PubMed HealthSearch

Biomedical subjects

G Tappeiner

Publications and source records attributed to G Tappeiner.

16 recordsLinked to original sources

[Lupus erythematosus panniculitis (author's transl)].

Lupus erythematosus panniculitis is a rare clinical variant of lupus erythematosus. In this report we described a 38 year-old female patient who had suffered from chronic discoid lupus erythematosus for several years before developing widespread inflammatory, sclerotic and ulcerative lesions, which were first diagnosed as Weber-Christian panniculitis. It was only when the patient developed other signs and symptoms of systemic lupus erythematosus that the subcutaneous lesions were recognized as lupus panniculitis. A combined regimen of tetracyclines and chloroquine resulted in a remission, both with regard to regression of the lesions and suppression of the serological parameters of disease activity. The findings in this particular patient and similar reports in the literature form the basis for a discussion of the entity of lupus panniculitis.

Chloroquine

[Danazol treatment of hereditary angioneurotic oedema (author's transl)].

Danazol, an attenuated androgen, was administered to four patients with hereditary angioneurotic oedema, with rapid and complete response without side-effects. The follow-up period has now been up to 17 months. In all patients there was an indirect indication that their hormonal state influenced the course of the disease.

Adult

Pure melanocyte cultures: differential serum requirements of guinea pig keratinocytes and melanocytes in primary epidermal cell cultures.

Contrary to melanocytes guinea pig keratinocytes do not attach and grow in primary epidermal cell cultures if plated in media containing guinea pig serum. This phenomenon is based on the low content or lack of guinea pig serum of a factor(s) promoting keratinocyte attachment. This factor, which is contained in fetal calf serum, binds to the keratinocyte cell surface and can be removed by trypsin. Guinea pig epidermal cell cultures plated in medium containing guinea pig serum therefore lead to pure or almost pure melanocyte cultures.

Animals

Comparison of three immunoassays for immune complexes in rheumatoid arthritis.

Three widely used radioassays that depend on different principles for the measurement of circulating immune complexes (CIC) in biologic fluids are the monoclonal rheumatoid factor (mRF), Raji cell, and C1q binding tests. A comparison of the ability of these methods to measure immune complex-like material in 71 sera and 30 synovial fluids of 91 patients with rheumatoid arthritis (RA) was carried out by a group working in adjacent laboratories in a single institution. The highest number of abnormal levels in the seropositive group was detected by the C1q binding assay (91%). Levels of CIC by the mRF and Raji cell tests were elevated in 81% and 76% of the patients, respectively. The closest correlation was between the Raji and mRF tests (r = 0.44 and P = 0.002) although one depends on complement fixation and one does not. Though significant correlations between the levels of CIC determined by the C1q test and either the mRF (P = 0.2) or Raji cell (P = 0.3) assay were not found in this group, 59% of the samples had elevated levels by all three tests. The frequency of CIC in the sera of patients with seronegative RA was much lower, with the C1q test again giving the highest number of abnormal results (29% versus 16% and 12% for the Raji and mRF tests). In view of the technical problems associated with these tests, particularly lack of a uniform reliable standard, it is likely that all three tests measure the same material in most RA sera and that some of the differences observed are related to inherent variability in the tests themselves rather than intrinsic differences among the CIC detected in these samples.

Adult

Keratinocyte substrate adhesion is magnesium-dependent and calcium-independent.

Primary cell cultures were prepared from guinea pig epidermis and six days old mice kidneys and hearts. Along with subcultures of Hep cells, plating experiments were performed using media devoid of bivalent cations containing dialzyed fetal calf serum. Upon stepwise supplementation with either calcium ++ or magnesium ++, keratinocytes displayed a unique dependency on magnesium ++ for cell attachment, whereas all other cell types could make use of either of these ions. Melanocytes, being a minority component of the epidermal cell population, do not conform to the predilection of keratinocytes for magnesium ++. Epidermal cell cultures prepared in media containing only calcium ++, therefore, result in pure melanocyte cultures.

Animals

Hereditary angio-oedema: treatment with danazol. Report of a case.

An 8-year-old boy with hereditary angio-oedema was treated with danazol under close endocrinological supervision. The boy's C1 esterase inhibitor (C1inh) and C4 levels increased rapidly to near normal values under a daily dose of 400 mg and were maintained at about 50% of the normal by maintenance doses of 200 mg danazol every other day. Throughout the entire treatment period (11 months) the boy has been maintained free from attacks of angio-oedema. No hormonal imbalance was detected in the follow-up period. Our results indicate that danazol should be suitable for the treatment of HAE not only in adults but also in prepubescent children.

Angioedema

Circulating immune complexes in cutaneous vasculitis. Detection with C1q and monoclonal rheumatoid factor.

To investigate the pathogeneic significance of immune complexes in cutaneous vasculitis, 107 patients with various forms of cutaneous vasculitis, including 59 patients with necrotizing (leukocytoclastic) vasculitis (group 1), and 48 patients with lymphocytic vasculitis, or a predominately lymphocytic perivascular infiltrate (group 2), were studied. Immunoglobulins or complement components in cutaneous blood vessels were detected by direct immunofluorescence in high frequency in both groups (91 and 88%, respectively). Using two radioassays for circulating immune complexes, Clq or monoclonal rheumatoid factor (mRF) reactive material was detected in 68% of the patients with necrotizing vasculitis but only 44% of the patients in the lymphocytic-perivascular group. The mRF radioassay was elevated in 58% of the first group of patients and 41% of the patients in group 2, although Clq binding activity was increased in 54% of the patients with necrotizing vasculitis but only in 9% of the patients with a lymphocytic vasculitis or lymphocytic perivascular infiltrate. By using both sucrose density gradient ultracentrifugation and Sepharose 6B gel filtration, the Clq and mRF reactive material detected in some patients with necrotizing vasculitis eluted in high molecular weight fractions that were also anticomplementary. In one patient with necrotizing vasculitis and hepatitis B antigenemia, these heavy molecular weight Clq and mRF reactive fractions contained a two- to three-fold increase in hepatitis B surface antigen when compared with lighter molecular weight fractions. Heavy and light molecular weight mRF reactive material could be detected in selected patients in the lymphocytic-perivascular group as well as in the necrotizing vasculitis group. These studies suggest that cutaneous vasculitis, including acute necrotizing (leukocytoclastic) vasculitis and some forms of lymphocytic vasculitis, and perhaps some diseases characterized by a lymphocytic perivascular infiltrate, may represent cutaneous expressions of immune complex disease.

