Cases reported "Schnitzler Syndrome"

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1/16. Acquired C1 inhibitor deficiency associated with systemic lupus erythematosus, secondary antiphospholipid syndrome and IgM monoclonal paraproteinaemia.

    In this case report, a woman is described who developed systemic lupus erythematosus at the age of 36 years affecting the central nervous system and kidneys, showing the serological evidence of an acquired C1 inhibitor deficiency, but also developing an antiphospholipid syndrome and IgM lambda type paraproteinemia. During the 25 years follow-up she did not show any signs of angioedema. Although the immunological abnormalities are still constantly present, the disease has been quiescent since the past three years. The possible pathophysiology of clinical and laboratory features are discussed. ( info)

2/16. A case of Schnitzler's syndrome with nodular regenerative hyperplasia of the liver.

    Schnitzler's syndrome is a rare condition of urticaria, macroglobulinemia, and sclerotic bone lesions. We report a case in a 70-year-old man in whom inflammatory polyarthralgia was followed by a nonpruritic urticarial eruption with a moderate decline in general health. Laboratory tests showed inflammation and a modest isolated peak of monoclonal IgM kappa. There was no evidence of waldenstrom macroglobulinemia. Schnitzler's syndrome was considered. However, an ultrasound scan of the abdomen done because of mild gamma-glutamyl-transferase elevation disclosed multiple hepatic lesions. The liver histology showed incipient nodular regenerative hyperplasia. Only about 30 cases of Schnitzler's syndrome have been reported since the seminal description in 1972. Hepatic involvement was a common but nonspecific finding, and we found no cases with nodular regenerative hyperplasia. However, this abnormality is often found in patients with autoimmune or hematological disorders. The pathogenesis of Schnitzler's syndrome remains unknown, but the possibility of progression to a hematological malignancy requires prolonged follow-up. ( info)

3/16. Schnitzler's syndrome: report of a case with progression to Waldenstrom's macroglobulinaemia.

    Schnitzler's syndrome is a rare but distinct entity in which chronic urticaria is a prominent feature. Our patient presented with an urticarial rash associated with intermittent fevers, lethargy, a migratory polyarthralgia and loss of weight. skin biopsy revealed a neutrophilic urticaria. An IgM kappa paraprotein was detected on serum protein electrophoresis at a level of 8 g/L (0.6-2.5 g/L). Bone marrow biopsy was normal. Marked improvement of the arthralgia, fever and lethargy was obtained with oral prednisolone. The urticarial rash, however, was only partly responsive. For the next 10 years the patient remained stable but corticosteroid dependent. Over a period of 10 years the IgM paraprotein had slowly risen to 47 g/L. Repeat bone aspirate and trephine revealed a diagnosis of a low grade lymphoplasmacytic lymphoma consistent with Waldenstrom's macroglobulinaemia. To date, despite six cycles of chemotherapy with oral chlorambucil, his disease remains persistent but stable. ( info)

4/16. schnitzler syndrome: a case study.

    schnitzler syndrome is a clinical entity of unknown etiopathogenesis and is an unusual cause of chronic urticaria. It is characterized by the presentation of a monoclonal band, especially IgM, as well as fever, asthenia and arthralgia. We present the case of a 48-year-old man with 5 years of recurrent urticarial lesions, slight itching, as well as lip, face and occasional pharyngeal edema. The patient had also suffered from fever and right hip and left knee arthralgia. The protein electrophoresis and the immunoelectrophoresis presented an IgG band and a slight lambda band, which is suggestive of a monoclonal gammopathy. The bone marrow examination showed a slight increase in the lymphoplasmatic cells. The marrow biopsy was negative. In order to rule out tumorous, infectious and rheumatic processes as well as collagen diseases, other complementary examinations were performed with negative results. The diagnosis of schnitzler syndrome as a cause of chronic urticaria requires a thorough study in order to rule out other systemic processes provoking the same symptoms. ( info)

5/16. Schnitzler's syndrome: no evidence for autoimmune basis in two patients.

