Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0409974 (lupus)
 22,386 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

A lupus-like syndrome involving chronic urticaria with cutaneous vasculitis, systemic symptoms, hypocomplementemia with preferential depletion of C1q, and low m.w. (7S) C1q-precipitins has recently been defined. The C1q-precipitin activity (C1q-p) seems to represent a diagnostic marker of the disease, but its chemical nature is not yet clear. We have partially purified and characterized C1q-p from the serum of two patients with this syndrome and compared its activity with the C1q-precipitating activity of aggregated human gamma-globulin (AHGG) anti-C1q antibodies, and several polynucleotides including DNA and polyinosinic acid. C1q-p was found to partition with IgG during precipitation by ammonium sulfate and low ionic strength buffer as well as during column chromatography on DEAE-cellulose and G-200 Sephadex. Like AHGG, but in complete contrast to the polynucleotides, the C1q-precipitating activity of C1q-p was sensitive to pepsin, trypsin, and acidic conditions, but unaffected by DNAse or RNAse; the C1q-precipitating activity of anti-C1q antibody was not diminished by any of these procedures. Thus, C1q-p consists of gamma-migrating protein of low m.w., and its C1q-precipitating activity is indistinguishable from that of AHGG. These results are consistent with the concept that C1q-p is comprised, at least in part, of IgG that binds C1q via the Fc portion of the molecule.
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PMID:Low molecular weight C1q-precipitins in hypocomplementemic vasculitis-urticaria syndrome: partial purification and characterization as immunoglobulin. 2 69

Twelve cases of chronic urticaria with histopathologic features of lecocitoclastic allergic angitis are studied. The type of cutaneous lesion, personal and familiar atopic history and the presence of autoimmune disease are described. Light microscopy, direct immunofluorescence, anti DNA, antinuclear, antithyroid, Ro, La, Rnp and Sm antibodies, total complement levels, C3 and C4, rheumatoid factor, latex, ASTO, cryoglobulines and complete workup were investigated, taking into account natural progression and response to therapy. Two different groups are defined: 1) normocomplementemic (5 patients) and 2) hypocomplementemic (7 patients). They were all women except one. The cutaneous lesions were indistinguishable in the two groups. Only in the second group there was an associated disease (systemic lupus erythematosus, Sjogren syndrome disease, lupus-Sjogren overlap, autoimmune thyroid disease). Urticaria had been present from the onset of the disease in 4 patients, and occurred later during its course in 8 others. Five patients had thyroid disease (Hashimoto thyroiditis or Graves disease), two of them being mother and daughter. Another patient had a family history of Grave's disease and urticaria. Anti DNA antibodies were found in 7 cases, and anti Ro + La + in 3 cases. Response to treatment was variable with spontaneous cycles of worsening and remissions. One of the patients found a relationship with certain foods. Histopathologic results are related in both clinical normocomplementemic and hypocomplementemic groups. No significant differences were found between the two groups, but Ro+ and La+ patients exhibit more intense cariorexis and neutrophilic infiltrates.
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PMID:[Vasculitic urticaria: study of 12 cases]. 226 94

A female patient aged 39 with urticarial vasculitis is described. She has developed stubborn not itching blisters on the skin, suffered from periodic fevers, arthralgias; laboratory analyses have shown leukopenia, hypocomplementemia, immune complexes circulating in the blood and fixed in the skin vessel walls. Histologic examination has revealed leukocytoclastic vasculitis. The differential diagnosis between this condition, chronic urticaria, and lupus erythematosus is discussed.
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PMID:[Urticarial vasculitis]. 276 11

A 58 year-old woman had atypical chronic urticaria, arthralgias and abdominal pain. Attacks of angioneurotic edema occurred. Proteinuria was discovered. She had clinical and biological signs of inflammation, leukoneutropenia , antileukocyte antibodies and low CH 50, C1q and C4 levels without functional C1 esterase deficiency. C1q precipitins were not detectable. Skin biopsy disclosed angiitis and by immunofluorescence a lupus band test was positive. Serologic investigations in search of SLE were negative. Renal biopsy showed mesangial deposits, capillary loop thickening and mesangial fixation of anti-IgG, C3 C1q and C4 antisera. In the interstitium, voluminous perivenular inflammatory infiltrates were visible. With corticosteroid treatment clinical manifestations subsided and proteinuria disappeared. This observation of McDuffie 's angiitis with renal venulitis leads to a review of the literature with discussion of the mechanisms of hypocomplementemia.
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PMID:[Hypocomplementemic urticarial vasculitis with glomerulopathy and renal venulitis]. 672 99

