BACKGROUND: Bullous pemphigoid is a rare autoimmune bullous disease characterized by subepidermal bulla. The serum of these patients contains detectable autoantibodies which bind to the hemidesmosome of the basement membrane zone. It is well known that there are two bullous pemphigoid antigen molecules, 230KD and 170KD protein. Serum studies in Caucasian patients show that 70-80% of the patients recognize 230KD antigen while 10-30% recognize 170KD antigen, In contrast, in herpes gestationis, which is an autommune-mediated bullous disease of pregnancy, 90% of the patients recognize 170KD and 10% recognize 230KD antigen, The autoantibody of herpes gestationis(HG factor) has a strong complement binding capacity and it may share the same epitope as the antibody of bullous pemphigoid patients which recognize 170KD antigen. OBJECTIVE: The purpose of this study was to characterize the clinical and histological manifestations of Korean patients with bullous pemphigoid and to characterize the autoantibodies of Korean bullous pemphigoid patients by immunoblotting. We also wished to compare the characteristics of the antibodies with that of American bullous pemphigoid patients, and to elucidate the hypothesis that the bullous pemphigoid autoantibody against 170KD protein has the same strong complement binding capacity as the herpes gestationis autoantibody. MEHTODS: We investigated the clinical and histological characteristics of 9 Korean patients and also performed a complement binding capacity and immunoblotting study on the sera of 9 Korean patients and 16 American patients. RESULTS: 1. Korean bullous pemphigoid patients clinically showed polymorphic skin eruption in addition to tense bullae. They frequently showed pruritic erythematous patches and urticarial plaques. Histologically, infiltration of subepidermal bullae with eosinophils, neutrophils and lymphocytes were observed in all patient's specimen, in which eosinophils were the most predominant cells, Uncommonly, eosinophilic spongiosis, vaculoar degeneration of basal cells were observed. These observations did not have any particular characteristics or racial differences compared to those patients reported in Western literature. 2. In the immunoblotting study of Korean bullous pemphigoid patients, 7 of 9 sera(785) recognized 230KD antigen and, also, 7 of 9 sera(78%)recognized 170KD antigen, In contrast, 15 of 16 American patients sera (94%) recognized 230KD antigen and 6 of 16 patients sera(38%) recognized 170KD antigen. The high incidence of the autoantibody against 170KD in Korean patients shows possible racial differences in autoantibody formation. 3. There was no relationship between the types of autoantibodies typed by immunoblotting and the complement binding capacity. In other words, autoantibodies against 170KD antigen do not carry the same potential as autoantibodies of herpes gestationis for the complement biding capacity, CONCLUSION: The above results suggest tha there may be racial difference in bullous pemphigoid autoantibodies between Korean bullous pemphigoid patients and American patients. In conclusion, We conclude that 170KD bullous pemphigoid antibodies do not always have the same strong complement binding as herpes gestationis antibody.
Antibodies ; Autoantibodies ; Basement Membrane ; Complement System Proteins* ; Eosinophils ; Female ; Hemidesmosomes ; Humans ; Immunoblotting* ; Incidence ; Lymphocytes ; Neutrophils ; Pemphigoid Gestationis ; Pemphigoid, Bullous ; Pregnancy ; Skin ; Transcutaneous Electric Nerve Stimulation

No abstract available.
Neurology*

The R-R interval analyzer was developed to measure the autonomic nervous system function using microcomputer. The system based on 8 bit microcomputer including bandpass filter, R-wave detector and clock generator in order to obtain the mean value, standard deviation, total time, CV value, maximum value and minimum value in the specific view point of R-R interval variation. The pattern of R-R interval change after resting, voluntary standing and deep breathing can be analyzed in normal subjects and diabetics with autonomic nervous dysfunction. The amplitude of the R-R interval variation showed sensitive pattern for normal subjects at resting, standing and deep breathing. On the contrary, the periodicities of amplitude for abnormal subjects with autonomic nervous dysfunction showed dull pattern. It was suggested that R-R interval analyzer is a good detection method for dysfunction of autonomic nervous system.
Autonomic Nervous System ; Methods ; Microcomputers* ; Periodicity ; Respiration

No abstract available.
Gastrectomy*

The authors present a case of spontaneous discitis in a 1-year-old female who presented with fever and limping gait. The erythrocyte sediment rate was increased. The roentgenograms revealed normal appearnce, and radioactive bne scan showed increased uptake at the affected level. Discitis should be considered in any child with limping gait or leg pain, refusal to walk, or abdominal pain. Early recognition may avoid unnecessary diagnotic and treatment procedures.
Abdominal Pain ; Child* ; Discitis* ; Disulfiram ; Erythrocytes ; Female ; Fever ; Gait ; Humans ; Leg

