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Article
March 1995

Woringer-Kolopp Disease (Localized Pagetoid Reticulosis) or Unilesional Mycosis Fungoides?An Analysis of Eight Cases With Benign Disease

Author Affiliations

From the Department of Dermatology, University of Michigan, Ann Arbor.

Arch Dermatol. 1995;131(3):325-329. doi:10.1001/archderm.1995.01690150089018
Abstract

Background:  The controversial nosology of Woringer-Kolopp disease (localized pagetoid reticulosis, unilesional mycosis fungoides) is being clarified by the systematic immunophenotypic and immunogenetic examination of infiltrating lesional T lymphocytes. The clinical course and immunohistochemical characteristics of eight cases of Woringer-Kolopp disease are described.

Observations:  Lesions measured 0.8×0.5 to 16.0×15.0 cm. Histologically, all cases resembled mycosis fungoides— type cutaneous T-cell lymphoma and phenotypic analysis supported their designation as an epidermotropic T-cell process. Phenotypic aberrancy was not noted on immunohistochemical analysis of paraffin-embedded tissue. Three of four patients with available fresh-frozen tissue specimens demonstrated reduced or absent expression of CD7 (Leu-9) and/or Leu-8, while loss of the pan— T-cell markers CD2, CD3, and CD5 was not observed. Only in half these patients was a lesional predominance of CD4+ T-cells revealed. Germline DNA was detected in a lesional skin specimen obtained from one patient tested for T-cell receptor gene rearrangements. After treatment, the observation of disease-free periods ranging from 18 months to 17 years (mean, 5.9 years) reinforces the view that Woringer-Kolopp disease is a focal pathologic event with a favorable prognosis. No patient experienced a local recurrence or distant spread of the disease.

Conclusion:  This and previous studies suggest that Woringer-Kolopp disease is a unique, benign unilesional T-cell lymphoproliferative process with certain histologic and phenotypic similarities to both early epidermotropic mycosis fungoides—type cutaneous T-cell lymphoma and other T-cell lymphoproliferations.(Arch Dermatol. 1995;131:325-329)

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