Localized mycosis fungoides with prominent epidermotropism: Woringer-Kolopp disease

Abstract

A patient had a single, large, sharply demarcated lesion on one leg. Under the mistaken diagnosis of malignant melanoma in situ, the lesion was excised and the area grafted in 1956. There has been no recurrence in the past 20 years. Histologic reevaluation led to a diagnosis of mycosis fungoides with pronounced epidermotropism of the cellular infiltrate. This case has close clinical and histologic resemblance with three cases published in Europe and regarded as a special form of reticulosis as first described by Woringer and Kolopp. Mycosis fungoides with marked epidermotropism is not always a benign localized disease but may be widespread and lead to death.

Unilesional Follicular Mycosis fungoides

“…The first case of a solitary lesion of MF has been reported by Woringer and Kolopp [14]in 1940. Woringer-Kolopp disease, also known as localized pagetoid reticulosis, is characterized by a single verrucous plaque with polycyclic borders and central clearing and is now regarded as a benign localized variant of MF with prominent epidermotropism [15]. More recently, other cases of unilesional MF, clinically and histologically different from Woringer-Kolopp disease, have been reported [1, 2, 3, 4, 5].…”

Clinicopathological spectrum of mycosis fungoides

“…WKD has an indolent course. [125][126][127][128][129] MF and WKD have some distinct histopathological and immunophenotypical features. WKD is distinguished histologically from MF in the presence of marked hyperkeratosis, prominent pagetoid epidermotropism of somewhat larger cerebriform cells, and paucicellular dermal infiltrate lacking eosinophils ( fig.…”

“…Finally, in some cases infiltrates in WKD may contain high numbers (> 50%) of CD30+ cells, whereas such CD30 reactivity is never observed in non-transformed MF. 125,126,128,129 The disseminated form of pagetoid reticulosis (Ketron-Goodman disease) is now regarded by many as MF as it shows similar clinical behaviour. Furthermore, some cases reported in the past as Ketron-Goodman disease may in fact represent primary cutaneous CD8-positive epidermotropic cytotoxic T-cell lymphoma.…”

Mycosis-fungoides-Type Cutaneous T Cell Lymphoma of the Hands and Soles: A Variant Causing Delay in Diagnosis and Adequate Treatment of Patients with Palmoplantar Eczema

“…While MF-type CTCL is typically comprised of CD4-positive T lymphocytes [15, 16, 17], tumor cells of localized pagetoid reticulosis display a more variable and heterogeneous phenotype characterized by increased CD4 and/or CD8 positivity [18, 19, 20, 21]or CD4/CD8 negativity [12, 22]. In an approach to reconcile the similarities to MF-type CTCL with the unilesional or paucilesional nature and the favorable clinical course of this disease variant, localized pagetoid reticulosis is increasingly considered part of the disease spectrum of MF, representing the early or benign end of the clinical spectrum [12, 13, 14, 23, 24]. …”