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Biomedical subjects

R E Jordon

Publications and source records attributed to R E Jordon.

At least 91 records · Page 5Linked to original sources

C1q binding substances in pemphigus and bullous pemphigoid. Detection with a [131I] C1q binding assay.

A modification of the [125I]C1q binding assay was developed to allow the estimation of C1q binding activity (C1q BA) in pemphigus and bullous pemphigoid sera. The modifications include lower final concentration of PEG 6000 (1-5%) which permitted the use of sera that had been stored at -20 degrees C for extended periods of time; use of 131I instead of 125I and an [131I] C1q concentration of 5 microng/ml rather than 1 microng/ml. EDTA was used at a final concentration of 0-13 M to obviate the need for heat inactivation of sera. Sera from seventy-one patients with pemphigus and from 142 patients with bullous pemphigoid were tested for C1q BA. Of these 40% of the pemphigus and 20% of the bullous pemphigoid patients showed elevated C1q BA. A relationship between elevated C1q BA in serum and active disease was noted. Sequential samples from forty patients with pemphigus and thirty-seven patients with bullous pemphigoid demonstrated two different types of relationship between serum antibody titres to cutaneous antigens and C1a BA. In some patients serum antibody titres and C1q BA increased and decreased simultaneously; in others, increase of C1q BA followed increase of antibody titre and coincided with its decrease. The latter relationship supports the hypothesis that C1q BA may represent at least in part antigen-antibody complexes containing cutaneous antigens.

Antibodies↗

Late cutaneous reactions due to IgE antibodies.

IgE antibodies can produce a late inflammatory response 6--12 h after allergen challenge which is characterized by diffuse edema, erythema, pruritus, tenderness and heat. That IgE is involved in inducing the late reaction was shown by the abolition of both immediate and late responses by passive transfer tests: (1) by heating atopic serum at 56 degrees C for 4 h; (2) by removing IgE from the atopic serum by a solid phase anti-IgE immunoabsorbent, and (3) by competitively inhibiting the binding of IgE antibodies to cells by an IgE myeloma protein. Also, both responses were induced by affinity chromatography-purified IgE antibody followed by antigenic challenge. Very similar lesions could be induced by intradermal injection of Compound 48/80. The late phase is characterized by edema and a mixed cellular infiltration, predominantly lymphocytic but also containing eosinophils, neutrophils and basophils. Direct immunofluorescent staining did not show deposition of immunoglobulins or complement components, except IgM in two of 15 and C3 in one of 15 patients, respectively.

Antibodies↗

Oral pemphigus vulgaris. A report of ten cases.

The conditions of ten patients were diagnosed as pemphigus vulgaris limited to the oral cavity. In eight patients the histopathologic findings were consistent with oral pemphigus. Direct immunofluorescence demonstrated intercellular substance (ICS) deposition of C3 either alone or in combination with IgG, IgA, IgM, C4, C1q, properdin, and factor B in all nine patients tested. Circulating ICS antibodies were detected initially in eight of the ten patients, nine of whom had positive titers at a later date, without subsequent development of cutaneous lesions. Physicians should be alerted to a diagnosis of pemphigus in patients who have chronic erosive disease confined to the oral cavity. Prompt diagnosis by means of routine histopathologic and immunofluorescence studies followed by early initiation of treatment are warranted.

Adult↗

Bullous pemphigoid antigen: isolation from normal human skin.

A protein moiety from epidermal PBS-soluble products was isolated by gel filtration (Bio-Gel A-1.5m) and ion exchange chromatography (DEAE-cellulose). This protein (A-1-Epid) was not retarded by DEAE-cellulose in Tris-HCl buffer, 15mM, pH 8.1. By IEP against an antiserum to epidermal antigens, it showed a single cathodal arc. On disc electrophoresis, at low pH (4.3) a single band was apparent. On SDS gels this protein demonstrated two bands, one with a molecular weight of 20,000, and the second with a molecular weight of 9,200. This purified antigen was able to block the staining of the basement membrane zone produced by bullous pemphigoid antibodies on monkey esophagus and normal human skin with the use of indirect immunofluorescence. This study also demonstrates that bullous pemphigoid antigen (A-1-Epid) and a second epidermal protein (A-2-Epid) are present in the PBS-soluble products of human esophageal mucosa, saliva, and urine. These antigens appear to be unrelated with the blood group substances or secretor status of the donors.

Animals↗

PBS-soluble substances with blood group activity from human epidermis and dermis.

