Purpura and leg ulcers in a patient with cryoglobulinaemia, non-Hodgkin's lymphoma, and antiphospholipid syndrome

Abstract

We report a 69-year-old Caucasian female with non-Hodgkin's lymphoma who subsequently developed leg ulcers as a manifestation of the antiphospholipid syndrome. Investigations revealed a mixed cryoglobulinaemia with monoclonal IgM-kappa and antiphospholipid activity with anticardiolipin antibodies, antimitochondrial type M5 antibodies and lupus anticoagulant. Significantly increased concentration of anticardiolipin antibodies was detected in the cryoprecipitate. Our case illustrates a connection between cryoglobulinaemia and lymphoproliferative and autoimmune disorders. Both cryoglobulins and anticardiolipin antibodies could participate in the vascular damage. Cutaneous manifestations in the presence of these disorders associated with non-Hodgkin's lymphoma have not been described previously.

Antineutrophil cytoplasmic antibody (ANCA)-associated autoimmune diseases induced by antithyroid drugs: comparison with idiopathic ANCA vasculitides

“…Simultaneous presence of cryoglobulins, aCL and anti-β2 GP I antibodies [42] in our two MPO-ANCA-positive patients with LLD synergistically contributed to development of cutaneous necrosis. However, we can assume that in patients with ISV, absolute or relative deficiency of α1 AT (Table 3), which is a specific PR3 inhibitor, could play a role in necrosis.…”

Painful ulcerations: the sole clinical sign of mixed cryoglobulinaemia secondary to marginal zone lymphoma

“…While ulcerations with mixed cryoglobulinaemia has been reported in the literature, most cases also presented with extracutaneous clinical manifestations, multisystem involvement or HCV infection. Of 44 results identified from a PubMed search of case reports, combining the keywords ‘mixed cryoglobulinaemia’ and ‘ulcers’ through the Boolean Operator AND, only four remained after excluding reports that were non-English, were type 1 cryoglobulinaemia, had extracutaneous or multisystem involvement on diagnosis or known HCV 6–9. A case with chronic perianal skin ulceration as the presenting manifestation later developed peripheral neuropathies in his hands and feet 6.…”

Influence of inherited and acquired thrombophilic defects on the clinical manifestations of mixed cryoglobulinaemia

“…Necrotizing skin ulcers can be the only manifestation of vasculitis in patients with mixed cryoglobulinaemia [6,7,[39][40][41]. Skin ulcers in these patients are difficult to treat by local [40] and systemic [6,41] therapies, and therefore novel preventive and therapeutic strategies are appealing. In this regard, our finding of a contribution of hyperhomocysteinaemia to the cutaneous manifestations of cryoglobulinaemia may have practical implications.…”