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

To evaluate the utility of gene rearrangement analysis, eight cases of malignant lymphoma, one case of Hodgkin's disease, two cases of angioiminunoblastic lymphadenopathy (AILD) and two cases of non-specific lymphadenitis were studied by immunohistochemical and genetic analysis. Southern blot analysis was perfon-ned by a using vacuum transfer system and a biotin labelled probe. This method was faster, safer, and more convenient than conventional methods. Gene rearrangement study showed rearranged novel bands in five of six cases of B cell lymphoma, in all cases of T cell lymphoma, and in all cases of AILD. No rearrangement of the B cell receptor(BCR) or of the T cell receptor(TCR) was seen in Hodgkin's disease or in nonspecific lymphadenitis. These results suggest that gene rearrangement analysis of BCR and TCR is a recommended method for the diagnosis of clonality in lymphoproliferative disorders. It would allow pathologists to differentiate lymphoma from polyclonal lymphoid proliferation and to provide information for cell lineage.

No abstract available.
Female ; Humans ; Neck*

No abstract available.
Humans

The cutaneous mucinose are a group of connective disorders characterized by the deposition of mucin in the dermis. Thc disease may be a primary (metabolic) or secondary (catabolic). Secondary mucinosis is common in lupus erythematosus and dermatomyositis but rare in morphea. We report a case of guttate morphea with secondary cutaneous mucinosis.
Dermatomyositis ; Dermis ; Dronabinol ; Mucinoses* ; Mucins ; Scleroderma, Localized*

The Dowling-Meara type of epidermolysis bullosa simplex(EBS) is genetic disease that is transmitted as an autosomal dominant trait and is charscterized clinically by pherpetiform clustering of blisters and palmo-plantar keratoderma. The disease usually presents at with or in early infancy. Although serious and hemorrhagic Wers may occur on any part of the body, the lesions heal without scaning in general. The disease shows a tendency to improve by progressian of age and it usually follows a relatively benign course. Microecopically, there are intraepidermal bli.ter s forming as a result of cytolysis of basal cells. In addition, the is a highly characteristic clumping of tonofilaments of keratinocytes in the lower epidermis, which is not seen in any other form of EBS. We report a case of Dowling-Meara type of EBS that is first destribed in Korean medical literatures.
Blister ; Epidermis ; Epidermolysis Bullosa Simplex* ; Epidermolysis Bullosa* ; Intermediate Filaments ; Keratinocytes

Allergic contact dermatitis is a prototype of delayed hypersensitivity reaction. Langerhans cells, keratinocytes and T lyrnphocytes play major roles in the pathogenesis of allergic contact dermatitis. We observed Langerhans cells and keratinocytes in contact sensitized epidermal sheets of mice and performed mixed epidermal cell lymphocytes reaction with normal epidermal cells and contact sensitized epidermal cells to determine the lymphocyte stimulating capacity of contact sensitized epidermal cell. We obtained following results : 1. The Langerhans cells were decreased in number and morphologically damaged in contact sensitized epidermis. 2. Ia antigen expression on keratinocytes was detected in almost all contact sensitized epidermal sheets. 3. The allogeneic lymphocyte stimulating capacity of contact sensitized epidermal cells was greater than that of normal epidermal cells. 4. The allogeneic lymphocyte stimulating capacity of contact sensitized epiderrnal cells was lost after treatment of epidermal cells with anti Ia antibody and complement. From these results, it is conceivable that the contact sensitized epidermal cells can amplify the immune reactions by stimulating the lynphocytes which are infiltrated in contact sensitized epidermis.
Animals ; Complement System Proteins ; Dermatitis, Allergic Contact ; Epidermis ; Histocompatibility Antigens Class II ; Hypersensitivity, Delayed ; Keratinocytes ; Langerhans Cells ; Lymphocytes ; Mice*

The physical conditions of the Korean and the Japanese young adults (aged 18-29) were analyzed for the 10 important body dimensions such as stature, body weight, sitting height, chest circumference, and etc.. The Korean data published in 1979 and 1992 were compared with the Japanese data published in 1984 and 1994, respectively. Also, the physical developments and the sex differences of the Korean in past 13 years were compared with those of Japanese. The results are as follows. 1) The stature, sitting height and perineum height of the Korean young male and female are significantly smaller than those of the Japanese. 2) In the past, the chest circumference, waist circumference, hip circumference, body weight of the Korean are larger values than those of the Japanese. But, there are no significant differences in recent surveys. 3) Most body dimensions of male show significantly larger than those of female in the two countries. 4) There is no significant difference in thigh circumference between the Korean male and female in 1992. 5) There are no significant differences in thigh circumference between the '84 Japanese male and female and in hip circumference between the '94 Japanese male and female. 6) Compared with the results of the past surveys, the relative body weight, perineum height, and chest circumference of female to male are decreased in the two countries. But, the relative hip circumference of female to male are increased in both countries.
Asian Continental Ancestry Group* ; Body Weight ; Female ; Hip ; Humans ; Male ; Perineum ; Sex Characteristics ; Thigh ; Thorax ; Waist Circumference ; Young Adult

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

BACKGROUND: Desmosomes are adhesive intercellular junctions that form an important component of the junction complexes of epithelial cells. They provide intercellular links between the intermediate filament cytoskeletons of adjacent cells and are thus involved in maintaining the structural integrity of tissues. OBJECTIVE: Calcium and retinoids are major regulators of epidermal differentiation and their role on keratin proteins are well known. However, their effects on desmosome moleucles are unknown. To address this question we initiated a study of the effects of these epidermal differentiation regulators on desmosomal components, i.e., desmoplakin, desmoglein, and pemphigus antigens. METHODS: We used monoclonal antibodies against desmoplakin(DP) and desmoglein(DG), and sera from patients with pemphigus vulgaris(PV), pemphigus foliaceus(PF) and paraneoplastic pemphigus (PNP) to study the effects of calcium and retinoic acids, which are major regulators of epidermal differentation, on desmosomal protein formation in human cultured deratinocytes. We performed immunofluorescence, immunoblotting and immunoprecipitation study using human keratinocytes cultured in high calcium media with or without retinoic acid and in low calcium media with or without retinoic acid. RESULTS: 1. In low calcium (0.15mM) media, PV antigen and DG were produced in a small amount and it appeared that these desmosomal proteins were located in cytosol. Whereas in high calcium (1.8mM) media, production of these desmosomal proteins was increased not they were assembled at the desmosomal structures located in cell-cell contact margins. 2. PF antigen, which was identical to the DG, were not produced or expressed in cultured keratinocytes even when cultured in high calcium media. 3. PNP antigen and DP were produced in cultured keratinocytes grown in both high low calcium media but their production was increased in high calcium media and only in high calcium media they were assembled at the desmosomal structures. 4. Retinoic acids induced loosening of cell-cell contacts of cultured keratinocytes and decreased the production of desmosomal proteins. CONCLUSION: Our results suggests calcium is a major regulator of the production and assembly of desmosomal proteins including pemphigus antigens, but PF sera and monoclonal antibodies against DG show different antigen binding characteristics. It appears that retinoic acids inhibit production of desmosomal proteins.
Adhesives ; Antibodies, Monoclonal ; Calcium* ; Cytoskeleton ; Cytosol ; Desmogleins ; Desmoplakins ; Desmosomes* ; Epithelial Cells ; Fluorescent Antibody Technique ; Humans ; Immunoblotting ; Immunoprecipitation ; Intercellular Junctions ; Intermediate Filaments ; Keratinocytes ; Pemphigus ; Retinoids ; Tretinoin*