Primary cutaneous acral CD8+ T-cell lymphoma (acral CD8+ TCL) is a rare form of cutaneous T-cell lymphoma characterized clinically by a solitary, slow-growing nodule arising most commonly on the ear. Other peripheral locations such as the nose, hands, and feet can be primary sites. Bilateral, symmetrical presentation has been reported, but multifocal presentation is rare. The solitary nodule most often lacks ulceration.

Acral CD8+ TCL typically presents in adults aged 50 and older, with a median age at diagnosis of 54. Males are more commonly affected than females.

Overall, prognosis of acral CD8+ TCL is excellent with a 5-year survival rate of 100%. It has an indolent course, and no staging investigations have shown progression to systemic disease during follow-up periods of up to 10 years.