CD8+ cutaneous T-cell lymphoma with pagetoid epidermotropism and angiocentric and angiodestructive infiltration

A 68-year-old woman had a peculiar clinical course of cutaneous lymphoma. She first developed nonpuritic erythematous macules with fine scales followed by development of erythematous infiltrated plaques. The clinical course could be interpreted as that of mycosis fungoides. Histologically, the lesions showed pagetoid infiltration of atypical lymphoid cells. Suddenly, with high fever, numerous purpuric, ulcerated, or crusted plaques with underlying infiltration or nodules began to appear on most areas of the patient's body. Biopsy specimens of the lesions revealed angiocentric and angiodestructive infiltration by neoplastic T cells and marked epidermotropism of these cells. These atypical cells immunohistochemically had CD8+ surface phenotype. The patient died of respiratory insufficiency due to acute pulmonary infiltration. Autopsy demonstrated angiocentric and angiodestructive lymphomatous infiltration in the lung.