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Papular Mycosis Fungoides is a Distinctive Variant of Early-stage Mycosis Fungoides

Extended Retrospective Study With Long-term Follow-up

Saggini, Andrea MD*,†; Fink-Puches, Regina MD*; Cota, Carlo MD; Lora, Viviana MD; Potzinger, Heide MD*; Massone, Cesare MD§; Cerroni, Lorenzo MD*

The American Journal of Surgical Pathology: April 08, 2019 - Volume Publish Ahead of Print - Issue - p
doi: 10.1097/PAS.0000000000001256
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Papular mycosis fungoides (PMF) is a rare variant of mycosis fungoides (MF). The exact nosology and prognosis of PMF are still unclear. We retrospectively identified cases of PMF from the files of the Department of Dermatology of the Medical University of Graz, Austria, and checked the follow-up data. The patients comprised 13 men and 5 women (median age: 57.5 y; range 13 to 77 y). In 4 patients, an initial clinicopathologic diagnosis of atypical pityriasis lichenoides was made; these cases were subsequently reclassified as PMF due to the onset of conventional patches of MF during follow-up. Follow-up data of our cases showed that 2 patients died of disease progression 50 and 199 months after the first presentation, respectively. Two patients are alive with progressive disease after 215 and 300 months, respectively. Ten patients are alive with stable disease (median: 70 mo). Four patients were in complete remission at last follow-up visit (median: 215 mo; 2 of them died of unrelated causes). Our data confirm that PMF represents a clinicopathologic variant of early MF with prognosis similar to conventional presentations of the disease. Familiarity with PMF and distinction from other cutaneous papular lymphoid proliferations is necessary for a precise diagnosis and management of these patients.

*Department of Dermatology, Research Unit Dermatopathology, Medical University of Graz, Graz, Austria

Department of Biomedicine and Prevention, Division of Anatomic Pathology, University of Rome Tor Vergata

San Gallicano Dermatological Institute, Rome

§Dermatology Unit, Galliera Hospital, Genova, Italy

Conflicts of Interest and Source of Funding: The authors have disclosed that they have no significant relationships with, or financial interest in, any commercial companies pertaining to this article.

Correspondence: Lorenzo Cerroni, MD, Department of Dermatology, Medical University of Graz, Auenbruggerplatz 8, Graz A-8036, Austria (e-mail: lorenzo.cerroni@medunigraz.at).

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