Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0024141 (systemic lupus erythematosus)
 44,322 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

A vesiculobullous eruption with clinical and histological features of bullous pemphigoid developed in a 38-year-old woman with proved systemic lupus erythematosus. The patient had a sulphone responsive blistering disease that was characterized by pruritic subepidermal bullae and linear, predominantly IgA basement membrane zone deposition and IgA pemphigoid antibodies in her sera. Because both diseases are associated with immune complexes of special immunoglobulin classes, this association may not be entirely fortuitous.
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PMID:Coexistent atypical bullous pemphigoid and systemic lupus erythematosus. 700 71

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

Serum dehydroepiandrosterone sulphate (DHEAS) levels and diagnostic autoantibody titers were measured in patients with pemphigoid/pemphigus [n = 46/4; 21 men and 29 women, 42 to 93 years of age (mean 79)]. Twenty-four patients were either on peroral Prednisolone (n = 11), or topical treatment with betamethasone (n = 13), and the other 26 were either receiving non-steroidal drugs or were untreated. Their DHEAS levels were compared to those of 20 patients with psoriasis, and to 23 patients with secondary osteoarthritis (OA). Assessing the patients by group, the mean DHEAS level was markedly lower in the pemphigoid/pemphigus than in the psoriasis and OA patients (geometric mean 600 vs. 2130 and 2100 nmol/l, respectively; p < 0.001). This difference was independent of steroid treatment. No correlation was found between the DHEAS levels and antibody titers. The low levels found in pemphigoid/pemphigus are concordant with those reported for systemic lupus erythematosus, rheumatoid arthritis and polymyalgia rheumatica/giant cell arteritis. DHEAS deficiency is a permanent feature in these autoimmune diseases, and may contribute to their etiology and/or pathophysiology.
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PMID:Blood dehydroepiandrosterone sulphate (DHEAS) levels in pemphigoid/pemphigus and psoriasis. 755 62

Dapsone is used to treat several systemic inflammatory diseases, many of which have head and neck manifestations, such as leprosy, systemic lupus erythematosus, rhinosporidiosis, relapsing polychondritis, dermatitis herpetiformis, pemphigus vulgaris and bullous pemphigoid. It has also been recently used prophylactically alone or in combination against malaria and in AIDS patients against Pneumocystis carinii infections. This is significant to the otolaryngologist-head and neck surgeon since approximately 40% of AIDS patients will have head and neck manifestations. Thus, the likelihood that otolaryngologists will be treating patients who are taking dapsone regularly is significant. We present a case of a 16-year-old female who presented with a presumptive diagnosis of discoid lupus for biopsy confirmation of her disease. Induction of general anesthesia was complicated by methemoglobinemia, an uncommon side effect of dapsone. We will discuss recognition and prevention of this side effect, its potential anesthetic implications, complications and treatment.
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PMID:Dapsone-induced methemoglobinemia: an anesthetic risk. 755 44

Recent studies have identified a group of cicatricial pemphigoid patients who have IgG anti-basement membrane autoantibodies that recognize epiligrin, a set of disulfide-linked polypeptides closely related if not identical to laminin 5 (formerly called kalinin, nicein, or BM600). To further understand the pathophysiology of blister formation in these patients, we have sought to identify the specific polypeptide(s) targeted by their autoantibodies. Comparative studies show that sera from these patients (nine of nine), P1E1 monoclonal anti-epiligrin antibody, and polyclonal as well as monoclonal anti-laminin 5 antibodies immunoprecipitate the same set of disulfide-linked polypeptides from media of biosynthetically radiolabeled human keratinocytes. Moreover, sera from eight of nine patients with anti-epiligrin cicatricial pemphigoid immunoblot the alpha subunit of laminin 5 but show no reactivity to its beta or gamma subunits. In addition, circulating IgG from a representative patient was affinity-purified against the alpha subunit of laminin 5 and shown to bind the dermal side of 1 M NaC1 split skin in the same manner as autoantibodies from all patients with anti-epiligrin cicatricial pemphigoid. Sera from patients with bullous pemphigoid (n = 5), other forms of cicatricial pemphigoid (n = 5), epidermolysis bullosa acquisita (n = 4), or bullous systemic lupus erythematosus (n = 1) show no reactivity against any subunit of this laminin isoform in immunoprecipitation or immunoblot experiments. These findings correlate with prior reports showing that a monoclonal antibody directed against the alpha subunit of laminin 5 (i.e., laminin subunit alpha 3) induces detachment of human keratinocytes from extracellular matrix in vitro as well as epidermis from human skin in situ. Together, these studies suggest that laminin subunit alpha 3 mediates attachment of basal keratinocytes to epidermal basement membrane and that autoantibodies directed against it may be pathogenic.
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PMID:Anti-basement membrane autoantibodies in patients with anti-epiligrin cicatricial pemphigoid bind the alpha subunit of laminin 5. 756 Nov 56

