What can vasculitic leg ulcers implicate?

Dear Editors, A 76-year-old woman presented with multiple irregular necrotic leg ulcers surrounded by palpable purpurae on the right leg, persisting for 2 years (Figure 1). Three years before, her left leg was amputated because of ulceration and concomitant septicemia. The patient had pallor, xerostomy, xerophthalmia, distal limb numbness without parotido-, lymphadenoor hepatomegaly. Haematoxylin–eosin-stained sections of the ulcer showed leucocytoclastic vasculitis with erythrocyte extravasation; direct immunofluorescence showed granular IgG and complement factor 3 depositions. Laboratory tests showed marked hypoproteinaemia, hypoalbuminaemia, elevated erythrocyte sedimentation rate, mild pancytopenia, high carbamide, creatinine, uric acid and C-reactive protein levels. Proteinuria, microscopic haematuria, granular, hyalin and cell cylinders suggested glomerular involvement. Autoimmune serology found positive anti-nuclear antibody, a robust elevation of anti-SS-A and rheumatoid factor and a moderate increase in anti-SS-B levels. Cryoprecipitation showed massive cryoglobulinaemia. Immunofixation electrophoresis detected non-quantifiable IgMκ biclonal and IgMλ monoclonal bands, and serum-free light chain assay showed very high κ level and abnormal κ:λ ratio (Table 1). Review of CT images revealed splenomegaly, but no lytic bone lesions related to multiple myeloma. Ultrasound and CT examinations showed bilateral parenchymal atrophy of the kidneys. Bone marrow trephine biopsy revealed discrete interstitial B-cell population suggestive of indolent B-cell lymphoproliferation, but no evidences of B-cell monoclonality was detected by PCR-based immunoglobulin heavy chain gene rearrangement analysis.