D. Nashan et al., CHRONIC URTICARIA, ARTHRALGIA, RAISED ERYTHROCYTE SEDIMENTATION-RATE AND IGG PARAPROTEINEMIA - A VARIANT OF SCHNITZLERS SYNDROME, British journal of dermatology, 133(1), 1995, pp. 132-134
Schnitzler's syndrome is a distinct disease entity characterized by th
e association of chronic urticaria, intermittent fever, arthralgia, el
evated erythrocyte sedimentation rate and IgM macroglobulinaemia. We r
eport a patient with the same symptoms, but a monoclonal IgG instead o
f IgM gammopathy, Histological examination of the urticarial lesions s
howed signs of mild leucocytoclastic vasculitis. Except for the differ
ent class of the monoclonal immunoglobulin, the clinical symptoms, lab
oratory findings and histology in this patient were identical with tho
se in classical Schnitzler's syndrome. IgG and IgM paraproteins may be
equivalent with regard to the putative pathophysiology of the disease
process in Schnitzler's syndrome. We therefore suggest that the spect
rum of Schnitzler's syndrome is expanded to include patients with chro
nic urticaria and monoclonal IgG gammopathy, as a closely related vari
ant.