Urticarial vasculitis associated with a monoclonal IgM gammopathy: Schnitzler's syndrome.
We describe two cases of Schnitzler's syndrome presenting with urticarial vasculitis, in which there was a monoclonal IgM of kappa light-chain isotype. Skin histology showed a perivascular leucocytic infiltrate and leucocytoclasis. One patient had an abnormal complement profile and positive rheumatoid factor while the other had antibodies to unidentified soluble nuclear antigens, anti-cytoplasmic antibodies and circulating immune complexes. In addition, in the first case deposits of IgM, C3 and, to a lesser degree, of IgG were observed in the walls of small blood vessels.
Borradori
L
L
Dermatology Clinic, Hôpital Saint Louis, Paris, France.
Rybojad
M
M
Puissant
A
A
Dallot
A
A
Verola
O
O
Morel
P
P
D000368:Aged; D002908:Chronic Disease; D006801:Humans; D007147:Immunoglobulin Light Chains / Q000032:analysis; D007075:Immunoglobulin M / Q000032:analysis*; D007145:Immunoglobulin kappa-Chains / Q000032:analysis; D008297:Male; D008875:Middle Aged; D010265:Paraproteinemias / Q000276:immunology / Q000473:pathology*; D013577:Syndrome; D014581:Urticaria / Q000276:immunology / Q000473:pathology*; D014657:Vasculitis / Q000276:immunology / Q000473:pathology*
D007147:Immunoglobulin Light Chains; D007075:Immunoglobulin M; D007145:Immunoglobulin kappa-Chains