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Biomedical subjects

G Tappeiner

Publications and source records attributed to G Tappeiner.

At least 37 records · Page 2Linked to original sources
PubMed1982

Systemic lupus erythematosus in hereditary deficiency of the fourth component of complement.

Three patients from two families with complete hereditary deficiency of the fourth component of complement (C4) and systemic lupus erythematosus are described. The syndrome presented by these patients is characterized by early onset in life; exquisite sensitivity to sunlight and to cold exposure, the latter resulting Raynaud's phenomenon; and skin lesions involving not only exposed areas of the body but also palms and soles and presenting as butterfly rashes, maculopapular eruptions, and lesions similar to those of chronic discoid lupus erythematosus, with marked scaling, atrophy, and scarring. Lupus erythematosus (LE) cell tests were negative and antinuclear antibody (ANA) titers low or negative. The male patient of our series died at the age of 31/2 years from septicemia, whereas the two girls, aged 18 and 11 years, respectively, were alive at the time of writing. The C4-deficient gene is associated with HLA-Aw32, Bw38, and Bf S in one family and with HLA-A30, B18, DR7, and Bf S1 in the other family; the latter is the second family in which this HLA haplotype has been found to be associated with hereditary C4 deficiency.

Adolescent↗
PubMed1981

Dermatitis herpetiformis: immune complex detection with C1q and monoclonal rheumatoid factor.

To determine the significance of circulating immune complexes in dermatitis herpetiformis, serum samples from thirty patients with active disease were tested by a C1q binding radioassay, while serum samples from twenty-one of these patients were tested by a monoclonal rheumatoid factor (mRF) inhibition radioassay. By direct immunofluorescence, all patients demonstrated typical IgA deposition in dermal papillae. Using the C1q binding assay, only seven of forty-two serum samples had elevated C1q binding activity, while by the mRF inhibition assay, thirteen of twenty-five samples had elevated immune complex levels. Nine of these latter thirteen positive serum samples, however, were minimally elevated. Thus, IgG and/or IgM containing immune complexes are infrequently present, or at very low levels, in sera of patients with active dermatitis herpetiformis.

Antigen-Antibody Complex↗
PubMed1981

Hormone binding globulin levels in patients with hereditary angiooedema during treatment with Danazol.

Five patients with hereditary angiooedema were treated with Danazol for periods up to 10 months. This therapy resulted in normalization of serum levels of the inhibitor of the first complement component and disappearance of clinical disease symptoms in all patients. Although a variety of hormones were measured, only those of the pituitary-gonadal axis altered during treatment: a moderate decrease in leuteinizing hormone levels and a sharp fall of testosterone in adult men were observed. In all patients, however, both sex hormone binding globulin and thyroxine binding globulin concentrations were considerably suppressed during Danazol therapy. However, levels of corticosterone binding globulin and thyroxine binding globulin concentrations were considerably suppressed during Danazol therapy. However, levels of corticosterone binding globulin remained unchanged. The fraction of endogenous testosterone not bound to sex hormone binding globulin invariably increased. The possible relevance of these findings to the understanding of the mode of action of Danazol both in these patients and in general, are discussed.

Adult↗
PubMed1981

[Fasciitis with eosinophilia - Shulman syndrome].

Fasciitis with eosinophilia (also Shulman's disease or eosinophilic fasciitis) is characterized by an inflammatory thickening of the fascia, eosinophilia and hypergammaglobulinemia. It has clinical and histopathological similarities with scleroderma, though evidence for systemic involvement is rarely found. In this report, we describe the clinical and laboratory features of two patients with this disease and their response to treatment and we discuss its relationship to scleroderma and the pseudosclerodermatous syndromes and its prognosis.

Adult↗
PubMed1980

Circulating immune complexes in systemic scleroderma and generalized morphea.

There is growing evidence that pathologic changes in the vascular system are implicated in the pathogenesis of systemic scleroderma. It has been suggested that immune complex deposition may be responsible for such changes. We measured circulating immune complexes in 10 patients with severe systemic scleroderma, 1 of whom had clinical evidence of renal disease, and in 3 patients with generalized morphea. None of the patients had significantly elevated levels. Our findings suggest that although circulating immune complexes are of diagnostic and prognostic value in other collagen vascular diseases, they do not play a major role in the pathogenesis of systemic scleroderma in patients who lack clinical evidence of renal disease.

Adult↗
PubMed1979

[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↗
PubMed1979

[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↗
PubMed1979

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↗
PubMed1979

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↗
PubMed1979

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↗
PubMed1979

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↗
PubMed1979

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↗
PubMed1979

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↗