T-Cell Antigen Deficiencies and Clonal Rearrangements of T-Cell Receptor Genes in Pagetoid Reticulosis (Woringer-Kolopp Disease)

Abstract

PAGETOID reticulosis (Woringer-Kolopp disease) is a rare skin disorder consisting of solitary or localized, often hyperkeratotic, cutaneous plaques. Histologically, the involved areas of skin show a prominent infiltrate of cytologically atypical mononuclear cells within a hyperplastic epidermis.^{1 , 2} Clinically, these lesions are indolent. There have been reports that the condition has resolved in several patients after local surgery or radiation therapy; however, other patients have presented with a disseminated form of pagetoid reticulosis, the Ketron-Goodman variant.^{2 , 3} Some of these patients have also had typical lesions of mycosis fungoides, a form of cutaneous T-cell lymphoma, and have died.^{4 5 6} The nature of the . . .