Pagetoid reticulosis (Woringer-Kolopp disease): An immunophenotypic, molecular, and clinicopathologic study

Pagetoid reticulosis (PR), also known as Woringer-Kolopp disease, is a form of cutaneous T-cell lymphoma that demonstrates striking epidermotropism on histologic examination. We present the histologic, immunologic, and molecu lar findings for seven patients who had PR. The patients ranged in age from 33 to 67 years. AU patients presented with one or several thick plaques in volving the distal extremities except for one patient, who presented with a tongue lesion. Immunohistochemical staining of the atypical lymphoid cells demonstrated a T-cell phenotype in all cases. In one of four frozen cases, the neoplastic cells were of T-helper cell phenotype (CD4 positive). Four of seven cases demonstrated a T-cytotoxic/suppressor cell phenotype (CD8 po sitive). The T-cell subset for the remaining two cases could not be determi ned. CD30 positivity and a high growth fraction as indicated by staining wi th Ki-67 were seen in three of seven and three of four cases, respectively. Genotypic analysis performed on three of our cases revealed T-cell recepto r (gamma and/or beta) rearrangement, indicating a clonal proliferation. The clinical follow-up ranged from 15 months to 13 years. Four of seven patien ts are alive and free of disease after treatment with excision or local irr adiation. One patient relapsed twice after treatment with radiation and pho tochemotherapy with 8-methoxypsoralen and UVA and was then lost to follow-u p. The lesions of another patient resolved spontaneously but recurred at th e same and in an additional site 5 years later. One patient recurred after electron beam therapy. The recurrent lesion improved with radiation therapy and local wound care but never resolved completely, The patient died of un related causes. Our findings suggest that PR is a distinct clinicopathologi c entity, separate from unilesional mycosis fungoides, demonstrating a slow disease course. The disease is a clonal cutaneous T-cell lymphoma with rel atively consistent clinical and histopathologic findings but a heterogeneou s immunophenotypic profile.