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PubMedDate not supplied

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.

Humans↗
PubMed2006

Schnitzler syndrome associated with systemic marginal zone B-cell lymphoma.

Schnitzler syndrome is a rare condition defined by chronic urticaria and monoclonal IgM gammopathy. Malignant evolution to Waldenström disease (WD) has been reported in several cases. We report a 49-year-old man who developed a marginal zone B-cell lymphoma (MZL) 3 years after the beginning of Schnitzler syndrome. This is the first report of MZL in association with Schnitzler syndrome. IgM gammopathy is a clue for the diagnosis of Schnitzler syndrome. This condition needs to be closely monitored; it can precede the onset of an authentic lymphoproliferative disorder including WD and rarely MZL.

Cell Transformation, Neoplastic↗
PubMed2000

Schnitzler syndrome: heterogeneous immunopathological findings involving IgM-skin interactions.

The Schnitzler syndrome is the association of chronic urticaria, intermittent fever, osteosclerotic bone lesions and a monoclonal IgM gammopathy. It is not yet firmly established whether the monoclonal immunoglobulin component plays a part in the pathophysiology of the urticarial lesions. Immunoblotting on epidermal and dermal human skin extracts as well as immunoelectron microscopic (IEM) studies on Lowicryl K4M-embedded skin sections were performed in three patients with the Schnitzler syndrome. Western blotting on epidermal extracts showed the presence of IgM-kappa antiskin autoantibodies in two patients. These antibodies displayed the same isotype as the monoclonal components and recognized a 280-290-kDa antigen in one patient and a 200-kDa antigen in the other patient. IEM studies showed sparse IgM deposits in the epidermis, around the keratinocytes, near the desmosomes in one patient and dense deposits below the lamina densa, in the region of the anchoring fibrils, in another patient. Antiskin IgM autoantibodies of the same isotype as their monoclonal gammopathies can be present in the serum of some patients with the Schnitzler syndrome. These IgM antibodies seem to deposit in vivo in the epidermis and at the dermal-epidermal junction, in the region of the anchoring fibrils. These findings suggest that the monoclonal gammopathy plays a part in the pathophysiology of the skin rash. They also suggest patient heterogeneity both in the skin antigens that are recognized as well as in their localization.

Autoantibodies↗
PubMed2001

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.

Adult↗
PubMed1995

Chronic urticaria, arthralgia, raised erythrocyte sedimentation rate and IgG paraproteinaemia: a variant of Schnitzler's syndrome?

Schnitzler's syndrome is a distinct disease entity characterized by the association of chronic urticaria, intermittent fever, arthralgia, elevated erythrocyte sedimentation rate and IgM macroglobulinaemia. We report a patient with the same symptoms, but a monoclonal IgG instead of IgM gammopathy. Histological examination of the urticarial lesions showed signs of mild leucocytoclastic vasculitis. Except for the different class of the monoclonal immunoglobulin, the clinical symptoms, laboratory findings and histology in this patient were identical with those in classical Schnitzler's syndrome. IgG and IgM paraproteins may be equivalent with regard to the putative pathophysiology of the disease process in Schnitzler's syndrome. We therefore suggest that the spectrum of Schnitzler's syndrome is expanded to include patients with chronic urticaria and monoclonal IgG gammopathy, as a closely related variant.

Arthralgia↗
PubMed1993

[Chronic urticaria-macroglobulinemia (Schnitzler syndrome): developing to IgM myeloma. Apropos of a case].

Schnitzler's syndrome has been described in 1972. It associates a chronic urticaria with leukocytoclastic vasculitis, a macroglobulinemia, bone pains, hyperostosis. In the twenty three cases reported, the evolution is benign but three patients including the princeps case developed a Waldenström's disease or a lymphoplasmocytic lymphoma. We present the case of one patient, aged 65, with Schnitzler's syndrome, who, after 6 years evolution, presents a IgM myeloma. Schnitzler's syndrome appeared as a pre-hemopathic state which has to be taken care of regularly and for a long time.

Adrenal Cortex Hormones↗
PubMed2000

[Schnitzler syndrome: a rare cause of systemic urticaria].

INTRODUCTION: The Schnitzler's syndrome first described in 1972, associates urticaria, bone pain, and monoclonal IgM gammapathy. EXEGESIS: A 50-year-old man presented symptoms of urticaria restricted to the trunk and lower members, with episodes of fever accompanied by inflammatory pain in the knees and legs. Slight deterioration of his general condition was also observed. Biological findings showed the existence of an inflammatory syndrome. Electrophoresis and immunoelectrophoresis provided evidence for the existence of underlying IgM gammapathy. Bone X-ray demonstrated the presence of tibial and peroneal metaphysis thickening, with hyperfixation on bone scintigraphy. The patient's condition improved after cortisone and colchicine treatment, allowing decrease in coricosteroid doses. Two years later, except for urticaria, clinical features have disappeared and no hematological disorder has been observed.