Ocular cicatricial pemphigoid (OCP) is a chronic autoimmune inflammatory disease which affects the conjunctiva and other squamous epithelial mucous membranes resulting in a scarring process. It is characterized by the deposition of an anti-basement membrane zone (BMZ) antibody in vivo. Sera from 11 patients with active OCP were studied. Using monkey esophagus and normal skin as substrate, weak staining of the BMZ was observed in conventional indirect immunofluorescence (IIF) assay. Using salt split human skin as substrate, the OCP sera demonstrated binding to the epidermal side of the split, in low titers with weak staining. Ten of the 11 sera were positive on an immunoblot assay using COLO and SCaBER tumor cell lysates demonstrating 230, 205, 160, and 85 kD proteins. Sera from six bullous pemphigoid (BP) patients, with only cutaneous involvement and high titer of anti-BMZ antibody, as detected by IIF, also bound to 230, 160, and 85 kD proteins on both lysates in comigration experiments. Serum from five normal individuals and two patients each with severe atopic conjunctival disease, erythema multiforme with chronic conjunctivitis and systemic lupus erythematosus (SLE), did not demonstrate those bands. When the two lysates were first absorbed with BP sera and then the same lysates were immunoblotted with OCP sera, in all ten OCP sera the 230, 160, and 85 kD bands were eliminated and only a single 205 kD band was uniformly present. These results indicate that OCP sera recognize peptide(s) present in 230, 205 and 160 kD proteins in lysates from COLO and SCaBER tumor cells. These proteins contain the immunodominant region of the BMZ molecule(s) in which the OCP antigen(s) reside. The OCP antigen(s) appears to be distinct from the BP antigen(s).
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PMID:Detection and partial characterization of ocular cicatricial pemphigoid antigens on COLO and SCaBER tumor cell lines. 822 35

Azathioprine, cyclophosphamide, and cyclosporine are immunosuppressive agents commonly used by dermatologists. Azathioprine has a good safety profile and is preferred by most dermatologists for the treatment of bullous pemphigoid, cutaneous vasculitides, chronic eczematous dermatitides, and cutaneous manifestations of connective tissue diseases. For more fulminant diseases such as Wegener's granulomatosis, lymphomatoid granulomatosis, severe cases of systemic lupus erythematosus, or pemphigus, cyclophosphamide is more effective and its use is justified even though it is more toxic. Cyclosporine has been shown to be effective in a variety of dermatoses; the most promising results are seen in psoriasis, lichen planus, pyoderma gangrenosum, and chronic eczematous dermatitides. The long-term safety of cyclosporine remains to be determined, and it should be used judiciously with careful evaluation of the risk-benefit ratio in each case. Dermatologists who use immunosuppressive agents should be familiar with their indications, side effects, dosage, and monitoring.
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PMID:Immunosuppressive agents in dermatology. 843 20

