Epidermolysis bullosa acquisita (EBA) is an autoimmune blistering disease of the skin occurring mostly in middle-aged persons with characteristic skin lesions of inflammatory in a vesiculobullae and mechanobullous lesions. Separation of the skin occurs at the dermoepidermal junction (DEJ) initiated by an immune process involving the anchoring fibrils (AF) 764, which have a role in the normal adherence of the epidermis and the dermis. Patients with EBA have autoantibodies of IgG to type VII collagen which is the main component of AF. An electron microscopic. picture of normal DEJ is shown in figure 1, and the antigen site 2011s of this disease (AF) is noted at the upper-most part of the dermis. In EBA, a biopsy specimen shows subepidermal bulla with a variable degree of dermal in-filtrates. Immunofluorescence (IF) demonstrates a linear deposit of IgG. The pattern of immune deposits along the DEJ is similar to that of bullous pemphigoid. However, the linear iing. fashion is thicker and coarser. When examined by the indirect method with a semi-horizontal section of normal human skin substrates the same patterns can be observed: a fine linear deposit with bullous pemphigoid antibodies and a slightly coarser linear pattern with EBA antibodies. With salt-split skin substrates, the serum autoantibodies of IgG are found to be bound only to the dermal side, the AF zone (Fig. 2). This immunopathologic study can provide a diagnostic finding. Transmission electron microscopic examination reveals blister to be localized just beneath the lamina densa, the site of the immune deposit. In immunoblot analysis of the patient's serum against the. dermal extracts, serum antibodies are found to recognize type VII collagen of 290/145 kD (Fig. 3). This is a confirmatory technique (with antibody-positive sera) in the diagnosis of EBA.
Antibodies ; Autoantibodies ; Biopsy ; Blister ; Collagen Type VII ; Dermis ; Diagnosis ; Epidermis ; Epidermolysis Bullosa Acquisita* ; Epidermolysis Bullosa* ; Fluorescent Antibody Technique ; Humans ; Immunoglobulin G ; Methods ; Pemphigoid, Bullous ; Skin

Skin lesions of erythema nodosum leprosum(ENL) occurs as crops of erythematous papules with microscopical features of vasculitis, and these are considered to he immune complex mediated. Recent studies by several investigators detected high levels of circulating immune complexes in over fifty percent of patients with ENL, using well known Clq binding assay or monoclonal rheumatoid factor assay. Moreover they found immuoglobulins (Ig) and complement components in the blood vessels of these patients. These immunopathologic data presented more support to the immune complex mediated pathogenesis of the skin lesions in ENL spectrum. In connection with these findings, examinations of the skin lesions of Korean patient with ENL by immunofluorescent techniques were done. Among seven biopsy specimens from each seven patients, four revealed deposits of Igs (G and M) along with CR in dermal blood vesseIs. Alpha-1 antitrypsin and fibrin were also found in some patients.
Antigen-Antibody Complex ; Biopsy ; Blood Vessels ; Complement System Proteins ; Erythema Nodosum* ; Erythema* ; Fibrin ; Humans ; Research Personnel ; Rheumatoid Factor ; Skin ; Vasculitis

Due to the limited availability of immunoelectron microscopy, an alternative method for the differentiation between anti-lamina lucida and anti-sublamina densa antibodies was introduced; indirect immunofluorescence using NaCl-treated human skin as the substrate. In this study author examined sera and lesional skin of 4 cases of bullous pemphigoid (BP), and 2 cases of epidermolysis bulloaa acquisita(EBA) with the above mentioned indirect imrnunofluorescence and modified direct immunofluorescence to evaluate the specificity of the tests. The results showed that in BP the fluorescence patterns were epidermal in 3 patients with 1 combined by indirect immunofluorescence, and epidermal in all 4 patients by modified direct immunofluorescence. In ERA the fluorescence were dermal patterns in both 2 patients by indirect and modified direct immunofluorescence. These data are further confirming the syecificity and the reproducibility of the NaCl extraction technique for the irnmunofluorescence to differentiate the localization of the autoantibodies in the above two bullous dermatoses.
Antibodies ; Autoantibodies ; Epidermolysis Bullosa Acquisita* ; Epidermolysis Bullosa* ; Fluorescence ; Fluorescent Antibody Technique ; Fluorescent Antibody Technique, Direct ; Fluorescent Antibody Technique, Indirect ; Humans ; Microscopy, Immunoelectron ; Pemphigoid, Bullous* ; Sensitivity and Specificity ; Skin ; Skin Diseases, Vesiculobullous

Immunologic or immunopathologic assays are neccesary for the diagnosis of autoimmune bullous dermatoses including pemphigus vulgaris (PV), bullous pemphigoid (BP), and epidermolysis bullosa acquisita (EBA). The objectives of this study is to compare the sensitivity and usefulness of indirect immunofluorescence 0F) with that of immunoblot assay using amplified alkaline phosphatase staining system in the diagnosis of the above diseases; detection of disease-specific IgG autoantibodies. We selected 4 patients in each bullous dermatosis of PV, BP, and EBA, who had serum levels of IgG autoantibodies at a titer of 1:80 or higher. In each three disease, 2 patients with negative serum antibodies or serum titer lower than 1:20, were also enrolled. Among the former 4-patient groups the titers of IgG antibodies found on indirect IF were in the range of 1:80 to 1:160, whereas the titers recognized by immunoblot assay were 1 or 2 dilutions higher in most of these patients. In the latter 2-patient groups, 4 out of the 6 cases revealed antibody-positive on immunoblot-staining membrane. The indirect IF can be performed easily and seems favorable in the aspect of cost-effectiveness. However, immunoblot assay with sensitive staining method would be warranted in cases of antibody-negative or atypical clinical variants of autoimmunebullous dermatoses to confirm the diagnosis.
Alkaline Phosphatase ; Antibodies ; Autoantibodies ; Diagnosis ; Epidermolysis Bullosa Acquisita ; Fluorescent Antibody Technique, Indirect ; Humans ; Immunoglobulin G ; Membranes ; Pemphigoid, Bullous ; Pemphigus ; Skin Diseases ; Skin Diseases, Vesiculobullous*

