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Pathology of Pagetoid Reticulosis (Woringer-Kolopp disease)

 Dr Sampurna Roy MD           


Dermatopathology Quiz Case 189

Diagnosis: Pagetoid Reticulosis (Woringer-Kolopp disease)




Solitary Pagetoid reticulosis (PR), also known as Woringer-Kolopp disease, is a distinct variant of mycosis fungoides.

Clinically, the lesion presents as slowly growing large well-demarcated reddish plaque or erythematous scaly patch.

It is most commonly seen in middle-aged men as a solitary lesion on distal extremities.

These are not found in extracutaneous locations.

Generalized presentation with diffuse cutaneous involvement and a more aggressive course is known as (Ketron-Goodman disease).

Microscopic features: (Quiz Case 189)            

- Prominent acanthosis, hyperkeratosis and focal parakeratosis.

- Marked epidermotropism by enlarged atypical lymphocytes.

- Atypical lymphoid cells are present in all levels of the epidermis.

- It is more prominent in the lower third of the epidermis.

- Epidermotropic lymphocytes  display a "pagetoid" appearance.

- In the dermis no atypical cells are noted.

- There is a dense lymphohistiocytic infiltrate in the upper dermis.

Immunohistochemical profile: 

Immunohistochemical staining of T-cell markers can be variable.

T-cells may be CD4+ and CD8- or CD4- and CD8+, and in many cases CD30+.

Clinicopathological correlation is necessary to establish the the diagnosis.

These are always localized, solitary lesions and are often located in the lower extremities.

Differential diagnosis:

- Classical Mycosis fungoides 

- Primary cutaneous CD8 positive epidermotropic cytotoxic T-cell lymphoma.

The tumour has an indolent course.

Treatment consists of surgical excision of the lesion with long follow-up.

Visit: Pathology of Subcutaneous Panniculitis-Like T-Cell Lymphoma

Visit: Pathology of Lymphomatoid Papulosis - Cells resembling 'chunks of coal'

Visit: Pathology of Primary Cutaneous Anaplastic Large Cell Lymphoma

Visit: Pathology of Cutaneous B-Lymphoblastic Lymphoma

Visit: Pathology of Intravascular Large B-cell Lymphoma

Visit: Pathology of Primary Cutaneous Marginal Zone B-Cell Lymphoma

Visit: Pathology of Primary Cutaneous Diffuse Large B-Cell Lymphoma


Further reading:

Pagetoid reticulosis (Woringer-Kolopp disease): an immunophenotypic, molecular, and clinicopathologic study

Woringer-Kolopp disease (localized pagetoid reticulosis) or unilesional mycosis fungoides?: an analysis of eight cases with benign disease

Localized and disseminated pagetoid reticulosis: diagnostic immunophenotypical findings

Woringer-Kolopp disease (pagetoid reticulosis): four cases with histopathologic, ultrastructural, and immunohistologic observations

Pagetoid reticulosis and solitary mycosis fungoide

Pagetoid reticulosis. A further case report with a review of the literature.

Pagetoid reticulosis: A disease of histiocytic origin

Pagetoid reticulosis

Pagetoid reticulosis, epidermotropic mycosis fungoides and mycosis fungoides: a disease spectrum

Woringer-Kolopp disease (pagetoid reticulosis).

The development of a Ki-1-positive large cell non-Hodgkin's lymphoma in pagetoid reticulosis.

Disseminated pagetoid reticulosis associated with mycosis fungoides: immunomorphologic study

Localized epidermotropic reticulosis (Woringer-Kolopp disease): a clinicopathologic study of 15 new cases

Woringer‐Kolopp disease. An epidermotropic variant of mycosis fungoides




Dr Sampurna Roy  MD

Consultant  Histopathologist (Kolkata - India)






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