed to an autoimmune reaction, infectioris, halogen compounds, sarcoidosis, SLE and a-antitrypsin deficiency. We report here in a case of Weber-Christian disease in a 8-year-old girl, who had indurated erythemataus subcutaneous nodules on the extremities, buttock and trunk accompanied by various general symptoms such as fever, malaise, arthralgia and edema of the abclorren and legs. This case was successfully treated with dapsone,
Arthralgia ; Buttocks ; Child* ; Dapsone ; Edema ; Extremities ; Female ; Fever ; Humans ; Leg ; Panniculitis, Nodular Nonsuppurative* ; Sarcoidosis