Phosphate-buffered saline (PBS) soluble extracts of human epidermis and dermis from secretor donors contain products with blood group antigenic activity. The predominant blood group in a sample was the same as the red cell phenotype of the donor. H substance activity was present in all PBS-soluble products (from A, B and O donors). By gel filtration chromatography on a Bio-Gel A-1.5 m column; the epidermal and dermal PBS-soluble blood group substances fractionate in the void volume.

Blood Group Antigens↗

Pemphigus-like antibodies in bullous pemphigoid.

A patient had clinical, histopathologic, and direct immunofluorescent findings compatible with a diagnosis of bullous pemphigoid. In addition, the patient's serum contained pemphigus-like antibodies with different antigenic specificities than true pemphigus antibodies. Absorption of the patient's serum with AB substance failed to block the intercellular substance staining but allowed the demonstration of a basement membrane zone reactive antibody as well. Atypical cases such as this should not cast doubt on the usefulness of immunofluorescence in the diagnosis of bullous skin diseases.

ABO Blood-Group System↗

Desquamative gingivitis. Clinical, histopathologic, and immunopathologic investigations.

Desquamative gingivitis is not a disease sui generis but is a descriptive term for a condition that requires thorough clinical evaluation and histopathologic and immunopathologic studies for accurate diagnosis. The present study emphasizes this statement. Six of seven patients with "desquamative gingivitis" were placed in a category of "possible cicatricial pemphigoid." During a 3-year observation period, five of six cases evolved into unequivocal cicatricial pemphigoid. Our studies lend credence to a hypothesis that immunopathologic mechanisms are operative in some forms of desquamative gingivitis.

Aged↗

Immunoglobulin and complement deposition in skin of rheumatoid arthritis and systemic lupus erythematosus patients.

Rheumatoid arthritis (RA) was differentiated from systemic lupus erythematosus (SLE) by direct immunofluorescent techniques on skin specimens, using monospecific antisera for IgG, IgM, C3, C1q, properdin, and fibrin. Of 30 patients with RA studied, 20 had dermal vessel deposits of immunoglobulins and complement components in unaffected skin without the characteristic dermal-epidermal junctional fluorescence of SLE. Of 24 SLE patients studied, 24 had granular deposits of immunoglobulins and complement components in unaffected skin at the dermal-epidermal junction.

Arthritis, Rheumatoid↗

The immunopathology of herpes gestationis. Immunofluorescence studies and characterization of "HG factor".

Nine skin biopsies from seven herpes gestationis patients were studied by immunofluorescence (IF) techniques. Basement membrane zone (BMZ) deposition of C3 and properdin was present in all nine skin specimens, while IgG deposition was apparent in only one. With in vitro C3 IF staining, positive BMZ staining (HG factor activity) was noted with all seven of our patients' serum samples tested. By standard indirect IF staining, however, only one of these serum samples contained BMZ antibodies of the IgG type. Two cord serum samples, tested by these same methods, yielded positive in vitro C3 staining (HG factor activity) but negative indirect IF staining (IgG). HG factor activity was found to be stable at 56 degrees C for 30 min and in two of three specimens at 56 degrees C for 1 h. Treatment of the complement source (normal human serum) used in the in vitro C3 staining assay with Mg2-EGTA or use of C2-deficient serum as the complement source inhibited HG factor activity. HG factor blocked the specific staining of the BMZ of normal human skin by labeled bullous pemphigoid antibodies. By sucrose density gradient ultracentrifugation and gel chromatography (Sephadex G-200), HG factor activity eluted with IgG-containing fractions. The highly purified IgG fraction of two herpes gestationis sera was also positive for HG factor activity. Our studies suggest that HG factor is an IgG antibody that may not be demonstrable by conventional IF methods, but which activates the classical complement pathway.

Basement Membrane↗

The late phase of the immediate wheal and flare skin reaction. Its dependence upon IgE antibodies.

IgE antibodies are usually thought to induce only immediate skin reactions. We have shown that the intradermal injection of a number of different allergens can produce a prolonged inflammatory reaction after the immediate wheal and flare in most sensitive subjects. This late inflammatory response occurs 6-12 h after challenge and is characterized by diffuse edema, erythema, pruritus, and heat. Both immediate and late responses can also be seen after passive sensitization of skin sites in nonatopic subjects. That IgE is involved in inducing the reaction was shown by the abolition of both immediate and late responses by passive transfer tests in the following experiments: (a) heating atopic serum at 56degreesC for 4 h, (b) removing IgE from the atopic serum by a solid phase anti-IgE immunoabsorbent, and (c) competitively inhibiting the binding of IgE antibodies to cells by an IgE myeloma protein. In addition, both responses were induced by affinity chromatography-purified IgE antibody, followed by antigenic challenge. Very similar lesions could also be induced by intradermal injection of Compound 48/80, thus suggesting a central role in the reaction for the mast cell or basophil. Histologically, the late phase is characterized by edema and a mixed cellular infiltration, predominantly lymphocytic but also containing eosinophils, neutrophils and basophils. Direct immunofluorescent staining did not show deposition of immunoglobulins or complement components, except IgM in 2 of 15 and C3 in 1 of 15 patients. This finding indicates that the late phase does not depend on the deposition of immune complexes. The results of the study suggest that IgE-allergen interaction on the surfaces of mast cells or on infiltrating basophils causes both immediate and late cutaneous responses.

