Pagetoid reticulosis (Woringer‐Kolopp disease)

Abstract

Pagetoid reticulosis (Woringer-Kolopp disease) is a rare subtype of cutaneous CD8-positive T-cell lymphoma. A 41-year-old man presented with a 7-year history with a slowly progressive erythematous plaque on his right buttocks. With the working diagnosis of psoriasis, he was treated with topical corticosteroids which produced no improvement. Histological examination showed an epidermotropic T-cell lymphoma with predominance of CD8- vs.CD4-positive lymphocytes. Based on the clinical picture and the histological findings, we diagnosed pagetoid reticulosis. Excision of the plaque and cream PUVA photo-chemotherapy produced long-term remission.

Woringer-Kolopp (Pagetoid Reticulosis) disease successful response to bexarotene gel

“…WK disease, first described in 1939, usually presents with a slow growing, solitary patch, or plaque on the extremities . The disease has been reported to have a predilection for middle‐age males.…”

“…Woringer–Kolopp (WK) disease (also known as pagetoid reticulosis), is a subtype of cutaneous T‐cell lymphoma that is considered a localized indolent variant of mycosis fungoides (MF) . The presentation of WK can mimic eczema, psoriasis, or various infections . WK disease can also have a variable immunophenotype .…”

A Case of Ketron-Goodman Disease

“…PR is a rare form of cutaneous T-cell lymphoma with a striking epidermotropism on histological examination [3, 4]. The term PR has been introduced by Braun-Falco et al in 1973 to identify this clinical entity [5], first described by Woringer and Kolopp [6] in 1939, for the resemblance of infiltrating cells characterizing this condition with Paget's cells present in the epidermotropic infiltrate of mammary Paget's disease.…”

“…Two clinical variants of PR may be distinguished: a localized form, also known as Woringer-Kolopp disease (WKD), which consists of a single lesion, usually located on the extremities, presenting a good prognosis when it can be totally removed by excision or high-dose radiation, and the generalized type or Ketron-Goodman disease (KGD) with disseminated lesions and a poor prognosis [1, 2]. In the WHO and EORTC classification of cutaneous lymphomas in fact, WKD is classified as a relatively indolent variant of mycosis fungoides (MF), whereas KGD, which is not classified yet, is generally considered an aggressive lymphoma with bad prognosis similar to the aggressive CD8-positive cutaneous T-cell lymphoma, the cutaneous γ/δ-positive T-cell lymphoma and the tumor stage of MF [3]. …”

Nichtepitheliotropes malignes B-Zell-Lymphom mit atypischer Spindelzellmorphologie bei einem Weimaraner

“…Sie ist durch einen ausgeprägten Epidermotropismus, ein lokal begrenztes Auftreten in solitären Veränderungen und sehr langsame Progression gekennzeichnet. Die neoplastischen lymphatischen Zellen befinden sich in nesterartigen Infiltraten in Hohlräumen der akantholytischen Epidermis (12,17).…”