Supplementary findings

Schnitzler syndrome

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Supplementary findings

Laboratory investigations

The typical finding is a monoclonal gammopathy, most frequently of the IgM type with kappa light chains. IgG is the variant type.

The erythrocyte sedimentation rate (ESR) is almost invariably elevated, in about 60% of cases accompanied by leukocytosis and / or anaemia.

Bone marrow aspirates must rule out a lymphoproliferative disorder.

Imaging findings

Hyperostosis is revealed by radiologic examination in 40-50% of cases, mostly at the sites of bone pain.

Histologic findings

The histopathologic features of skin biopsies are heterogeneous. Neutrophilic urticaria is the most common finding, consisting of a superficial dermal and perivascular infiltrate of polymorphonuclear cells, mostly neutrophils. In some patients, a superficial perivascular mononuclear infiltrate suggestive of chronic urticaria and lymphocytic inflammation, was found. Urticarial vasculitis was reported in 22 out of 53 patients.

For references see literature page

Last update/check: June 30, 2007

Monoclonal IgM kappa bands in Schnitzler syndrome patient (see arrows).