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 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 be an 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.
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 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 be an 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.