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)

We describe a 23-year-old Japanese man with systemic lupus erythematosus (SLE) who developed massive cutaneous mucinosis. He was diagnosed with SLE when he was 11 years old. Prednisolone therapy (30 mg/day) was initiated and reduced to 10 mg/day 3 months later; the SLE had been well-controlled with this dose of prednisolone for 12 years. However, infiltrated erythematous plaques developed on the middle-lateral area of his back at 17 years old and progressed to erythematous and elastic soft tumorous masses over 20 cm in diameter at 23 years old. Biopsies of the lesions on the nape revealed massive mucin deposition. Topical injection with hyaluronidase decreased the lesion. This cutaneous mucinosis can be distinguished clinically and histopathologically from papular and nodular mucinosis associated with SLE. The present case might be an unusual clinical variant of cutaneous mucinosis associated with SLE.
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PMID:Massive cutaneous mucinosis associated with systemic lupus erythematosus. 934 48

Cutaneous lupus mucinosis (CLM) is a rare variant of lupus erythematosus eruptions. Our 5 cases with CLM were reviewed. All were men with systemic lupus erythematosus (SLE). CLM occurred as asymptomatic cutaneous papules, nodules, or plaques on the trunk, upper and lower extremities, and face. Histopathology of CLM mainly revealed abundant mucin deposits among splayed collagen bundles throughout the dermis. However, some CLM lesions showed discoid lupus erythematosus-like epidermal and dermal changes and/or lupus profundus. Vasculitis was also revealed in the CLM lesions of 2 cases. The pathogenesis of CLM may be closely related to its two important features, the male preponderance and the association with SLE. Vasculopathy may also be involved in the development of CLM.
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PMID:Cutaneous lupus mucinosis: a review of our cases and the possible pathogenesis. 940 52

The cell surface protein tyrosine phosphatase CD45 is a major target of IgM anti-T cell autoantibodies in systemic lupus erythematosus (SLE). The autoreactive determinants on CD45 are O-linked glycans expressed on activated T cells and certain T cell lines, rather than linear or conformational polypeptide epitopes or N-linked glycans. To identify oligosaccharide structures that may play a role in the functional interactions of CD45 or are candidate target epitopes of SLE anti-CD45 autoantibodies, autoreactive CD45 purified from Jurkat T cells and non-autoreactive CD45 purified from CLL B cells were tested by ELISA for expression of mucin-type O-glycan structures. Monoclonal antibodies (mAbs) directed against blood group A, type 1 H chains, type 2 H chains, T, Le(a), sialylated-Le(a), Le(b), sialylated-Le(c), Le(x), sialylated-Le(x), multi-fucosylated Le(x), Le(y), and sialylated-extended Le(v) failed to react with CD45 from either B cells or T cells. However, mAbs directed against Tn (galNAcalpha1-->O-ser/thr) or sialosyl-Tn (neuNAcalpha2-6gaINAcalpha1-->O-ser/thr) structures reacted with CD45 derived from Jurkat T cells, but not from CLL B cells. Anti-Tn mAbs also reacted in western blotting procedures with CD45 isolated from Jurkat T cells, but did not react with CD45 isolated from CEM, MOLT-3, or PEER T cells; Daudi, Raji, or CLL B cells; or resting or Con A-activated PBL. However, anti-sialosyl-Tn mAbs stained blots of CD45 isolated from Jurkat and CEM T cells and Con A-activated PBL, a pattern of reactivity similar to that of the anti-CD45 autoantibodies. Flow cytometric analyses demonstrated that the sialosyl-Tn epitopes are expressed on a subpopulation of CD4 +/CD8- T cells.
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PMID:Expression of the sialosyl-Tn epitope on CD45 derived from activated peripheral blood T cells. 984 19

A 38-year-old female with systemic lupus erythematosus presented with abdominal pain, diarrhea and iron-deficient anemia. Computed tomogram showed a 2 x 4 cm inhomogeneous lesion of the right adnexa. An unusual mass was identified extending from the appendiceal orifice at colonoscopy, and an 8 cm tubular appendix, apparently prolapsed into the cecum, was identified at celiotomy. An appendectomy with cecectomy was performed. On cut section, mucin was extruded from the lumen of the appendix. A mucinous neoplasm of the appendix with mucinous dissection to the serosal surface was reported at the time of frozen section. No gross ovarian pathology or peritoneal implants were noted. Cystadenoma with associated mucocele formation was verified by permanent histology. Mucocele of the vermiform appendix is a rare condition associated with neoplastic transformation in approximately 75% of all cases. Benign mucinous cystadenoma of the appendix should be differentiated from cystadenocarcinoma by frozen section at the time of celiotomy to ensure appropriate treatment. While systemic lupus erythematosus can lead to cutaneous mucinosis, an association with mucinous cystadenoma of the appendix has not been previously reported. Surveillance for metachronous colonic neoplasms is warranted in patients diagnosed with a mucinous neoplasm of the appendix.
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PMID:Mucinous cystadenoma of the appendix in a patient with systemic lupus erythematosus. 992 68

