Localized and disseminated pagetoid reticulosis. Diagnostic immunophenotypical findings.
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."
Mielke
V
V
Department of Dermatology, University of Kiel, Federal Republic of Germany.
Wolff
H H
HH
Winzer
M
M
Sterry
W
W
D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D000911:Antibodies, Monoclonal; D004817:Epidermis / Q000473:pathology; D005260:Female; D006801:Humans; D008232:Lymphoproliferative Disorders / Q000473:pathology*; D008297:Male; D010641:Phenotype; D012871:Skin Diseases / Q000473:pathology*; D013601:T-Lymphocytes / Q000145:classification / Q000473:pathology
D000911:Antibodies, Monoclonal