Pathology of 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 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.
- 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.
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
Woringer-Kolopp disease (pagetoid reticulosis): four cases with histopathologic, ultrastructural, and immunohistologic observations
Localized epidermotropic reticulosis (Woringer-Kolopp disease): a clinicopathologic study of 15 new cases
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