Lichen planus is an autoimmune disease that typically involves skin and the mucosa of the genitalia and mouth. Conjunctival involvement is rare, and the microscopic abnormalities of affected conjunctivae are not well characterized. We treated two patients with cicatrizing conjunctivitis and extraocular conjunctival lichen planus confirmed by biopsy. We found irregular, thickened basement membrane with reduplications similar to the findings in oral mucosa affected by lichen planus. The absence of basement membrane immunoreactants excluded ocular cicatricial pemphigoid. Results of laboratory tests for collagen vascular diseases including sarcoidosis and lupus were also negative. Treatment with 2% cyclosporine eyedrops controlled inflammation and stopped cicatrization in one patient who was followed up for 12 months. The other patient, in whom lichen planus was recently diagnosed, responded favorably to topical cyclosporine. Lichen planus should be included in the differential diagnosis of cicatrizing conjunctivitis and its immunohistopathologic features should be studied in conjunctival biopsies. Topical cyclosporine may be used successfully for conjunctival lichen planus.
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PMID:Immunohistopathologic features and therapy of conjunctival lichen planus. 847 Jul 22

Cell-cell and cell-matrix adhesion molecules as well as extracellular matrix components are target structures of antibody-mediated autoimmunity that have recently been well characterized at the molecular biological level. Pathogenic autoantibodies against these molecules are causally related to disturbances of cell and tissue adhesion that become apparent as various (muco-)cutaneous blistering diseases. Desmosomal cadherins (desmogleins and desmocollins) mediate epidermal intercellular adhesion. Among these, desmoglein 1 and desmoglein 3 are the autoantigens of pemphigus foliaceus and pemphigus vulgaris, respectively, exhibiting intraepidermal blistering. The pathogenic relevance of autoantibodies against desmocollins. (IgA pemphigus) and desmoplakins (paraneoplastic pemphigus) still remains unclear. Hemidesmosomes contain the plaque protein BPAG1 and the partly collagen-like transmembrane protein BPAG2, representing the autoantigens of bullous pemphigoid and pemphigoid gestationis with subepidermal blistering. A certain subtype of cicatricial (benign mucous membrane) pemphigoid is characterized by autoantibodies against laminin 5 present in the subhemidesmosomal anchoring filaments, while epidermolysis bullosa acquisita and bullous SLE exhibit autoantibodies against collagen type VII constituting the anchoring fibrils. In addition, autoantibodies against a particular collagen type IV chain of the glomerular basement membrane are responsible for the manifestation of Goodpasture's syndrome. These recent molecular biological findings might be the basis for the development of novel therapeutic strategies.
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PMID:[Cellular adhesion molecules and components of the extracellular matrix as target structures of autoimmunity]. 892 91

Epidermolysis bullosa acquisita (EBA) is an acquired blistering skin disease characterized by the presence of IgG autoantibodies to type VII collagen. EBA autoantibodies recognize four major immunodominant epitopes localized within the amino-terminal, noncollagenous (NC1) domain. In this study, we developed a rapid, quantitative enzyme-linked immunosorbent assay (ELISA) to detect autoantibody activity against the complete NC1 domain of type VII collagen with the use of an eukaryotic-expressed, recombinant human NC1 antigen. With the ELISA, we tested serum from patients with EBA (n = 24), bullous systemic lupus erythematosus (BSLE) (n = 3), bullous pemphigoid (n = 16), pemphigus (n = 11), and normal controls (n = 12). All EBA and BSLE serum, including four sera that were negative by indirect immunofluorescence, demonstrated reactivity with immobilized NC1 in the ELISA. In contrast, none of the sera from healthy control subjects or patients with unrelated blistering skin diseases reacted with NC1. The EBA sera also reacted with recombinant NC1 by immunoblot analysis but with less sensitivity. Thus, the newly developed ELISA using recombinant NC1 is a sensitive, specific assay and a useful tool for rapidly screening EBA and BSLE serum.
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PMID:Development of an ELISA for rapid detection of anti-type VII collagen autoantibodies in epidermolysis bullosa acquisita. 898 Feb 90


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