Pathology of Pagetoid Reticulosis (Woringer-Kolopp disease)

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

It first described by Frederick Woringer and Pierre Kolopp in 1939, in Strasbourg, France.

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:

- 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.