Primary cutaneous acral CD8+ T-cell lymphoma is a rare cutaneous lymphoma that follows an indolent course and which can be managed conservatively.

Clinical features:

(1) usually affects older adults (age > 50 years)

(2) usually arises on a peripheral site (ear, nose, hands and/or feet)

(3) may be solitary or bilateral

(4) slow-growing and indolent

(5) without preceding patches and plaques (preceding lesions seen with mycosis fungoides)


Histologic findings:

(1) small to medium-sized CD8 positive lymphocytes

(2) diffuse dermal infiltrate with a Grenz zone from uninvolved epidermis (not epidermotropic)

(3) monoclonality

(4) CD4-negative with aberrant loss of T-cell antigens

(5) Ki-67 low (< 10%), occasionally moderate

(6) CD68 positive in Golgi apparatus (dot-like perinuclear positivity)

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