The author attempted to allelic association between the a1 allele of Dopamine D2 receptor and alcoholism in Korean. The allelic disribution of Taq I polymorphism of the D2 dopamine receptor gene with alcoholism was examined in 67 Korean alcoholics and compared with 100 Korean controls. In alcoholics, the numbers of alcoholics with A1A1, A1A2 and A2A2 were 11(16.4%), 30(44.8%) and 26(38.8%) respectively and in control with A1A1, A1A2 and A2A2 were 17(17.0%), 42(42.0%), respectively. The prevalence of the A1 allele in alcoholics was 61.2% and 59.0% in controls. And the frequency of the A1 allele in alcoholics and controls were 0.39 and 0.38, respectively. There was not significant difference in the frequency of the A1 allele between alcoholics and controls. This data suggest that the A1 allele is not associated with alcoholism in Koreans. The author conclude that our data do not support on allelic association between the A1 allele at Dopamine D2 receptor and alcoholism. Further systemized studies will be necessary to determine whether the role of allele of Dopamine D2 receptor is major effect gene or modifying effect gene in the pathogenesis of alcoholism.
Alcoholics* ; Alcoholism ; Alleles ; Dopamine* ; Humans ; Prevalence ; Receptors, Dopamine ; Receptors, Dopamine D2

Aplasia cutie congenita is a rare congenital disease showing focal abscence of skin. In the msjority of instances it is limited ta the scalp especially on the vertex although other areas of the body may also be involved. Other congenital malformation have been reported to ccur with ACC odcasionally. Diagnosis depends on primarily clinical findings. The histologic finding virious from an absence of appendages to the involvement of varying layers of skin, occasionally extending to the bane or dura. Healed lesions often demonstrate a flattened epidermis, a proliferation of fibrobasts in a loose connective tissue stroma, newly formed capillaries, and a complete absence of adnexa stuctures. We observed a newborn infant with aplssia cutis congenita. The akir, defect was found on the posterior fontanelle. No associating cangenital anomalies were found in this case.
Capillaries ; Connective Tissue ; Cranial Fontanelles ; Diagnosis ; Ectodermal Dysplasia* ; Epidermis ; Humans ; Infant, Newborn ; Scalp ; Skin

No abstract available.

Phakomatosis pigmentovascularis is characterized by the coexistence of pigmentary nevus and hemangioma. Since the term was first described by Ota et al in 1947, about 80 cases have been reported in the literature. In 1979, Hasegawa et al subclsssified the disorder into eight types : type I, nevus flammeus and nevus pigmentosus et verrucosus, type II, nevus flamme is and dermal melanocytosis, type III nevus flammeus and nev.us spilus, type IV, nevus flammeus, dermal melanocytosis and nevus spilus. Each type is subdivided into localized(a) and systemic(b) types, A 25-year-old male had brown papules on the left knee and thigh and purple patches on the left leg that had been present for approximately 7 years. He had no concon ittant systemic disease. The histopathologic findings on brown papules and purple patches are compatible with epidermal nevus and nevus flammeus respectively. We herein report a case of phakomatosis pigmentovascularis type I a.
Adult ; Down Syndrome ; Hemangioma ; Humans ; Knee ; Leg ; Male ; Neurocutaneous Syndromes* ; Nevus ; Port-Wine Stain ; Thigh

Cytomegalovirus (CMV) is a major cause of morbidity and mortality in immunocompromised patients. CMV can cause pneumonia, retinitis, gastrointestinal ulcers, and widely disseminated disease, but cutaneous CMV is rare. We report two cases of cutaneous CMV infection presenting as perianal ulcers. A 54 year-old male who had liver transplantation and a 72-year-old male, who was treated with chemotherapy for angioimmunoblastic T-cell lymphoma, presented with perianal ulcers and had systemic symptoms of CMV infection with CMV antigenemia. They had multiple ulcerations with erythematous bases on the perianal area, and histopathologic examinations showed large atypical cytomegalic cells in the dermis, and immunohistochemical stains, with the anti-CMV antibody showed positive reactions.
Aged ; Coloring Agents ; Cytomegalovirus Infections* ; Cytomegalovirus* ; Dermis ; Drug Therapy ; Humans ; Immunocompromised Host* ; Liver Transplantation ; Lymphoma, T-Cell ; Male ; Mortality ; Pneumonia ; Retinitis ; Ulcer