SCHNITZLER SYNDROME SUCCESSFULLY TREATED WITH METHOTREXATE

Abstract

Schnitzler syndrome is a rare disorder characterized by a chronic urticarial rash and a monoclonal gammopathy (usually IgM type) accompanied by 2 of the following features: intermittent unexplained fever, arthritis or arthralgia, bone pain, lymphadenopathy, hepatomegaly or splenomegaly, and elevated inflammatory markers. 1 De Koning HD Bodar EJ Simon A van der Hilst JCH Netea MG van der Meer JWM Beneficial response to anakinra and thalidomide in Schnitzler's syndrome. Ann Rheum Dis. 2006; 65: 542-544 Crossref PubMed Scopus (131) Google Scholar , 2 Lipsker D The Schnitzler syndrome: a treatment at last?. Dermatology. 2002; 205: 1-2 Crossref PubMed Scopus (10) Google Scholar This disease was first described in 1972 by L. Schnitzler, a French dermatologist, and since that time, only 60 cases have been reported in the literature. 2 Lipsker D The Schnitzler syndrome: a treatment at last?. Dermatology. 2002; 205: 1-2 Crossref PubMed Scopus (10) Google Scholar The etiology and exact pathogenesis of this disease remain unknown, but cytokines such as interleukin 1 (IL-1) may play an important role. 3 Thonhofer R Uitz E Graninger W Schnitzler's syndrome: exacerbation after anti-TNF treatment. Rheumatology. 2007; 46: 1041-1042 Crossref PubMed Scopus (25) Google Scholar Treatment of this disorder has been challenging and has included the use of nonsteroidal anti-inflammatory agents, high-dose glucocorticoids, thalidomide, interferon alfa, colchicine, IL-1 receptor antagonists, and anti–tumor necrosis factor agents, all prescribed with varying success. 1 De Koning HD Bodar EJ Simon A van der Hilst JCH Netea MG van der Meer JWM Beneficial response to anakinra and thalidomide in Schnitzler's syndrome. Ann Rheum Dis. 2006; 65: 542-544 Crossref PubMed Scopus (131) Google Scholar , 2 Lipsker D The Schnitzler syndrome: a treatment at last?. Dermatology. 2002; 205: 1-2 Crossref PubMed Scopus (10) Google Scholar , 3 Thonhofer R Uitz E Graninger W Schnitzler's syndrome: exacerbation after anti-TNF treatment. Rheumatology. 2007; 46: 1041-1042 Crossref PubMed Scopus (25) Google Scholar , 4 Gach JE Sabroe RA Greaves MW Kobza Black A Methotrexate-responsive chronic idiopathic urticaria: a report of two cases. Br J Dermatol. 2001; 145: 340-343 Crossref PubMed Scopus (72) Google Scholar A definitive treatment remains to be found. We present a case of Schnitzler syndrome successfully treated with weekly methotrexate and low-dose prednisone.