Cutaneous features of intravascular lymphoma

Intravascular lymphoma (IVL) is a subset of extranodal non-Hodgkin lymphoma, with an estimated incidence of <1 case per million people. It is characterised by extensive proliferation of lymphoma cells within small to medium-sized blood vessels. Most IVLs are B-cell tumours. IVL can present primarily in any organ system, including the skin. The disease is often disseminated at diagnosis. The overall mortality rate is thought to be >80%, and >50% of patients are diagnosed at postmortem examination. There is wide variability in the clinical appearance of cutaneous lesions, which may simulate inflammatory skin disease. Therefore, awareness by dermatologists is important to enable early diagnosis when cutaneous signs are present. We report two patients with unexplained systemic disease and a skin eruption, leading to the diagnosis of IVL, and outline the range of cutaneous features reported.