Antigen-Antibody Complex

Lichen planus and bullous pemphigoid.

A patient is described who had clinical and histopathological features of lichen planus and bullous pemphigoid; deposits of in vivo bound C3 at the basement membrane zone, which, at the electron microscopy level, were deposited in the lamina lucida; and with circulating IgG antibasement membrane zone antibodies which exhibited a pronounced C3 binding capacity. The similarity of our case to others described in the literature suggests that bullous lichen planus in fact represents the coexistence of two distinct diseases, namely lichen planus and bullous pemphigoid.

Adolescent

[Circulating immune complexes and necrotizing vasculitis].

55 patients with necrotizing and with various forms of lymphocytic vasculitis were investigated for the presence of vascular deposits of immunoglobulins (Ig) and C3 by immunofluorescence testing of skin biopsies and with a 125I-Clq-binding assay for the presence of circulating immune complexes. Vascular deposits of Ig and C3 were found frequently both in patients with necrotizing and with lymphocytic vasculitis. In contrast, C1q binding activity was found almost exclusively in sera of patients with systemic necrotizing vasculitis. With one exception, all sera with C1q binding activity were from patients with vascular deposits of Ig and C3. The implications of these findings for our understanding of the development of system involvement in necrotizing vasculitis are discussed.

Antigen-Antibody Complex

[Necrotizing vasculitis: its manifestation as urticaria].

A case of urticarial vasculitis, a distinct form of necrotizing vasculitis is described. This entity is characterized by a chronic, persistent urticaria with petechiae and the histological features of necrotizing vasculitis. Serum complement is depressed and multisystem involvement can often be found. There is evidence for immune complexes to play a role in the pathogenesis of this disease.

Female

Clq binding activity in lupus erythematosus: correlation with the "lupus band test".

A 131I C1q binding assay was employed to estimate C1q binding activity (C1q BA), presumably due to the presence of circulating immune complexes, in lupus erythematosus sera. Thirteen of 16 sera from patients with systemic lupus erythematosus (SLE) had elevated C1q BA. Only 1 of 17 sera from patients with generalized discoid LE (GDLE) and none of 5 localized DLE patient's sera had elevated C1q BA. A correlation between a positive LE band test and the level of C1q BA was apparent. A positive LE band test tended to occur in patients with higher levels of C1q BA, whereas a negative LE band test tended to occur in patients with low levels of C1q BA.

Adolescent

Binding of soluble immune complexes to Raji lymphocytes. Role of receptors for complement components, C1q and C3-C3b.

We have found that, although the binding of particulate antigen-antibody complement complexes such as EAC to lymphoblastoid Raji cells is mediated largely through receptors for C3b, the binding of complement-containing soluble complexes such as those prepared with aggregated human IgG (AHG) occurs also via receptors for C1q. Evidence supporting this conclusion included: (1) Binding of AHG to Raji cells takes place after incubation in EDTA serum; (2) Binding of AHG does not occur in C1q deficient EDTA serum but does take place after addition of C1q; (3) The extent of binding of AHG in EDTA serum is a function of the amount of C1q present; (4) Raji cells can bind up to 5-4 times 10(5) molecules of 125I C1q per cell which can be blocked by unlabelled C1q; (5) AHG pre-incubated with C can bind to a T-cell line MOLT, which lacks receptors for C3b but possesses receptors for C1q to the same extent as Raji cells; (6) Immunoassays for immune complexes in human sera yield similar results whether Raji cells, MOLT cells or C1q precipitation is used for assay; (7) EAC-Raji cell rosettes can be inhibited with inulin-treated, C1q deficient serum containing C3b or C3d whereas binding of AHG or immune complexes in patient samples to Raji or MOLT cells is not inhibited by this reagent. We conclude that receptors for C1q on certain B and T lymphocytes may play an important role in physiologic functions of lymphocytes depending on binding of soluble immune complexes to their surfaces.

Antigen-Antibody Complex

[Hyperacute rejection of an HL-A identical renal allograft (author's transl)].

Hyperacute renal allograft rejection is described in a patient suffering from mesangio-proliferative glomerulonephritis. The transplanted kidney was HL-A identical and the direct cytotoxic cross-match between the recipient's serum and donor lymphocytes was negative. Intrarenal consumption of C 3, but not of C 1 q, C 4, total haemolytic complement, IgG or Igm was demonstrated. Immunofluorescence studies exhibited dense granular deposits of C 3, but not of IgG, IgM C 1q or C 4. These findings together with the observation of beta 1 C-beta 1A converting activity in the patient's serum, raised the possibility that the alternative pathway of complement activation induced by nephritic factor could have operated in this case. Further studies will be necessary to clarify the question whether hyperacute rejection of renal allografts is only antibody mediated or not.

Acute Disease

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

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