PAGETOID

 

RETICULOSIS

 

Pagetoid reticulosis (Woringer-Kolopp disease) is a distinct variant of MF that is characterized by the presence of localized patches or plaques with an intraepidermal proliferation of neoplastic cells. Patients present with a solitary psoriasiform or hyperkeratotic patch or plaque, which is usually localized on the extremities  and is slowly progressive. Unlike in classic MF, extra-cutaneous dissemination has not been observed.

The disseminated form (Ketron-Goodman type) is much more aggressive and is now considered to be an aggressive primary cutaneous epidermotropic CD8⁺ T-

cell lymphoma (see the section Primary Cutaneous Aggressive Epidermotropic CD8⁺ T-Cell Lymphoma).

 

GRANULOMATOUS SLACK SKIN

Granulomatous slack skin is a rare sub-type of MF characterized by the slow development of folds of lax skin in the major skinfolds, with a predilection for the axillae and groins . Light microscopy reveals a dense granulomatous infiltrate in the dermis. In addition to small atypical cells with cerebriform nuclei, macrophages and numerous multinucleated giant T cells occur and palisade around necrobiotic areas, where destruction of the elastic tissue is observed. The neoplastic cells express a CD3⁺CD4⁺CD8^- phenotype.