Schnitzler Syndrome

Schnitzler SyndromeSchnitzler syndrome is characterized by chronic urticaria, nonpruriginoasa in association with recurrent fever, bone pain, arthralgia and arthritis and gammopatie monoclonal immunoglobulin M with a concentration below 10 g / l. Most patients with Schnitzler's syndrome have a chronic benign evolution. Spontaneous remission has not been reported. About 10-15% of patients develop a lymphoproliferative disease, including limfoplasmocitic lymphoma, Waldenstrom macroglobulinemia or myeloma IgM.
All patients with chronic urticaria shows this syndrome, the appellant. Pruritus is absent, but may become slightly itchy lesions over time. There were no identified risk factors. Pathogenesis of the syndrome is not yet well defined.Nonsteroidal anti-inflammatory corticosteroids and immunosuppressants shows a degree of improvement in rash and arthralgia. Skin manifestations respond poorly to antihistamines. They used numerous medications to treat this syndrome but not effective.
Pathogenesis and causes
Exact pathogenesis of Schnitzler syndrome is unclear. It is believed that IgM paraproteinemic storage leads to the formation of immune complexes and complement activation cascade, responsible for skin manifestations. Another proposed theory is uncontrolled activation of interleukin 1.
Signs and symptoms
Schnitzler syndrome patients are aged between 13-71 years at diagnosis. All patients have chronic urticarial rash, appellant. Itching is not common but lesions are poorly pruritigene debut after 2-3 years at 45% of patients. Rash is usually the first symptom that appears affected mostly the trunk and extremities, and avoiding the palms and soles, neck and head.Approximately 90% of patients complain fever recurrence. Each febrile episode usually resolves in a few hours, however, fever may persist for up to 24-48 hours. The episodes can occur daily or infrequently, twice a year. 80% of patients experience pain, bone pain and myalgia 70%. Bone pain affects especially the hip bone and the tibia. Femur, spine, arms and collar bone are rarely involved. Fatigue and weight loss occur in some patients.
Physical examination.Urticarial rash consisting of papules and pink plates, slightly elevated, 0. 5-3 cm in diameter. Injuries occur every day in November. They persist for 12-24 hours and resolve without sequelae. Angioedema is possible but very rare. Lymphadenopathy can be found in 50% of patients, hepatomegaly and splenomegaly 30% to 10%.
There is a set of criteria to classify patients in Schnitzler syndrome:-Recurrent fever, arthralgia or arthritis, bone pain-Lymphadenopathy, hepatosplenomegaly, leukocytosisHigh-ESR, abnormal bone.
Diagnosis
Laboratory studies:-All cases are associated with serum gammopatie to imunoforezaESR and C-reactive protein-high, Leukocytosis, thrombocytosis, anemia-Abnormal lymphoid proliferation in the bone marrow.
Histological examination. Examination of skin samples showed perivascular infiltrates of neutrophils, neutrophilic, lymphocytic inflammation, lymph vessels are intact and slightly superficial dermis shows edema. Note deposit of IgM and complement in the upper dermis and at the junction dermoepidermica.The differential diagnosis is made with the following diseases: lupus erythematosus, acute urticarial vasculitis, chronic idiopathic urticaria, cryoglobulinemia, pressure urticaria.
Treatment
NSAIDs, corticosteroids and immunosuppressive shows an improvement of arthralgia and bone pain but not the eruption. Cutaneous and extracutaneous manifestations respond poorly to H1 and H2 antihistamines. Some patients responded to treatment with thalidomide, rituximab, cloroquina, chlorambucil, cyclophosphamide, azathioprine, plasmapheresis, intravenous immunoglobulin. Phototherapy with psoralen and ultraviolet A small eruption in some patients.It is also used pefloxacin mesylate and anakinra, a recombinant form of natural IL-1 receptor.
PrognosisSchnitzler syndrome require long-term assessment because of the potential of developing lymphoproliferative disease, particularly Waldenstrom macroglobulinemia. No recorded complete spontaneous remission. The prognosis is still good. 10-15% of patients will develop cancer limfoplasmocitar.