Woringer-Kolopp disease (pagetoid reticulosis). Four cases with histopathologic, ultrastructural, and immunohistologic observations

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Abstract
Four patients had localized lesions of Woringer-Kolopp disease (pagetoid reticulosis). The clinical appearances and histopathologic features with light microscopy of the 4 cases were similar. EM in all 4 cases and enzyme histochemistry studies and immunophenotyping with monoclonal antibodies in 3 cases disclosed 2 subgroups of patients. The features of the predominant cells in the intraepidermal infiltrate of 3 cases were most consistent with those of helper T lymphocytes with cerebriform nuclei, as found in many cases of mycosis fungoides. In 1 case, the epidermis contained a predominant population of small, medium and large lymphoid cells of cytotoxic/suppressor T-lymphocyte cell phenotype. It remains to be determined whether Woringer-Kolopp disease is a variant of mycosis fungoides (i.e., within the spectrum of the cutaneous T-cell lymphomas), a benign reactive process or a syndrome with various causes.

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