    We report two cases of Schnitzler's syndrome in which anti-interleukin-1alpha autoantibodies and functional autoantibodies against the high affinity IgE receptor (FcepsilonRIalpha) or against IgE were absent. One patient responded well to TL-01 phototherapy, a treatment which may be considered in patients with Schnitzler's syndrome if, as is usually the case, they are unresponsive to antihistamine therapy. ( info)

6/16. 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. ( info)

7/16. Malignant evolution of Schnitzler's syndrome--chronic urticaria and IgM monoclonal gammopathy: report of a new case and review of the literature.

    Schnitzler's syndrome, initially described in 1974 is an uncommon condition defined by chronic urticaria and monoclonal IgM gammopathy. Additional features include fever of unknown origin, elevated ESR, bone pain and frequently a benign clinical course. We conducted a literature search of medline, EMBASE and Cancerlit and found 56 cases of Schnitzler's syndrome reported to date. The absence of lymphoproliferative disease in this condition is typical, but nine patients have progressed to develop lymphoplasmacytic neoplasias, particularly Waldenstrom's macroglobulinemia (WM). Malignant evolution of Schnitzler's syndrome is a rare complication, but emphasizes the importance of long term follow-up and the need for these patients to undergo periodic assessment of the bone marrow and lymph nodes. Treatment of this condition is difficult, with varying response to corticosteroids and largely unsuccessful results with standard chemotherapy used for WM. We describe a case of Schnitzler's syndrome in a 50-year old man with lymphocytic aggregates in the bone marrow after 9 years of chronic urticaria, fever, arthralgias and bone pain. We review the clinical features and treatment, with emphasis on the hematologic aspects of this unusual condition. ( info)

8/16. Report of a case of Schnitzler's syndrome treated successfully with interferon alpha 2b.

    Schnitzler's syndrome (SS) is characterized by the association of generalized chronic urticaria, osteocondensation and monoclonal IgM gammopathy. Nonsteroidal anti-inflammatory drugs and systemic steroids are the most promising treatments. In our patient, they were ineffective. By contrast, during the follow-up period of 18 months, interferon alpha(2b) therapy (IFN-alpha) relieved the patient from its urticarial lesions and bone pain. IFN-alpha was tried to be stopped twice: each time, relapse of urticaria was noticed and, each time, the cutaneous lesions disappeared after IFN-alpha had been reintroduced. Furthermore, our observation supports the idea of the interleukin (IL)-1-mediated pathogenesis of SS as IFN-alpha induces high levels of IL-1 receptor antagonists. IFN-alpha could be an alternative treatment in disabling SS resisting other drugs. ( info)

9/16. Schnitzler's syndrome with monoclonal IgG kappa gammopathy: good response to cyclosporin.

    Schnitzler's syndrome (SS) is a rare entity characterized by the association of chronic urticaria and monoclonal IgM gammopathy. Usually, intermittent fever, arthralgia and elevation of erythrocyte sedimentation rate also occur. We report a patient with the same symptoms, but with monoclonal IgG instead of IgM paraproteinaemia. Histological examination of the urticarial lesions showed signs of leucocytoclastic vasculitis. After 20 years of therapeutic failure, cyclosporin has achieved a total clearance of urticarial lesions in our patient. Two previous similar cases with clinical features of SS and monoclonal IgG immunoglobulin have been described. We suggest our case also represents a variant of SS with IgG gammopathy. ( info)

10/16. Schnitzler's syndrome with IgG kappa gammopathy.

    A seventy-year-old man with a variant type of Schnitzler's syndrome is reported. physical examination showed pruritic urticarial lesions on the extremities, arthralgia of knee joints, and intermittent fever. Laboratory investigations revealed a high level of IgG, an increased enythrocyte sedimentation rate, urinary Bence-Jones protein, and an M-bow in serum protein electrophoresis, which was shown to be a monoclonal IgG kappa type. Histological examination showed perivascular neutrophil and lymphocytic infiltration into the upper dermis and diffuse neutrophilic infiltration in the middle dermis. One of the clinical features of typical Schnitzler's syndrome is IgM macroglobulinemia, and this is a very rare case of this syndrome with IgG gammopathy. ( info)
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