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)

Four patients with concurrent, chronic, progessive, localized scleroderma and discoid lupus erythematosus were studied; the condition originated as linear scleroderma in three of them. Three of the four patients were young females at the onset of the first skin disease. Dermatopathologic study confirmed the scleroderma and lupus erythematosus (LE). Direct immunofluorescence showed a positive band test in three cases. Unusual serological results included a positive LE clot test in three cases, a positive extractable nuclear antigen test in one case, and a negative antinuclear antibody test on repeated occasions in all four cases. Rare cutaneous disease similar to systemic, "mixed," or "overlap" connective tissue disease exists and offers an opportunity to study unusual immunologic and pathological events in both scleroderma and LE.
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PMID:Concurrent localized scleroderma and discoid lupus erythematosus. Cutaneous 'mixed' or 'overlap' syndrome. 71 82

Quantitative television microscopy of nailfold capillaries of the fingers was performed in 12 patients with cutaneous lupus erythematosus (six with discoid type and six with disseminated type), in six patients with localized scleroderma (two with circumscribed type, two with linear types, and two with atrophic type), in 10 patients with systemic lupus erythematosus, and in eight patients with systemic scleroderma. The following features were analyzed and compared with a control group (n = 15) of similar age: venous plexus visibility; density of capillaries; avascular fields; hemorrhages; giant capillaries; diameters of the transitional segment, the arterial, and the venous limbs; loop width; and flow stop caused by local cooling test. The patient groups with cutaneous lesions only showed no essential differences as compared with the controls. Patients with systemic scleroderma differed in almost every finding from the controls and from patients with localized scleroderma. Patients with systemic lupus erythematosus exhibited significant differences in several findings as compared with the controls and the cutaneous lupus erythematosus group, but there was overlap.
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PMID:Quantitative nailfold capillary microscopy in cutaneous and systemic lupus erythematosus and localized and systemic scleroderma. 186 81

Three patients presented with septal fasciitis and panniculitis, associated with clinical and laboratory features which precluded straight-forward classification into eosinophilic fasciitis, localised scleroderma, or lupus erythematosus profundus. Treatment with cimetidine caused the remission of cutaneous manifestations and the extracutaneous abnormalities, such as nailfold capillary disturbances and the presence of antithyroid antibodies, improved. It is concluded that features of eosinophilic fasciitis or localised scleroderma and certain additional atypical elements should be categorised as atypical fasciitis-panniculitis syndrome.
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PMID:Treatment with cimetidine of atypical fasciitis panniculitis syndrome. 224 Dec 70

A 40-year-old man presented with an ulcerated tumour in a fibrotic plaque on the dorsum of his left foot. Due to severe localized scleroderma, the patient had been treated with azathioprine 10 years earlier. Histopathology of the excised tumour revealed an anaplastic squamous cell carcinoma within a scar of localized scleroderma. The case demonstrates that not only patients with tense scar tissue following burning, congelation, chronic radiodermatitis, lupus vulgaris or lupus erythematosus but also patients who have had localized scleroderma may run a greater risk of developing squamous cell carcinoma. Immunosuppressive therapy has to be discussed as an additional risk factor in our patient. Therefore, narrow clinical follow-up was recommended for early detection of relapse.
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PMID:Squamous cell carcinoma in localized scleroderma following immunosuppressive therapy with azathioprine. 810 25

Systemic lupus erythematosus, chronic cutaneous lupus erythematosus and subacute and acute cutaneous lupus erythematosus are associated with distinct cutaneous manifestations, and each disease has a particular clinical course and prognosis. Dermatomyositis, an immune-mediated disorder of unknown etiology that is often associated with a malignancy, consists of an inflammatory myopathy combined with characteristic cutaneous findings. Some patients with dermatomyositis also have clinical features that overlap with those of systemic lupus erythematosus or scleroderma. Manifestations of scleroderma range from cutaneous involvement alone to multisystem internal disease. Morphea, progressive systemic sclerosis, eosinophilic fascitis, eosinophilia myalgia syndrome and mixed connective tissue disease are all within the spectrum of scleroderma.
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PMID:Cutaneous manifestations of selected rheumatologic diseases. 862 90

