Intravascular large B-cell lymphoma is a rare disease with multiorgan involvement that also affects the skin. Skin manifestations include purpuric to red macules, plaques, or nodules with occasional edema and tenderness. We report a 68-year-old woman with bilateral leg edema and occasional high fever. A biopsy specimen from a subcutaneous nodule showed that the blood vessels in the dermis and subcutaneous tissue were filled with irregularly shaped chromatin-rich large atypical lymphocytes positive for CD20 and bcl-2, consistent with the diagnosis of intravascular large B-cell lymphoma. In addition, immunohistochemical analysis showed expression of CXCR3 in the atypical lymphocytes; its ligand, CXCL9, was detected in blood vessels. Although limited to a single case, our study could provide a possible new clue to the pathogenesis of intravascular large B-cell lymphoma by virtue of the characteristic expression of CXCL9-CXCR3.
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