Aftercare↗
PubMed1993

[Schnitzler syndrome and Waldenström disease. Fatal outcome of the original case].

The association between chronic urticaria, macroglobulinaemia and various other manifestations has been individualized as Schnitzler's syndrome. We report the terminal course of an original case followed up for 20 years, which ended as lymphoplasmocytic lymphoma with multiple sites, whereas no lymphomatous proliferation, meticulously looked for, had never been found hitherto. The lymphoma, associated with a macroglobulinaemia level above 5 milligrams, was diagnosed as Waldenström disease. Although most cases of Schnitzler's syndrome seem to follow a benign course (but the follow-up is not always long) a few cases have been reported showing evolution towards, or association with, lymphoma. This indicates that the follow-up of patients with Schnitzler's syndrome should be prolonged.

Aged↗
PubMed1993

[Schnitzler syndrome].

A 60-year-old man has been suffering from worsening chronic urticaria for 15 years. In recent years the skin changes have been associated with bone pain, intermittent fever and pruritus. X-rays show localized bone condensation. Immunoelectrophoretic analysis documents an IgM kappa monoclonal gammopathy. Bone biopsy and bone marrow aspirate revealed no atypical lymphoid infiltration. This syndrome was first described by Schnitzler et al. Other cases have been reported, most of them by French authors. The pathogenesis of the syndrome remains unclear although anti-interleukin-1-alpha-antibodies have been found in several cases. Symptomatic treatment with ibuprofen seems promising.

Arthritis↗
PubMed1996

[The Schnitzler syndrome as a cause of recurrent fever of unknown origin].

HISTORY AND CLINICAL FINDINGS: For 4 years a 56-year-old woman had been suffering from chronic urticaria. In the past two years she had developed recurrent fever, each 1-3 days in duration. In the last 6 to 8 months she also had severe aching in the legs. There were no significant findings on physical examination other than non-itching urticarial rash over trunk and limbs. INVESTIGATIONS: Blood sedimentation rate was increased (maximum 88/110 mm), as were WBC count (16,200/microliter, 83% neutrophils) and the activity of alkaline leucocyte phosphatase (225 U/l). Monoclonal IgM gammopathy type kappa and circulating IgM immune complex (15.4 mg/dl) were demonstrated in serum. Skin biopsy of a new urticarial lesion revealed vasculitis. Iliac crest biopsy was unremarkable and showed no lymphoid cell infiltration. Bone scintigraphy revealed bilaterally increased storage in femur and tibia. Magnetic resonance imaging demonstrated marrow infiltration without space-occupying features in the affected femur and tibia. TREATMENT AND COURSE: Conjunction of fever, generalised urticaria, joint/bone pain and monoclonal gammopathy of kappa type having established the diagnosis of Schnitzler's syndrome, treatment with ibuprofen was started (initially 1,200 mg daily by mouth, followed by reduction to 600 mg daily). All symptoms rapidly improved and the bone changes regressed, but not the monoclonal gammopathy. But when the ibuprofen dosage had been decreased to 600 mg daily, the urticaria recurred, though in milder form. CONCLUSION: This case demonstrates the efficacy of ibuprofen in the treatment of the Schnitzler's syndrome. Furthermore reversibility of scintigraphic bone lesions could be demonstrated under ibuprofen treatment.

Anti-Inflammatory Agents, Non-Steroidal↗
PubMed1992

[Systemic urticaria, sclerosing osteopathy, monoclonal gammopathy (Schnitzler syndrome). Apropos of a case].

A 65 years old man has been suffering from the upper limbs and legs for 2 years. Bone pain was associated with a non pruritic chronic urticaria and fever. Bone scintigraphy showed lesions of the lower limbs. X rays showed only one sclerotic bone lesion. Immunoelectrophoretic analysis showed an IgM kappa monoclonal gammapathy. Bone marrow aspirate and bone marrow biopsy were normal. The association of chronic urticaria, bone pain and macroglobulinemia has been first reported by L. Schnitzler. Seventeen other cases have been reported in the literature. The pathogenesis of the syndrome is unknown although anti-interleukin 1 alpha autoantibodies were found in several cases (8 out of 13).

Aged↗