Although symptoms can be a nuisance, the condition does not lead to serious disease in the majority of patients. Monoclonal igm gammopathy is the main pathological feature that leads to symptoms while a chronic relapsing course is usually observed. 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 lymph glands and enlarged spleen and liver. Abstract the schnitzlers syndrome is defined by a unique constellation of clinical and biological signs including chronic urticaria, intermittent fever, bone pain, arthralgia or arthritis, and a monoclonal igm. Pastor ma, vargasmachuca i, carrasco l, fariaa mc, martan l, requena l. List of schnitzler syndrome medications 1 compared. The following list of medications are in some way related to, or used in the. Schnitzler syndrome is a rare and underrecognized syndrome characterized by chronic urticaria, a monoclonal protein, and a variety of. Aug 30, 2015 the longterm outlook prognosis for people with schnitzler syndrome is generally good. Schnitzler syndrome is a rare disorder characterized by a chronic reddish rash that resembles hives urticaria and elevated levels of a specific protein in the blood monoclonal igm gammopathy. Between 1972 and 1974, schnitzler reported on the association between chronic urticaria and an igm monoclonal protein that ultimately led to the recognition of a distinct clinical syndrome that bears her name. Schnitzler syndrome genetic and rare diseases information. However, approximately 1015% of people with schnitzler syndrome develop a lymphoproliferative disorder. Schnitzler syndrome the classic feature of schnitzler syndrome is the chronic, urticarial rash that does not generally present as itchy pruritic, but can progress to being more pruritic.
This study summarizes the clinical, biological, histological, and morphological data of all the patients with a schnitzler syndrome reported to date. Schnitzler syndrome is a chronic condition, and it has not been reported to resolve. Schnitzler syndrome is a very rare immunological disease. The longterm outlook prognosis for people with schnitzler syndrome is generally good. Mar 12, 2019 schnitzler syndrome is an autoinflammatory disease for which the exact pathophysiology remains unclear. However about 15% of patients progress to a lymphoproliferative disorder such as waldenstrom macroglobulinemia or bcell lymphoma. The rash generally is present on the torso, arms and legs, but is not often seen on the head, neck, palms of the hands, or soles of the feet. The schnitzler syndrome is characterized by a recurrent febrile rash, joint andor bone pain, enlarged lymph nodes, fatigue, a monoclonal igm component, leucocytosis and systemic inflammatory response. Presenting signs and symptoms are an urticarial rash, intermittent fevers, myalgia, arthralgia, bone pain, lymphadenopathy, and peripheral neuropathy. Schnitzlers syndrome is an autoinflammatory disorder characterized by the association of a monoclonal igm or igg gammopathy, a chronic urticarial rash, and signs and symptoms of systemic. Schnitzler syndrome chronic urticaria with gammopathy. Schnitzler syndrome nord national organization for rare.
Increased levels of several members of the cytokine il1 family have been found. Entidade clinica a ser considerada em doentes com urticaria cronica introducao. Schnitzler s syndrome is an autoinflammatory disorder characterized by the association of a monoclonal igm or igg gammopathy, a chronic urticarial rash, and signs and symptoms of systemic. Dec 08, 2010 the schnitzler syndrome is characterized by a recurrent febrile rash, joint andor bone pain, enlarged lymph nodes, fatigue, a monoclonal igm component, leucocytosis and systemic inflammatory response. Sindrome di schnitzler, primi risultati positivi per. The rash generally is present on the torso, arms and legs, but is not often seen on. Cravo, m goncalo, margarida vieira, r brites, mm coelho, s figueiredo, a.