A CASE OF WORINGER-KOLOPP DISEASE WITH KI-1 (CD30)+ CYTOTOXIC SUPPRESSOR CELLS

Background.- Woringer-Kolopp (W-K) disease is a rare, localized, histologically malignant, but clinically indolent lymphoproliferative disorder. Most authors have regarded W-K disease as a variant of mycosis fungoides. However, a recent case suggests that W-K disease may represent a spectrum of lymphoproliferative disorders that may not be related to mycosis fungoides. Observations.- A patient with a 40-year history of a localized cutaneous eruption characterized by markedly atypical epidermotropic lymphocytes was seen at Stanford (Calif) University Hospital. The lymphocytes were predominantly CD30+ cytotoxic/suppressor T cells, an immunophenotype not previously described in W-K disease. Genotype analysis revealed a clonal rearrangement. Conclusions.- The findings in our patient, along with a review of all cases previously reported in the literature, suggest that W-K disease may bean entity with a uniform clinical and histologic presentation, but one with marked immunophenotypic heterogeneity of the malignant-appearing atypical cells. Some cases showed immunophenotypic similarities to mycosis fungoides. However, in almost half of the reviewed cases, including the one presented here, the immunophenotypic differences exceeded the similarities.