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Localized and disseminated pagetoid reticulosis. Diagnostic immunophenotypical findings
V Mielke1, H H Wolff, M Winzer
1Department of Dermatology, University of Kiel, Federal Republic of Germany.
Abstract:
Pagetoid reticulosis (PaRet) is a rare skin disease with an intraepidermal infiltrate of atypical lymphocytes. We performed phenotypic studies on two patients with classic localized PaRet (Woringer-Kolopp disease) and one patient with the disseminated type (Ketron-Goodman disease) and compared these with all published reports of cases; both variants show intraepidermal, highly activated, proliferating T cells with variable loss of pan-T-cell antigens, contrasting with nonactivated dermal reactive T cells. This pattern is unique among cutaneous lymphomas and can be used for diagnosis. Despite the fact that localized PaRet does not show a malignant course, recent genotypic analysis of one patient showed monoclonality. Comparable constellations are found in other diseases like lymphomatoid papulosis, which also represent monoclonal disorders of activated T cells with a benign course. To unify these seeming discrepancies, we introduce the concept of "benign cutaneous lymphomas."
Insights
Pagetoid reticulosis (PaRet) involves atypical lymphocytes in the skin. Both localized and disseminated PaRet show unique intraepidermal T-cell patterns, suggesting a concept of "benign cutaneous lymphomas."
Area of Science:
- Dermatology
- Immunology
- Oncology
Background:
- Pagetoid reticulosis (PaRet) is a rare intraepidermal skin disease characterized by atypical lymphocytes.
- It presents in localized (Woringer-Kolopp disease) and disseminated (Ketron-Goodman disease) forms.
Observation:
- Phenotypic studies revealed intraepidermal, highly activated, proliferating T cells with variable pan-T-cell antigen loss in both PaRet variants.
- These intraepidermal T cells contrasted with nonactivated dermal reactive T cells.
- This unique immunophenotype is distinct from other cutaneous lymphomas.
Findings:
- Genotypic analysis indicated monoclonality in a localized PaRet case, similar to lymphomatoid papulosis.
- Both localized PaRet and lymphomatoid papulosis exhibit monoclonal disorders of activated T cells with a benign clinical course.
- The findings support a unified concept of "benign cutaneous lymphomas".
Implications:
- The unique T-cell pattern in PaRet aids in diagnosis.
- The concept of "benign cutaneous lymphomas" helps reconcile the benign clinical course with genotypic findings of monoclonality.
- This classification may refine understanding and management of specific T-cell-driven skin conditions.