In the older literature, the condition was divided into localized disease (Woringer-Kolopp disease) and generalized disease (Ketron-Goodman disease). Nowadays, the latter is thought to represent a variety of lympohmas including epidermotropic CD8-positive cutaneous T-cell lymphoma, cutaneous γ/δ T cell lymphoma and mycosis fungoides.

Clinical features

•Very rare indolent form of CTL classified as a variant of MF

•Presents as a solitary, slowly growing, scaly, sharply delineated erythematous lesion

•Up to 30.0 cm diameter

•Predilection for the lower legs but can present elsewhere

Histological features

•Hyperkeratosis/parakeratosis

•Acanthosis often psoriasiform

•Very marked epidermotropic infiltrate of atypical lymphocytes including Sézary cells

•Perinuclear halo often present

•Pautrier-like microabscesses

•Variable mitotic activity

•Adnexal involvement

•Superficial dermal perivascular lymphohistiocytic infiltrate with only sparse or absent atypical forms

•CD45, CD2, CD3, CD5

•CD7 often reduced or lost

•CD4+ve or CD8+ve variants

•Variable CD30 & Ki-67 expression

•Monoclonal TCR