hypergammaglobulinemia/ödem

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The syndrome of hypergammaglobulinemia M, eosinophilia, edema, and fatigue. Immunologic studies and long-term evaluation of two patients.

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2 patients with an elevated serum IgM, eosinophilia, nonpitting edema, and fatigue previously reported, were reevaluated. Their clinical course remains relatively benign without evidence of organ damage after 19 patient years of evaluation. Corticosteroid therapy suppresses clinical manifestation,

[Ascites and edema of the lower extremities (with hypergammaglobulinemia): myeloma (highly differentiated type with Russell bodies)].

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Primary Sjögren syndrome: report of a 10 years old girl with local edema and positivity of anti SS-A and anti SS-B autoantibodies.

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Sjogren-Larsson syndrome (SLS) is an autoimmune disease, uncommon in childhood. We report a case of SLS in a 10-year-old girl with a history of tumor, calor and rubor in the back of her toes almost every month, which resolved in 4-5 days without therapy. She did not complain of dry mouth or dry

[TAFRO syndrome and monoclonal gammapathy: Uncommon association!].

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Castleman's disease is a lymphoproliferative disorder characterized by angiofollicular lymph node hyperplasia. Recently, a new variant of multicentric Castleman's disease has been identified in Japan called TAFRO syndrome. It is characterized by a constellation of symptoms: thrombocytopenia,

Eosinophilic fasciitis: an atypical presentation of a rare disease.

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Eosinophilic fasciitis, or Shulman's disease, is a rare disease of unknown etiology. It is characterized by peripheral eosinophilia, hypergammaglobulinemia, and high erythrocyte sedimentation rate. The diagnosis is confirmed by a deep biopsy of the skin. The first line of treatment is

Rapidly progressive glomerulonephritis in a patient with Chlamydia pneumoniae infection: a possibility of superantigenic mechanism of its pathogenesis.

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Herein we describe a case of a patient with rapidly progressive glomerulonephritis after Chlamydia pneumoniae infection. An 88-year-old woman who had had C. pneumoniae infection two months previously was admitted to our hospital with complaints of dyspnea and generalized edema. Laboratory tests

Generalized pustular drug eruptions: confirmation by in vitro tests.

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BACKGROUND Generalized pustular eruptions are characterized by acute onset of aseptic pustules in febrile patients with leukocytosis after exposure to the offending drug. They have been regarded as uncommon manifestations of adverse drug reactions. Until now few confirmation studies have been

Acute interstitial nephritis with polyclonal B cell infiltration and development of mantle cell lymphoma.

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A 69-year-old man with proteinuria, hematuria and abnormal renal and hepatic function developed acute renal failure. His clinical and laboratory examinations showed splenomegaly, edema, anemia and polyclonal hypergammaglobulinemia with high IgG levels. Renal biopsy showed interstitial nephritis with

Bullous morphea profunda.

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A deep sclerotic process developed on the shins of a 58-year-old man, and eosinophilic fasciitis or morphea profunda was suspected clinically. Bullae later arose on the plaques, and histologic examination of a skin biopsy specimen revealed sclerosis and inflammation of the deep dermis, panniculus,

[A case of lupus erythematosus preceded by right heart failure due to pulmonary hypertension].

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A 40-year-old woman was admitted because of increasing exertional dyspnea. Right heart failure was suggested by the presence of hepatomegaly, pretibial edema and also echocardiographic findings. Physical examination and echocardiography showed no evidence of valvular disease or congenital heart

Idiopathic multicentric Castleman's disease. A clinicopathologic and immunohistochemical study of five cases.

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Several lymphoproliferative disorders may be interpreted as multicentric Castleman's disease (MCD) clinicopathologically. These include HIV infection, autoimmune-disease-associated lymphadenopathy, idiopathic plasmacytic lymphadenopathy with polyclonal hyperimmunoglobulinemia, "idiopathic MCD",

[Serum and secretory immunoglobulins in allergic diseases].

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A total of 158 patients with pollinosis, bronchial asthma, urticaria and Quincke's edema were examined. The immunoglobulin and C3 levels in sera and the immunoglobulin and albumin levels in saliva were determined by the method of single radial immunodiffusion with the corresponding monospecific

Eosinophilic fasciitis: Current concepts.

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Eosinophilic fasciitis (EF) is an uncommon connective tissue disease characterized by abrupt onset of edema, followed by progressive induration of primarily the distal extremities. Patients may exhibit inflammatory arthritis, joint contractures, decreased mobility, and nerve entrapment. Almost half

A child with primary Sjögren syndrome and a review of the literature.

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Primary Sjögren syndrome (pSS) is an uncommon disease in childhood. Childhood pSS might have different clinical manifestations than adult pSS. We describe a 13-year-old girl with multiple episodes of bilateral parotid swelling lasting 2 years. Her history included severe arthralgia, local edema, and

Perinatal manifestation of mevalonate kinase deficiency and efficacy of anakinra.

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BACKGROUND Mevalonate kinase deficiency is a metabolic autoinflammatory syndrome caused by mutations in the MVK gene, mevalonate kinase, the key enzyme in the non-sterol isoprenoid biosynthesis pathway. Two phenotypes of mevalonate kinase deficiency are known based on the level of enzymatic

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