Autoantibodies to CRP were reported previously in patients suffering from toxic oil syndrome. This syndrome resembles autoimmune diseases such as systemic lupus erythematosus (SLE) or systemic scleroderma. We therefore examined the prevalence of antibodies to CRP and other acute-phase proteins in autoimmune diseases, including SLE, subacute cutaneous lupus erythematosus (SCLE), systemic scleroderma (SSc), and primary biliary cirrhosis (PBC), as well as in bone marrow transplantation-induced chronic graft-versus-host disease and eosinophilia-myalgia syndrome. IgG antibodies to CRP were found in 78% of SLE and in 30% of SCLE patients, while 16% of patients with PBC were positive. In up to 45% of patients with SSc predominantly IgG antibodies to ceruloplasmin were detectable. Lack of systemic involvement as in discoid lupus erythematosus and localized scleroderma (morphea) correlated with low or absent antibody formation. However, no correlation was found between anti-acute-phase protein antibodies with liver disease or other organ involvement. Adsorption studies revealed that non-native epitopes on the CRP molecule, termed modified CRP, are the main target of antibodies. Chronic inflammatory tissue injury in systemic autoimmune disease might increase the presentation of cryptic epitopes of CRP to the threshold required for T cell activation.
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PMID:Autoantibodies to C-reactive protein (CRP) and other acute-phase proteins in systemic autoimmune diseases. 973 58

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

Lupus erythematosus panniculitis is a rare disease characterized by deep subcutaneous nodules, most commonly localized on the upper limbs and face. Unique clinical presentations, such as linear configuration or 'overlap' forms between lupus erythematosus panniculitis and localized scleroderma have been reported. We present here the clinical characteristics, course and laboratory findings of 2 patients having linear lupus erythematosus panniculitis with localized scleroderma-like changes. The 2 patients (of the 14 patients with lupus erythematosus panniculitis seen by us since 1990) were females with a young age at the onset of disease (median, 25 years). In 1 case, evolution into systemic lupus erythematosus with severe renal involvement occurred whereas the other patient, who had a spontaneous abortion and exhibited anticardiolipin antibodies, should be followed and screened for the emergence of antiphospholipid syndrome. Thus, the clinical behavior of this variant seems to be more aggressive, as compared with the usual course of lupus erythematosus panniculitis, which is considered to be a benign disease, although some reports have suggested that its prognosis is not always favorable. The linear distribution could be the clinical hallmark of such a unique, 'sclerodermic' subset of lupus erythematosus panniculitis.
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PMID:Sclerodermic linear lupus panniculitis: report of two cases. 1594 22

Morphea and other scleroderma-like skin conditions are occasionally linked with exposure to chemical compounds such as silicone. We treated a 56-year-old woman with generalized severe skin induration accompanied with systemic symptoms and peripheral eosinophilia, which appeared 2.5 years after breast silicone implantation and abdominal liposuction. Blood test results and histopathological examination of her skin suggested the diagnosis of morphea overlapping with eosinophilic fasciitis. Her skin disease was presumed to be an autoimmune reaction to silicone implantation. While the removal of the implants did not improve her illness, treatment with 1 mg/kg prednisone and PUVA bath was initiated, with some improvement. This patient illustrates an example of ASIA (Autoimmune Syndrome Induced by Adjuvants), as her disease appeared following exposure to an adjuvant stimulus, with 'typical', although not well-defined, autoimmune manifestations.
Lupus 2012 Feb
PMID:Autoimmune syndrome induced by adjuvants (ASIA) in the Middle East: morphea following silicone implantation. 2223 43

Morphea is an autoimmune sclerotic skin disease of unknown pathogenesis. As type 1 interferons (IFN) have been implicated in the pathogenesis of systemic sclerosis, we proposed that type 1 IFN promote localized inflammation and fibrosis in morphea. To investigate the expression of the type 1 IFN-inducible protein myxovirus A (MxA) and the presence of plasmacytoid dendritic cells (pDC) in lesions of morphea, lesional skin of 10 patients with morphea was examined by immunohistochemistry for the presence of the type 1 IFN-inducible protein, myxovirus A (MxA), and the pDC markers, CD123 and BDCA-2, and was compared with lesional skin of cutaneous lupus erythematosus, lichen planus and keloid. Lesional and non-lesional morphea skin was compared. MxA was expressed in the epidermis as well as the reticular dermis and subcutis in morphea. pDCs were abundant around vessels and between fibrous bundles. Non-lesional biopsies demonstrated little or no expression of MxA and pDC markers. Keloid showed minimal expression of MxA and pDC markers. We demonstrate the expression of type 1 IFN-related protein MxA and plasmacytoid DCs in lesional but not in non-lesional biopsies of morphea. These findings suggest a potential role for type 1 interferons in the pathogenesis of morphea.
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PMID:Type 1 IFN-induced protein MxA and plasmacytoid dendritic cells in lesions of morphea. 2250 98


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