Pagetoid reticulosis (Woringer-Kolopp disease). Histopathologic and ultrastructural observations

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Abstract
Histopathologic and ultrastructural observations in a human case of Pagetoid reticulosis (Woringer and Kolopp disease) are reported. The most important clinical feature of this disease is manifestation as a single, very slowly enlarging verrucoid, plaque-type skin lesion without internal organ involvement. Histologically, the epidermis is extremely acanthotic and densely infiltrated with numerous atypical appearing cells that are entirely absent from the dermis, which simply contains a banal dense chronic inflammatory infiltrate. Ultrastructural study showed that there are 2 major types of unusual cells infiltrating the epidermis. One type is a large lymphoid cell similar to the stimulated lymphocyte. The 2nd type differs from the 1st in having paler staining of the nucleus and cytoplasm and showing cell membrane damage. These cells probably derive by degeneration from the large lymphoid cells. The presence of transitional forms favors this interpretation. Occasionally, degeneration of these cells proceeds to their complete necrosis.

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