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Article
March 1989

Localized and Disseminated Pagetoid ReticulosisDiagnostic Immunophenotypical Findings

Author Affiliations

From the Departments of Dermatology and Venereology, University of Kiel (Drs Mielke and Sterry) and Medical University of Lübeck (Drs Wolff and Winzer), Federal Republic of Germany.

Arch Dermatol. 1989;125(3):402-406. doi:10.1001/archderm.1989.01670150092016
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 panT-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."

(Arch Dermatol 1989;125:402-406)

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