Adult↗

Serum and blister fluid anticomplementary activity in pemphigus and bullous pemphigoid. Sucrose density gradient studies.

By sucrose density gradient ultracentrifugation and standard hemolytic complement assays, complement fixing activity of about 19 S and greater was found in blister fluids of one patient with pemphigus and three of five patients with bullous pemphigoid. Control blister fluids, including experimentally induced blisters, lacked such activity. Complement fixing activity was apparent, however, in 6 of 10 pemphigus sera and in two of five bullous pemphigoid sera tested. The material present in both pemphigus and bullous pemphigoid appears to activate the classical complement pathway.

Antigen-Antibody Complex↗

T and B lymphocytes in contact and atopic dermatitis.

The pattern of distribution of T and B lymphocytes was investigated in a group of patients with contact, atopic, and mixed dermatitis by means of immunofluorescence and T-cell rosette tests. A significant increase (P less than .001) in B lymphocytes with surface IgD but without changes in the lymphocytes bearing other immunoglobulins or C3 and T cells was noted in patients with contact dermatitis when compared with a control group. A significant increase (P less than .001) in B lymphocytes carrying IgE and a significant decrease (P less than .001) in the total numbers of T cells were noted in patients with atopic dermatitis, and a significant increase in B lymphocytes positive for IgD (P less than .001) and IgE (P less than .003) with a decrease in the number of lymphocytes bearing IgG were the most important changes observed in patients with mixed contact and atopic dermatitis.

Adolescent↗

Hypocomplementemic ear effusion in relapsing polychondritis.

In a case of relapsing polychondritis it was possible to aspirate a collection of subcutaneous fluid from the patient's involved ear. A determination of total hemolytic complement activity of this fluid was low, suggesting that activation of the complement system may have occurred in the course of the patient's disease and might be related to the pathogenesis of this disorder.

Complement System Proteins↗

Complement activation in bullous skin diseases.

Pemphigus, bullous pemphigoid, cicatricial pemphigoid, dermatitis herpetiformis, and herpes gestationis are members of the chronic vesiculobullous skin diseases of man. The complement system, including both the classical and alternative pathways, may be important in the pathogenesis of these diseases. In pemphigus, early complement components (C1, C4, and C2) appear to be activated in addition to later components (C3 and C5), suggestive of classical pathway activation. Participation of properdin in addition to early complement components suggests local activation of both complement pathways in bullous pemphigoid and cicatricial pemphigoid. Herpes gestationis and dermatitis herpetiformis may be bullous skin diseases entirely mediated by the alternate or properdin pathway. The specific immunopathologic findings in these diseases are discussed.

Antibody Formation↗

Dermal-epidermal deposition of complement components and properdin in systemic lupus erythematosus.

Skin lesions and clinically normal skin of thirteen patients with systemic lupus erythematosus (SLE) were examined, by the use of immunofluorescent techniques, to determine the presence of CIq, C3, C3 proactivator (C3PA), properdin, immunoglobulins (IgG, IgA, and IgM), and fibrin. IgM deposition was present in all thirteen skin lesions and in eleven normal areas tested, whereas IgG deposition occurred in eleven of the lesions but in only six normal areas. IgA was the least frequently encountered immunoglobulin. CIq deposition occurred in all thirteen skin lesions, and C3 deposition was present in twelve. Seven of nine and eight of eleven clinically normal areas demonstrated CIq and C3 deposition, respectively. Although less intense that CIq and C3 deposition, properdin deposition occurred in eight of the thirteen skin lesions tested but in only two of nine normal areas. C3PA deposition was of greater intensity than was properdin, but was seen in only five lesions and three clinically normal areas. Seven skin lesions and two normal areas also demonstrated fibrin deposition. Although alternate pathway activation is apparent, our findings suggest that the classical pathway (CI to C9) is the primary complement pathway involved in SLE skin.

Adult↗