Localized and disseminated pagetoid reticulosis. Diagnostic immunophenotypical findings

Arch Dermatol. 1989 Mar;125(3):402-6.

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."

Publication types

  • Case Reports
  • Research Support, Non-U.S. Gov't

MeSH terms

  • Adult
  • Aged
  • Aged, 80 and over
  • Antibodies, Monoclonal
  • Epidermis / pathology
  • Female
  • Humans
  • Lymphoproliferative Disorders / pathology*
  • Male
  • Phenotype
  • Skin Diseases / pathology*
  • T-Lymphocytes / classification
  • T-Lymphocytes / pathology

Substances

  • Antibodies, Monoclonal