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Schnitzler syndrome is a rare disease characterised by chronic hives (urticaria) and periodic fever, bone pain and joint pain (sometimes with joint inflammation), weight loss, malaise, fatigue, swollen lymph glands and enlarged spleen and liver. The urticarial rash is non-itching in more than half of cases, which is unusual for hives. It is most prominent on the trunk, arms and legs, sparing the palms, soles, head and neck. Associated angioedema has been reported in a few patients. A review of 94 cases found a mean age at onset of 51 years, and only four patients developed symptoms before the age of 35.〔 The cause and disease mechanism of Schnitzler syndrome remain largely unknown.〔 Schnitzler syndrome is considered an autoinflammatory and autoimmune disorder. Chronic hives and a monoclonal gammopathy have been proposed as the major criteria, while the others represent minor criteria. ==Diagnosis== Blood tests show a high concentration of specific gamma-globulins (monoclonal gammopathy) of the IgM type. It almost always has light chains of the κ-type. A variant in which IgG is raised has been described, which appears to be ten times as rare. The immunoglobulins may show up in the urine as Bence Jones proteins. Signs of inflammation are often present: these include an increased white blood cell count (leukocytosis) and a raised erythrocyte sedimentation rate and C-reactive protein. There can be anemia of chronic disease.〔 Bone abnormalities can be seen on radiological imaging (often increased density or osteosclerosis) or biopsy. Because it is such a rare condition (as of September 2014, only 281 cases have been reported), it is important to rule out other conditions which can cause periodic fevers, paraproteins or chronic hives. These include (and are not limited to) autoimmune or autoinflammatory disorders such as adult-onset Still's disease, angioedema, hematological disorders such as lymphoma or monoclonal gammopathy of undetermined significance, other causes of hives, cryoglobulinemia, mastocytosis, chronic neonatal onset multisystem inflammatory disease or Muckle-Wells syndrome. It is however possible to have more than one rare condition as seen by a patient with Schnitzler's syndrome and cold induced urticaria.〔http://www.ncbi.nlm.nih.gov/pubmed/21886751〕 抄文引用元・出典: フリー百科事典『 ウィキペディア（Wikipedia）』 ■ウィキペディアで「Schnitzler syndrome」の詳細全文を読む スポンサード リンク
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