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Although symptoms can be a nuisance, the condition does not lead to serious disease in the majority of patients. However about 15% of patients progress to a lymphoproliferative disorder such as Waldenström macroglobulinemia or B-cell lymphoma. Additional nonspecific symptoms that have been reported in individuals with Schnitzler syndrome include unintended weight loss, fatigue and a general feeling of poor health (malaise). Rapid swelling due to fluid accumulation just beneath the surface skin (angioedema) is very rare.
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Symptoms associated with Schnitzler syndrome may include repeated bouts of fever, joint inflammation (arthritis), joint pain (arthralgia), bone pain, and other findings such as enlarged lymph nodes (lymphadenopathy).
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… 2018-02-14 · The disorder was first described in 1972 by the French dermatologist Liliane Schnitzler, who subsequently identified five patients with hives, episodes of prolonged fever, bone pain and enlarged Schnitzler syndrome. Summary: Schnitzler syndrome is a rare autoinflammatory condition.Signs and symptoms of the condition vary but may include urticaria; recurrent fevers; joint pain and inflammation; organomegaly (abnormally enlarged organs); and/or blood abnormalities. Se hela listan på emedicine.medscape.com Se hela listan på ctajournal.biomedcentral.com Schnitzler syndrome or Schnitzler's syndrome is a rare disease characterised by onset around middle age of chronic hives (urticaria) and periodic fever, bone pain and joint pain (sometimes with joint inflammation ), weight loss, malaise, fatigue, swollen [en.wikipedia.org] Schnitzler syndrome is an acquired autoinflammatory disease sharing similar symptoms with periodic fever syndromes, in particular the cryopyrin-associated periodic syndrome.
Red rash with raised patches of skin (urticaria)
Schnitzler syndrome is a rare disorder characterized by a chronic reddish rash Symptoms associated with Schnitzler syndrome may include repeated bouts of
Nov 26, 2020 Objective: To determine ex vivo cytokine profiles of Peripheral Blood Mononuclear Cells (PBMCs) from SchS patients prior to treatment and after
Schnitzler syndrome is an acquired autoinflammatory disease sharing similar symptoms with periodic fever syndromes, in particular the cryopyrin-associated
Mar 17, 2020 Background: Schnitzler syndrome (SchS) is a rare acquired systemic autoinflammatory disease. The major clinical features of SchS are
Schnitzler's syndrome usually follows a benign course, although in a few patients evolution to a malignant lymphoproliferative disorder was reported [3].
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Schnitzler syndrome is characterized by chronic, recurrent, urticarial (hives) eruption: Occurs in all patients but there is a slight male predominance. The mean age of disease onset is 51 years. The first clinical sign is usually a mildly or non-pruritic (non-itchy) skin rash. Schnitzler syndrome is a very rare acquired systemic disease with many similarities to hereditary autoinflammatory syndromes. The main characteristics are generalized exanthema and a monoclonal gammopathy with IgM. Other clinical features include fever, muscle, bone and/or joint pain, and lymphadenopathy.
It is uncertain what causes Schnitzler syndrome, but it does not appear to be hereditary.
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Labs: increased WBC, sed rate, CRP. High IgM with a IgM monoclonal gammopathy on immunofixation.