Circulating immune complexes (C.I.C.) were investigated in 244 patients with various skin diseases and 100 healthy subjects. C.I.C. were detected by the PEG-C4 assay, firstly proposed by Digeon et al. using the precipitation by polyethylene glycol (PEG 3,5 p. 100) and the determination by laser nephelometry of complement component C4 in sera and in precipitates. The percentage of C4 precipitated and of positive subjects were significantly increased in numerous cutaneous diseases: systemic lupus erythematosus, scleroderma, pemphigus, bullous pemphigoid, dermatitis herpetiformis, psoriasis, contact dermatitis and lichen planus. Two cases of dermatomyositis, 3 cases of post herpetic erythema multiformis and 2 cases of Kaposi-Juliusberg syndroma were also positives but no definite conclusion can be given because of the few patients tested. On the contrary, the values of precipitated C4 are normal in most cases of atopic dermatitis (the method does not detect IgE-C.I.C.) scabies, porphyria cutanea tarda, cutaneous epithelioma and discoid lupus. In chronic urticaria and in mycosis fongoides the mean values of precipitated C4 are significantly increased but the number of positive subjects is low and the significance of these results is uncertain because of the wide range of the values. The results of the present study are compared with the literature data. The value of C.I.C. determination in determining the evolutivity of skin diseases and their possible role in pathogenesis are discussed.
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PMID:[Circulating immune complexes in skin disease patients. Study and literature data (author's transl)]. 730 15

We have assessed the cutaneous signs in 73 patients with systemic lupus erythematosus (SLE), seen during a 5-year period in an English hospital. Most previous information about the cutaneous manifestations of SLE has been obtained from studies performed in the U.S.A. We classified lesions as specific cutaneous and mucosal LE (acute, subacute and chronic) or non-specific LE-related, e.g. photosensitivity, urticaria, erythema, Raynaud's phenomenon or vasculitis. Acute cutaneous LE lesions included a butterfly rash with erythematous macules, telangiectasia or papulosquamous lesions, seen in 37 patients (51%) and facial oedema seen in four patients (5%). Five patients (7%) had psoriasiform subacute cutaneous LE. Chronic cutaneous LE was common: 18 patients (25%) had chronic discoid lesions (DLE) and, in 12 (15%), these had preceded systemic disease. One patient had facial lupus profundus. Ten patients (14%) had scarring alopecia secondary to DLE. Fifteen patients (20.5%) had chronic chilblain lupus. Twenty-three patients (31.5%) had a history of mouth ulceration. Of these, 11 (15%) gave a history of ulcers at the onset of their disease. Three (4%) had erythema and superficial ulceration of the palate, not typical of aphthous ulcers, and three (4%) had chronic buccal plaques. Cheilitis due to DLE was seen in three (4%), episcleritis in three (4%), five (7%) had nasal disease, six (8%) bullous skin eruptions, one 'the bullous eruption of SLE', four bullae associated with cutaneous vasculitis, and one bullae associated with ultraviolet radiation. Forty-six (63%) observed photosensitivity. A non-scarring alopecia occurred in 29 (40%). Vascular phenomena were common: three patients (4%) had chronic palmar erythema, Raynaud's phenomenon occurred in 44 patients (60%), chronic urticaria, worsened by sun exposure, was noted by 32 (44%) (in whom the lesions often lasted more than 36 h), eight (11%) had cutaneous vasculitis and three (4%) livedo reticularis. Skin changes play a prominent part in SLE and may provide helpful diagnostic information. In this British population, chilblains and urticaria were particularly common. Lesions of subacute cutaneous LE were relatively unusual in this group of patients with SLE.
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PMID:Cutaneous manifestations of systemic lupus erythematosus. 894 25

Basophil granulocytes and tissue mast cells and their mediators play a role in the pathogenesis of several immune and inflammatory disorders. Human basophils and mast cells (FcepsilonRI+ cells) can be activated through immunological interaction with the IgE-FcepsilonRI network. FcepsilonRI+ cells can be triggered by cross-linking between the Fab portions of IgE and multivalent antigens (direct anaphylaxis). 'Reverse type' anaphylaxis can occur through three distinct mechanisms: antibodies against the Fcepsilon portion of IgE (anti-IgE), antibodies against epitopes of the alpha chain of FcepsilonRI (anti-FcepsilonRIalpha) and anti-IgG acting on IgG-IgE complexes bound to FcepsilonRI. Anti-IgE autoantibodies are occasionally present even in normal donors and more frequently in a variety of allergic (chronic urticaria, atopic dermatitis and bronchial asthma) and autoimmune disorders (rheumatoid arthritis, lupus erythematosus and systemic sclerosis). IgG anti-IgE from a small percentage of patients induces the release of mediators from human FcepsilonRI+ cells. Some of the anti-IgE autoantibodies present in allergic patients are non-anaphylactogenic, thus representing a possible protective mechanism preventing the association of IgE with FcepsilonRI. Anti-FcepsilonRIalpha autoantibodies also occur in a significant percentage of patients of chronic urticaria and probably non-allergic asthma and some autoimmune diseases. Although anti-IgE and anti-FcepsilonRIalpha autoantibodies, present in a percentage of patients with immune disorders, are relevant to the pathogenesis of these conditions, much remains to be learnt about their immunochemistry, their prevalence and precise role in various inflammatory diseases.
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PMID:The anti-IgE/anti-FcepsilonRIalpha autoantibody network in allergic and autoimmune diseases. 1005 98