The exact pathomechanism of anti-epidermal cell pemphigus antibodies in developing acantholytic changes is unknown. Recent investigations have suggested that pemphigus antibodies, after binding to the antigenic site, induce activation of epidermal plasminogen activator. This increased activity of the plasminogen activator converts plasminogen to plasmin in high level degrades intercellular bridges resulting in loss of adhesion between epidermal cells. Author examined, by modified direct immunofluorescence, the deposition of plasmin and its inhibitor proteins such as alpha 1-antitrypsin and alpha 2-macroglobulin, with the early lesional skin specimens from 5 patients of pemphigus All these lesional skin demonstrated intense deposits of plasmin and aIpha 2-mscrogIobulin, and to a less degree alpha l-antitrypsin, all having indentical patterns to that of IgGautoantibodies. These proteins were also stained at the dermoepidermal junction and upper dermis, but less intensely. The identification of these particular proteins ; plasmin, alpha 1 antitrypsin, and alpha 2 macroglobulin, could be an alternate mean for the enzyme-histologic diagncsis of pemphigus.
alpha 1-Antitrypsin ; alpha-Macroglobulins ; Antibodies ; Dermis ; Fibrinolysin* ; Fluorescent Antibody Technique, Direct ; Humans ; Pemphigus* ; Plasminogen ; Plasminogen Activators ; Skin*

Ten serum samples from the patient with bullous pemphigoid with the baseruent mernbrane zone autoantibody titers of 320 or greater were tested, by the method of in vitro complement immunofluorescence, for their ability to fix Factor B and properdin in addition to Clq, C4 and C3. Five samples yielded positive C3 and properdin staining reaciions while four samples demonstrated positive Factor B stainings. All ten samples yieled positive C3, C4 and Clq staining reactions, Heat inactivation or treatment of the complement source with EDTA, MG2-EGTA abolished both C3, properdin and Factor B staining in all ten cases. This result suggest that pemphigoid antibody will fix properdin and Factor B in addition to Clq, C4 and C3, a phenomenon explained by assembly of the C3b amplification mechanism following activation of the classical pathway of complement system.
Complement Factor B* ; Complement System Proteins ; Edetic Acid ; Fluorescent Antibody Technique ; Hot Temperature ; Humans ; Pemphigoid, Bullous* ; Properdin*

We describe four patients with herpes-associated erythema multiforme, who had clinical and histopathological features consistent with the so-called erythema multiforme minor. The appearance of lesions of herpes simplex were followed with intervals between 6 and 10 days by the recurrent developments of erythema multiforme several times in a year in each patient. Sinee herpes simplex virus infection is considered one of the major factors provocating erythema multiforme one may have to pay more attention to patient's history to define the nature of erythema multiforme.
Erythema Multiforme* ; Erythema* ; Herpes Simplex ; Humans ; Simplexvirus

No Abstract available.
Dermatomyositis* ; Erythema*

New born rat heart cells were dissociated using trypsin and/or collagenase to elucidate the dissociation efficiency of these two enzymes. And the effect of dimethyl sulfoxide during and immediately after cell dissociation was also investigated to clarify the so-called protective activity of dimethyl sulfoxide on cell performance. The results can be summarized as follows. 1. Cold trypsin 18 hours pretreatment followed by warm collagenase treatment resulted best cell viability and cell yield. 2. Single warm trypsin treatment gave the poorest result. 3. Dimethyl sulfoxide did not seem to play any protective role during or immediately after rat heart cell dissociation. It had very damaging effect on rat heart cells.
Animals ; Cell Survival ; Collagenases* ; Dimethyl Sulfoxide* ; Heart* ; Rats* ; Trypsin*

Acromelanosis Progressiva which was described by Mishirna et al. In 1962 is a very rare pigmentary disorder and it seems that this is the first case reporting in Korean literature. The patient we observed was 11 years old girl having dark brown macules on the dorsa of her hands and feet, forearms, lower legs and face. These pigmented macules at first appeared on the dorsal surfaces of her fingers and toes when her age of 7. Thereafter these macules spread somewhat progressively to the proximal portions of the extremities and recently those have been spread upon her forearms and lower legs. The same pigmented macules appeared meanwhile on her face when her age of 9 and also have been spread diffusely on the cheeks and eyelids at visiting. Sh was otherwise normal on physically and had no familial history of such pigmentary disorder. But she had mental deficiency (I.Q. 60).
Cheek ; Child ; Extremities ; Eyelids ; Female ; Fingers ; Foot ; Forearm ; Hand ; Humans ; Intellectual Disability ; Leg ; Toes