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Urticarial vasculitis associated with a monoclonal IgM gammopathy: Schnitzler's syndrome
Author(s) -
BORRADORI L.,
RYBOJAD M.,
PUISSANT A.,
DALLOT A.,
VEROLA O.,
MOREL P.
Publication year - 1990
Publication title -
british journal of dermatology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 2.304
H-Index - 179
eISSN - 1365-2133
pISSN - 0007-0963
DOI - 10.1111/j.1365-2133.1990.tb01831.x
Subject(s) - isotype , medicine , gammopathy , vasculitis , immunology , pathology , paraproteinemias , monoclonal antibody , antibody , rheumatoid factor , monoclonal , disease
summary 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, C 3 and, to a lesser degree, of IgG were observed in the walls of small blood vessels.