Woringer-Kolopp Disease

Abstract

A typical clinical and histologic case of so-called ‘pagetoid reticulosis’ or Woringer-Kolopp disease is reported. This is a chronic cutaneous condition usually with a single focus or at most with a few patches confined to a circumscribed region. A disseminated form has been described as Dupont-Vandaele type, but its relationship to the classic Woringer-Kolopp disease is uncertain. The nosological location of pagetoid reticulosis has not been established, but there are reasons to regard it as a particular and well-defined entity. In relation to the nature of this condition and to the origin of the ‘pagetoid cells’, it is tempting to consider the possibility that Woringer-Kolopp disease might be a lymphoproliferative epidermotropic disorder, classifiable as a benign form of ‘cutaneous T-cell lymphoma’, but the early histologic changes are rather indicative of an epidermal origin.

Microenvironment of Woringer-Kolopp’s Disease

“…Ryan et al [29] described U cells as originating from Langerhans' cells and evolving towards lymphoid cells; they probably studied the indeterminate cells. Toribio et al [39] suggested the same hypothesis in a case of Woringer-Kolopp's disease, they observed 'Pagetoid' cells comparable to Langerhans' cells, but without the cytoplasmatic Langerhans' granules.…”

“…Some of these cases fit in with the diagnosis of mycosis fungoides [5,8,10,17,19,20,22], Ultrastructural and immu nological studies of this disease have multiplied the hypotheses about the nature of the infiltrating cells. Braun-Falco et al [3] and other authors [1,8,10,17,21,39,41] believe they are T cells.…”