Urticarial vasculitis and Schnitzler syndrome.
نویسندگان
چکیده
Both urticarial vasculitis and Schnitzler syndrome are serious diseases but a recently gained better understanding of their underlying pathomechanism allows better treatment options than only a decade ago.
منابع مشابه
Hypocomplementemic Urticarial Vasculitis with Crescentic Glomerulonephritis, Interstitial Nephritis and Small Vessel Vasculopathy: Case Report and Mini-Review
Hypocomplementemic urticarial vasculitis (HUV) is the rare immune complex vasculitis, affecting small vessels and associated with anti-C1q antibodies, presenting with urticaria and hypocomplementemia. Glomerulonephritis, arthritis, obstructive pulmonary disease, and ocular inflammation are common in HUV. HUV may present as an idiopathic disease or be a part of systemic autoimmune or autoinflamm...
متن کاملThe Schnitzler syndrome - a treatment at last?
Accessible online at: www.karger.com/journals/drm L. Schnitzler, a French dermatologist, first described the Schnitzler syndrome in 1972 [1]. It is a disabling chronic disorder defined by the presence of an urticarial eruption and a monoclonal IgM gammopathy as well as at least 2 other signs among the following: fever, bone pain, joint pain, abnormal findings on bone morphological investigation...
متن کاملThe 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...
متن کاملLong-term control of refractory Schnitzler syndrome with anakinra: a case report
Schnitzler syndrome is a rare inflammatory disorder characterized by chronic urticarial rash, monoclonal gammopathy, periodic fever, arthralgia/arthritis and bone pain. However, the results of management of Schnitzler syndrome are often disappointing and its treatment remains a challenge. No cases of spontaneous complete remission have been reported. Anakinra is an interleukin 1 receptor antago...
متن کاملChronic urticaria and monoclonal IgM gammopathy (Schnitzler syndrome): report of 11 cases treated with pefloxacin.
BACKGROUND Schnitzler syndrome is characterized by chronic urticarial rash and monoclonal IgM gammopathy and is sometimes associated with periodic fever, arthralgias, and bone pain. Current treatment is unsatisfactory. OBSERVATIONS Eleven patients with Schnitzler syndrome were treated with oral pefloxacin mesylate (800 mg/d). In 10 patients, we observed a dramatic and sustained improvement of...
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ورودعنوان ژورنال:
- Immunology and allergy clinics of North America
دوره 34 1 شماره
صفحات -
تاریخ انتشار 2014