Chronic urticaria remains a major problem in terms of etiology, investigation, and management. It is important to identify patients in whom physical urticaria is the principal cause of disability. Once confirmed by appropriate challenge testing, no further investigation is required. Urticarial vasculitis (UV) is a major differential diagnosis of "idiopathic" urticaria (CIU). I perform biopsy of most patients in this category because UV cannot be considered confirmed in the absence of histologic evidence. Patients with confirmed UV need to be thoroughly investigated for paraproteins, lupus erythematosus hepatitis B and C, and inflammatory bowel disease. Of patients with CIU, a few (<5%) prove to have food additive reactivity confirmed by placebo-controlled challenge testing. There is no convincing evidence of the involvement of Helicobacter pylori or parasite infestation as a cause of chronic urticaria, although H pylori could have an indirect role. Recently it has become clear that 27% to 50% of patients with CIU have functional autoantibodies directed against the alpha-chain of the high-affinity IgE receptor or less commonly against IgG. These antibodies, whose involvement has now been independently confirmed in several centers, are identified by autologous serum skin testing and confirmed by histamine release studies or immunoblotting. Their removal (by intravenous Ig or plasmapheresis) or treatment by cyclosporine has proved highly beneficial in severely affected patients. However, the routine treatment of all CIU patients, irrespective of etiology, remains the judicious use of H(1) antihistamines.
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PMID:Chronic urticaria. 1075 14

The three major groups of immunoglobulins (gamma G, gamma A, and gamma M) associated with this disease are reviewed. The presence or absence of atopic disease may account for percentage variability of gamma A because reagins (skin-sensitizing antibodies) are found in this immunoglobulin. The gamma A is the antibody usually responsible for anaphylaxis, rather than the gamma G precipitins, so stressed in the past. All three immunoglobulins may be found in serum sickness, which could account for the complex nature of this type of penicillin urticaria. The merits of the immunological tests (penicilloyl-polylysine, benzyl penicillin, hemagglutination, basophil degranulation) for the detection of penicillin sensitivity are analyzed, particularly as each applies to the various types of penicillin urticaria (serum sickness, anaphylactic, dermographic, delayed dermographic, and simple chronic urticaria and the lupus diathesis). The penicilloyl-polylysine test is greatly overrated as a means of predicting possible anaphylaxis. The benzyl penicillin skin test properly performed is an excellent means of indicating this.
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PMID:Recent advances in the understanding of the penicillin urticarias. 1185 Sep 31

Part I of this report deals with the topical use of cortisone in a variety of skin diseases. Fifteen patients with chronic discoid lupus erythematosus, four patients with necrobiosis lipoidica diabeticorum, four with psoriasis, one with lichen planus and one with granuloma annulare were treated with cortisone ointment. All the patients with chronic discoid lupus erythematosus had some degree of improvement. In two patients with chronic lupus erythematosus, complete clearing of the eruption occurred. In four patients with necrobiosis lipoidica diabeticorum remarkable involution resulted. Patients with psoriasis, lichen planus and granuloma annulare were not benefited. Part II deals with the systemic use of cortisone. Eight patients with severe serum sickness-like penicillin reaction responded dramatically to parenterally administered cortisone. In two cases of pemphigus vulgaris and one case of Sulzberger-Garbe disease, the disease was kept in remission with cortisone administered intramuscularly as well as orally. Partial improvement resulted in a case of localized myxedema associated with malignant exophthalmus. Two patients with exfoliative dermatitis due to therapy with heavy metals responded dramatically to cortisone. No beneficial effects were noted in patients with chronic urticaria and atopic dermatitis.The systemic use of ACTH and cortisone in dermatology at present should be confined to patients with known fatal or hopelessly incapacitating diseases and to patients with extreme hypersensitivity reactions which may be protracted or life-endangering, and which can be controlled or cured with a relatively small total dosage of the agents in a short time.
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PMID:The topical and systemic use of cortisone in dermatology. 1488 29


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