Schnitzler's syndrome: monoclonal gammopathy associated with chronic urticaria.
Schnitzler's syndrome (SS) is defined by monoclonal gammopathy and chronic urticaria combined with at least two of the following features: fever, arthralgia or arthritis, bone pain, hepato- and/or splenomegaly, palpable lymph nodes, elevated ESR, and leukocytosis. We report a 49-year-old man with monoclonal IgM gammopathy and a 4-year history of recurrent urticarial rash, unexplained fever and arthralgias. The skin biopsy from an acute lesion revealed perivascular lymphocytic infiltrates consisting of CD4+ and CD8+ T lymphocytes. To our knowledge, this is the first report of an immunophenotypic characterization of skin infiltrates in SS. A lower CD4+/CD8+ ratio of circulating T lymphocytes was also detected. SS usually has a benign course, but in 15% of patients a lymphoproliferative disorder develops.
Rizzi
Rita
R
Hematology Section, Department of Pathology, University of Bari Medical School, Bari, Italy. ritarizzi@ematba.uniba.it
Curci
Paola
P
Rinaldi
Erminia
E
Rinaldi
Francesco
F
Cimmino
Antonia
A
Ricco
Rosalia
R
Specchia
Giorgina
G
Liso
Vincenzo
V
D016516:CD4-CD8 Ratio; D002908:Chronic Disease; D006801:Humans; D007075:Immunoglobulin M / Q000097:blood; D008264:Macrophages / Q000473:pathology; D008297:Male; D008875:Middle Aged; D010265:Paraproteinemias / Q000150:complications* / Q000276:immunology; D019873:Schnitzler Syndrome / Q000175:diagnosis* / Q000276:immunology / Q000473:pathology; D012867:Skin / Q000276:immunology / Q000473:pathology; D016176:T-Lymphocyte Subsets / Q000276:immunology / Q000473:pathology; D014581:Urticaria / Q000150:complications* / Q000276:immunology / Q000473:pathology
D007075:Immunoglobulin M