Intravascular lymphoma (IVL) is a rare and aggressive variant of extranodal non-Hodgkin's lymphoma, characterized by almost exclusive proliferation of neoplastic lymphocytes within the blood vessel lumen, and a predilection for the skin and brain ( 1 ). Cases of IVL of T-cell or natural killer (NK)-cell lineage are even rarer. We report here a case of cutaneous intravascular NK-cell lymphoma (IVNKL) presenting with multiple erythematous, indurated, tender plaques over both the lower extremities.
CASE REPORTA 42-year-old Taiwanese woman visited our dermatology clinic with complaints of increasing numbers of erythematous, indurated, tender plaques over both the lower extremities, especially the thighs, for 7 months (Fig. 1 ). Constitutional symptoms, such as malaise, dizziness, and chilly sensations, were also noted. Under the preliminary impression of panniculitis, a biopsy of the skin of the left thigh was performed. A histological study revealed many distended vessels filled with atypical large lymphoid cells in the dermis and subcutis (Fig. 2A). The tumour cells were all confined within the vessels and had large, irregular hyperchromatic nuclei with ample eosinophilic cytoplasm (Fig. 2B). Scattered mitotic figures were found. Fibrin thrombi mixed Fig. I. A 42-year-old Taiwanese woman with increasing number of erythematous indurated tender plaques over both the lower extremities for 7 months.with atypical lymphoid cells were occasionally found. The vessels were surrounded by a small number of non-neoplastic lymphocytes. Immunohistochemical studies showed that the tumour cells were CD3s% CD56% granzyme B*, ßFl , CD4 , CD5 , CD8% CD20 , CD30 , Pax5 , and TdT . Approximately 99% of the tumour cells were Ki-67-positive. In situ hybridization for Epstein-Barr virus-encoded RNA (EBER) revealed the presence of the Epstein-Barr virus (EBV) (Fig. SI, available at