Woringer-Kolopp disease (pagetoid reticulosis). Four cases with histopathologic, ultrastructural, and immunohistologic observations
D. G. Deneau, G. S. Wood, J. Beckstead, R. T. Hoppe and N. Price
Four patients had localized lesions of Woringer-Kolopp disease (pagetoid
reticulosis). The clinical appearances and histopathologic features with
light microscopy of the four cases were similar. However, electron
microscopy in all four cases and enzyme histochemistry studies and
immunophenotyping with monoclonal antibodies in three cases disclosed two
subgroups of patients. The features of the predominant cells in the
intraepidermal infiltrate of three cases were most consistent with those of
helper T lymphocytes with cerebriform nuclei, as found in many cases of
mycosis fungoides. In one case, however, 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 (ie, within the spectrum of the cutaneous T-cell lymphomas), a
benign reactive process, or a syndrome with various causes.
