1/30. The schnitzler syndrome. Four new cases and review of the literature.The schnitzler syndrome is characterized by a chronic urticarial eruption with a monoclonal IgM gammopathy. The other signs of the syndrome include intermittent elevated fever, joint and/or bone pain with radiologic evidence of osteosclerosis, palpable lymph nodes, enlarged liver and/or spleen, elevated erythrocyte sedimentation rate, and leukocytosis. The mean delay to diagnosis is more than 5 years, and this syndrome is of concern to internists and many medical specialists. patients with this syndrome are often initially considered to have lymphoma or adult-onset Still disease, which are the main differential diagnoses. However, hypocomplementic urticarial vasculitis, systemic lupus erythematosus, cryoglobulinemia, acquired C1 inhibitor deficiency, hyper IgD syndrome, chronic infantile neurologic cutaneous and articular (CINCA) syndrome, and Muckle-Wells syndrome should also be excluded, because diagnosis relies on a combination of clinical and biologic signs and there is no specific marker of the disease. The disease pursues a chronic course, and no remissions have yet been reported. Disabling skin rash, fever, and musculoskeletal involvement are the most frequent complications. Severe anemia of chronic disease is another serious complication. The most harmful complication, however, is evolution to an authentic lymphoplasmacytic malignancy, which occurs in at least 15% of patients. This hematologic transformation can occur more than 20 years after the first signs of the disease, thus patients deserve long-term follow-up. Treatment is symptomatic and unsatisfactory. The skin rash is unresponsive to treatment, and nonsteroidal antiinflammatory drugs, antihistamines, dapsone, colchicine, and psoralens and ultraviolet A (PUVA) therapy give inconstant results. fever, arthralgia, and bone pain often respond to nonsteroidal antiinflammatory drugs. In some patients, these symptoms and/or the presence of severe inflammatory anemia require steroids and/or immunosuppressive treatment, which ameliorate inflammatory symptoms but do not change the course of the skin rash.- - - - - - - - - - ranking = 1keywords = lupus erythematosus, erythematosus, systemic lupus erythematosus, systemic lupus, lupus (Clic here for more details about this article) |
2/30. Flame figures in urticarial lesions accompanying systemic lupus erythematosus.We present the case of a 38-year-old female patient with systemic lupus erythematosus and atypical urticarial skin lesions with an alteration in shape, but not complete disappearance, within 24 hours. hematoxylin-eosin-stained sections revealed flame figures. We review urticarial lesions in lupus erythematosus and discuss the possible pathomechanisms.- - - - - - - - - - ranking = 5.8221101666842keywords = lupus erythematosus, erythematosus, systemic lupus erythematosus, systemic lupus, lupus (Clic here for more details about this article) |
3/30. Hypocomplementemic urticarial vasculitis: report of a 12-year-old girl with systemic lupus erythematosus.Urticarial vasculitis, a form of leukocytoclastic vasculitis involving the postcapillary venules, is classified as a type III hypersensitivity reaction and has been associated with connective tissue disease. The lesions resemble urticaria and typically persist for more than 24 hours. Urticarial vasculitis usually affects young women, and the diagnosis is confirmed at histologic examination. patients with urticarial vasculitis can be divided into 2 types--those with normal complement levels and those with hypocomplementemic urticarial vasculitis (HUV). patients with normocomplementemic urticarial vasculitis have a milder course than do patients with HUV, a condition that has a strong association with systemic lupus erythematosus. angioedema, ocular inflammation, obstructive lung disease, and glomerulonephritis are commonly associated with HUV. We describe the case of a girl with systemic lupus erythematosus and HUV who also had pancreatitis, hypothyroidism, and elevated levels of antiphospholipid antibodies.- - - - - - - - - - ranking = 6keywords = lupus erythematosus, erythematosus, systemic lupus erythematosus, systemic lupus, lupus (Clic here for more details about this article) |
4/30. Urticarial vasculitis: report of a case and review of the literature.A woman with cutaneous vasculitis had a severe bullous eruption that was suggestive of erythema multiforme. The patient also had a history of recurrent urticaria that continued intermittently for over a year of follow-up examination. skin biopsy specimens of both urticarial and erythema and multiforme lesions showed leukocytoclastic vasculitis. An illness resembling systemic lupus erythematosus (SLE) is suggested by transient, low-titer, positive antinuclear antibody tests, persistent deposits of immunoglobulin and complement in normal skin, arthralgias, circulating immune complexes, and chronic hypocomplementemia. This case is similar to cases previously reported as "hypocomplementemic vasculitis," an "unusual SLE-related syndrome," and "urticaria with vasculitis."- - - - - - - - - - ranking = 1keywords = lupus erythematosus, erythematosus, systemic lupus erythematosus, systemic lupus, lupus (Clic here for more details about this article) |
5/30. Transverse myelitis and chronic urticaria in systemic lupus erythematosus. Case report.We report a 40 years old woman with chronic urticaria and acute transverse myelitis associated with systemic lupus erythematosus. The urticaria appeared in her adolescence and after 26 years was followed by photosensitivity, peripheral polyarthritis and acute transverse myelitis, with positive antiphospholipid and antinuclear antibodies. Both chronic urticaria and acute transverse myelitis have been described associated with or appearing as the first manifestation of systemic lupus erythematosus. Transverse myelitis is a rare and still poorly understood condition reported in about 2% of patients with systemic lupus.- - - - - - - - - - ranking = 6.0707785247089keywords = lupus erythematosus, erythematosus, systemic lupus erythematosus, systemic lupus, lupus (Clic here for more details about this article) |
