BACKGROUND: Intravascular lymphoma (IVL) is fatal when it is diagnosed late in the course. Sometimes skin lesions enable early diagnosis, but criteria for diagnosis are not well established. OBJECTIVES: To demonstrate the clinical spectrum of skin lesions of IVL and to correlate it with clinical outcome; to identify features differentiating between B-cell and T-cell IVL with skin involvement. METHODS: Review of 97 articles reporting on total of 224 patients with IVL. RESULTS: Skin lesions were mentioned in 90 of 224 patients. They were nodules and/or plaques (49%) or macules (22.5%) of red (31%) or blue to livid (19%) colour on the leg (35%), the thigh (41%) and the trunk (31%). Telangiectases were present in only 20% of the patients. Oedema (27.5%) of the legs and pain (24%) were often accompanying. No criteria enabled distinction between lesions restricted to the skin and skin lesions concurrent with IVL in other organs, but when the disease was restricted to the skin, the prognosis was favourable (10% vs. 85% fatal outcome). Skin lesions of T-cell IVL are indistinguishable from those of B-cell IVL. CONCLUSIONS: Forty per cent of all patients with IVL have skin lesions, these being red, sometimes painful plaques located typically on the lower extremities, accompanied by oedema. A clinician risks misinterpreting these changes as thrombophlebitis, erythema nodosum or erysipelas. Neither clinical course nor differentiation of the lymphoma can be predicted from the morphology of skin lesions, but involvement of other organs at the time of diagnosis indicates a poor prognosis.
