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

Epidermolysis bullosa simplex (EBS) is a group of autosomal dominantly inherited genetic disorders characterized by blistering due to mechanical- stress-induced degeneration of basal epiderrnal cells. Recently, it was discovered that EBS is induced by keratin 5 and 14 gene mutations. Weber Cockayne (W-C) EBS is the mildest type, with blistering concentrates primarily on palar and plantar regions, and basal cell cytolysis by keratin filament perturbations is present. Herein we report a case of W-C EBS with its ultrastructural findings. Electron microscopy showed cytolysis and separation of the basal epidermal cells, mainly at the subnuclear cytoplasm. The cyto- plasm of basal cells showed edema, loosening and intact rnitochondria. Besides the cytoplasmic changes, the nucleus also showed lytic degeneration. Characteristically, dense condensation of tonofilarnent was observed, which suggests that W-C EBS is. also a disorder of keratin.
Blister ; Cytoplasm ; Edema ; Epidermolysis Bullosa Simplex* ; Epidermolysis Bullosa* ; Keratin-5 ; Microscopy, Electron

No abstract available.
Fentanyl ; Intensive Care Units ; Miliaria*

No abstract available.
Dermatitis, Contact*

Sclerosing lymphangitis of the penis is a peculiar disorder characterized by painless, firm, cord-like lesion in the coronary sulcus of the penis. Histologic findings include thickened lymphatic collecting vessels, fibrin thrombi and few inflammatory changes. The etiology is unknown, but the condition is benign and self-limited. We describe herein a 27-year-old man who had a typical painless, tender, firm, cord-like lesion in the coronary sulcus. Histologic findings disclosed a markedly thickened and fibrosed lymphatic vessel with a organizing thrombus. Whatever the cause of thrombi formation, it is prohable that the thrombi formation would be a main pathologic process, followed by the thickening of the vessel wall.
Adult ; Fibrin ; Humans ; Lymphangitis* ; Lymphatic Vessels ; Male ; Penis* ; Thrombosis

The third complementarity determining region (CDR3) of the immunoglobulin (Ig) kappa () chain is known to be located at the center of antigen binding groove and critical for antibody specificity. Ig chain has been characterized by limited junctional diversity due to the absence of N-region addition resulting in relative conservation of CDR3 lengths with 9 or 10 amino acids. CDR3 region of 11 amino acids is only possible with N-region addition. Recently, x transcripts with 11 amino acids CDR3 was found to be expressed in normal individuals, and in autoimrnune disease such as rheumatoid arthritis, the fraction of 11 amino acids CDR3 of humkv325-derived chains was overexpressed compared to conventional adult peripheral B cells. However, the significance of this bias is difficult to interpret without a clear understanding of normal repertoire of CDR3 length during development. The purpose of this study is to determine whether developmental regulation of CDR3 amino acids codon lengths exists in chains expressed in the fetal liver, cord blood, and adult peripheral blood lymphocytes (PBL). Lymphocytes were seperated from fetal liver, cord blood and adult PBL and cDNA was generated from extracted mRNA. PCR-based CDR3 finger- printing assay was performed with VI-IV family specific primers. CDR3 length diversity of Ig x chain increases as the development proceeds. The length diversity most frequently occured in Vlll family derived transcripts including 11 amino acids CDR3. transcripts with 11 amino acids CDR3 were consitently expressed in both fetal and adult Ig repertoire. These results support the hypothesis that v chain CDR3 length is developmentally regulated and implicates the diversity of antigen-antibody specificity generation.
Adult ; Amino Acids ; Antibody Specificity ; Arthritis, Rheumatoid ; B-Lymphocytes ; Bias (Epidemiology) ; Codon ; Complementarity Determining Regions ; DNA, Complementary ; Fetal Blood ; Humans* ; Immunoglobulin kappa-Chains* ; Immunoglobulins* ; Liver ; Lymphocytes ; RNA, Messenger ; Sensitivity and Specificity

No abstract available.
Actinomycosis*

No abstract available.
Neurothekeoma* ; Scalp

Panniculitis, Lupus Erythematosus*

It is evident that an elevation of airway albumin excreation rate without clinical proteinuria strongly predicts a later progression on diabetic renal disease. So we studied the correlation between Microalbumin checkly RIA & Micral-Test®. We collected urine between 08:00 h and 08:00 h next day and then checked microalbuminuria by radioimmunoassay method and Micral-Test® The results are as follows: 1. There was significant correlation between microalbuminuria checked by RIA & Micral-Test® 2. There was poor correlation between diabetes duration or HV-A1c and maximal change in albumin excreation rate. 3. So we concluded that Micral-Test® can be used in laboratory instead of RIA.
Methods ; Proteinuria ; Radioimmunoassay