6/30. hypersensitivity to mycophenolate mofetil in systemic lupus erythematosus: diagnostic measures and successful desensitization.Despite therapeutic advances in the treatment of systemic lupus erythematosus (SLE), several patients are still afflicted with severe and uncontrolled symptoms. Recently, mycophenolate mofetil (MMF) has been introduced in the treatment of SLE, with a significant therapeutic benefit, and minor side effects have been reported. Data on the adverse effects of MMF in SLE are lacking. We present an SLE patient who developed urticaria during MMF treatment. Rechallenge with MMF established the diagnosis of MMF-induced urticaria. As MMF was considered a necessary therapy, a desensitization protocol was devised, which successfully induced tolerance to MMF. This is the first published protocol for MMF desensitization, which induced tolerance in an SLE patient previously reacting with generalized urticaria.- - - - - - - - - - ranking = 5keywords = lupus erythematosus, erythematosus, systemic lupus erythematosus, systemic lupus, lupus (Clic here for more details about this article) |
7/30. Hypocomplementemic urticarial vasculitic syndrome, asthma and anaphylactic reaction with ampicillin.A patient with hypocomplementemic urticarial vasculitic syndrome (HUV) is presented. This is an immunological pathology, limited to skin or multisystemic, that requires a differential diagnosis with erythematosus systemic lupus on the same occasions. The ever-present symptom is skin participation, such as urticaria-angioedema or fixed exanthema; biopsy shows necrotizing venulitis with polymorphonuclear infiltration and leukocytoclastic powder. Typical laboratory data are: diminished C3, C4 and C1q; C1 inhibition can be low or normal; the more characteristic finding is the presence of C1q associated immunocomplexes. Leukocytoclastic necrotizing vasculitis was found in the skin biopsy. During the course of illness (three years) the patient presented moderate cutaneous symptoms and asthma, without other systemic participation. During this period, antihistamines and, occasionally, corticoids were administered with improvement. Moreover, the patient presented urticaria related to ampicillin ingestion, and furthermore, the presence of anaphylaxis to beta-lactam was diagnosed in vivo and specific IgE was found in the laboratory study. This feature was previously observed by other authors; however, we cannot determine why the IgE-mediated allergy to beta-lactam and a complement pathology like HUV are related.- - - - - - - - - - ranking = 0.32585945700964keywords = erythematosus, systemic lupus, lupus (Clic here for more details about this article) |
8/30. Hypocomplementemic urticarial vasculitis: a rare presentation of systemic lupus erythematosus.BACKGROUND: Urticarial vasculitis is a small-vessel vasculitis, presenting clinically as persistent urticarial skin lesions and microscopically as leucocytoclastic vasculitis. Hypocomplementemic urticarial vasculitis syndrome (HUVS) is a distinct type of urticarial vasculitis with multiorgan involvement, whose etiology and link with other diseases are still unknown. Some authors have suggested that HUVS can be accompanied by systemic lupus erythematosus (SLE), and others believe that it is a rare subtype of SLE. Urticarial vasculitis is seen in 7-8% of SLE, while 50% of HUVS patients are diagnosed with SLE. OBSERVATIONS AND RESULTS: We report a case of HUVS associated with SLE with fatal outcome unresponsive to the combination of systemic corticosteroids and azathioprine. CONCLUSIONS: SLE and HUVS share both clinical and laboratory features and are probably not separate entities. It is mostly likely that HUVS and SLE fall into the same spectrum of autoimmune diseases. HUVS is probably a subset of SLE. As both diseases can fatally, it should be kept in mind that the overlap of SLE and HUVS may exhibit a relatively rapid progression and poor prognosis.- - - - - - - - - - ranking = 5keywords = lupus erythematosus, erythematosus, systemic lupus erythematosus, systemic lupus, lupus (Clic here for more details about this article) |
9/30. dapsone is an effective therapy for the skin lesions of subacute cutaneous lupus erythematosus and urticarial vasculitis in a patient with C2 deficiency.The deficiency of second component of complement (C2d) is the most common hereditary complement deficiency. patients with C2 deficiency are frequently associated with an auto-immune disease process, in particular, systemic lupus erythematosus (LE)-like syndrome and/or vasculitic syndrome or bacterial infections. C2d has been associated with the LE subset of subacute cutaneous LE (SCLE), the presence of anti-Ro (SSA) antibodies, and the human leukocyte antigen (HLA) types A10, B18, DR2. We describe the clinical, serologic and immunogenetic data in a patient with manifestations of sjogren's syndrome who developed urticarial vasculitis and photosensitive annular SCLE which were effectively treated with oral dapsone. Our case illustrates the dynamic nature of LE.- - - - - - - - - - ranking = 2.158924572903keywords = lupus erythematosus, erythematosus, systemic lupus erythematosus, systemic lupus, lupus (Clic here for more details about this article) |
10/30. urticaria, nephritis, and pseudotumor cerebri.The spectrum of chronic urticarial disease ranges from chronic urticarial skin lesions alone to well-characterized systemic lupus erythematosus with urticarial vasculitis as the major skin manifestation. Within this spectrum is the syndrome of urticarial vasculitis associated with systemic disease manifestations. There have been six previously recorded cases of urticarial vasculitis associated with pseudotumor cerebri. At least two of these have included membranoproliferative glomerulonephritis. The authors report a case of chronic urticarial disease associated with pseudotumor cerebri and membranoproliferative glomerulonephritis, but without demonstrable vasculitis. It is possible that this represents a distinct entity within the spectrum of chronic urticarial disease and cca be easily screened for in clinical practice.- - - - - - - - - - ranking = 1keywords = lupus erythematosus, erythematosus, systemic lupus erythematosus, systemic lupus, lupus (Clic here for more details about this article) |
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