Lupus tumidus is a rare subtype of chronic cutaneous lupus erythematosus that was first described by Gougerot and Bournier in 1930. Clinically, lupus tumidus presents as smooth, shiny, red-violet plaques of the head and neck that may be pruritic and have a fine scale. These lesions characteristically clear without scarring and recur in their original distribution. Histologic features include superficial and deep lymphohistiocytic infiltrates and abundant dermal deposits of mucin. We describe lupus tumidus as a distinct form of cutaneous lupus erythematosus and report 4 cases.
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PMID:Lupus tumidus. 1042 96

Periorbital edema associated with lupus erythematosus is not frequently reported. To our knowledge, periorbital edema from increased dermal mucin has not been reported with any form of lupus. We present a patient with discoid lupus exhibiting periorbital edema from massive mucinosis.
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PMID:Acute periorbital mucinosis in discoid lupus erythematosus. 1053 74

We report the case of a woman with clinical and histopathological features of reticular erythematous mucinosis syndrome (REM) who six years later developed diagnostic criteria for systemic lupus erythematosus (SLE). Two biopsies performed at the same area initially showed mucin dermal deposits and further development of characteristic dermo-epidermal changes of lupus erythematosus. Our findings suggest that SLE may present with an REM-like condition.
Lupus 2000
PMID:Systemic lupus erythematosus presenting with a reticular erythematous mucinosis-like condition. 1078 13

Tumid lupus erythematosus (TLE) is a variant of cutaneous lupus erythematosus. Most patients who present with these skin lesions are young women. The condition clinically resembles polymorphous light eruption, systemic lupus erythematosus (SLE), reticulated erythematous mucinosis, or gyrate erythema. Histopathologically, the lesions resemble classic lupus erythematosus because of their superficial and deep lymphohistiocytic inflammatory infiltrates and dermal mucin. However, unlike classic lupus erythematosus, there is little or no epidermal or dermo-epidermal involvement. Antinuclear antibody test results are usually negative. We describe 4 cases of TLE and discuss the differential diagnosis.
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PMID:Tumid lupus erythematosus. 1192 44

We present two Japanese cases of involutional lipoatrophy. The first case is that of a 30-year-old woman, who first appeared at our hospital complaining of a localized, well-demarcated depression, approximately 3 x 4 cm in size, normal to slightly erythematous in coloration, on the lateral side of the left upper arm (Fig. 1a). The condition was asymptomatic, and she had noticed this anomaly a month prior to consultation. She received intramuscular injections of corticosteroids of unknown dosage at the affected site for the treatment of allergic rhinitis 4 months prior to her present consultation. The second patient, a 23-year-old woman, appeared at our hospital complaining of a similar macule 4 x 4 cm in size, which she noticed several weeks prior to her most recent consultation. She had no history of injury or injection at the site before the development of the condition (Fig. 1b). She had been under treatment for atopic dermatitis since early childhood and was treated only with topical applications of white petrolatum containing 2% salicylic acid for the past several years. In order to rule out the possibility of acquired partial lipodystrophy associated with localized scleroderma, lupus profundus and the other connective tissue diseases, a histological examination was performed for both patients. Histopathological analysis of the region exhibited a well-defined fat lobule composed of numerous small adipocytes (Fig. 1c) embedded in hyaline connective tissue. Edema and dilated capillaries were noticeable in the subcutaneous tissue surrounding the area. Inflammatory cells were not prominent, although mononuclear cells were observed in both patients. No epidermal change was seen in either patient. Direct and indirect immunofluorescence studies revealed no deposits of immunoreactants in the skin of either patient. Immunohistochemical studies with the antibody against macrophage (anti-CD68 antigen; DAKO.) showed that positive cells were scattered around blood vessels and shrunken lipocytes in the subcutaneous tissues (Fig. 1d). Most of these cells in the fat lobules were also positive for mucin stains such as Alcian blue. No abnormal findings came to light in the ordinary hematological and blood chemistry examinations of both patients. The autoantibody screening tests using antinuclear, anti-DNA, anticentromere, and anti-Scl-70 antibodies were negative in both patients.
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PMID:Two Japanese cases of localized involutional lipoatrophy. 1201 Mar 46

The patient was a 44-year-old female. She was diagnosed as having systemic lupus erythematosus at the age of 21 years and had been receiving systemic steroid treatment (5 mg prednisolone/day). Nodules began to appear on her neck, chest and back from June in 2000, and she was referred to our clinic for examination and treatment. Examination of a biopsy sample of an eruption on the neck revealed mucin deposition in the dermis. Based on the clinical and histopathological findings, she was diagnosed as having typical nodular cutaneous lupus mucinosis. The dose of steroid was increased, and the eruptions gradually disappeared.
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PMID:A case of nodular cutaneous lupus mucinosis. 1270 73


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