Case Report
Copyright ©The Author(s) 2016. Published by Baishideng Publishing Group Inc. All rights reserved.
World J Dermatol. Nov 2, 2016; 5(4): 136-143
Published online Nov 2, 2016. doi: 10.5314/wjd.v5.i4.136
Papular mycosis fungoides: Six new cases and association with chronic lymphocytic leukemia
Eric C Vonderheid, Marshall E Kadin, Gladys H Telang
Eric C Vonderheid, Sidney Kimmel Cancer Center, Johns Hopkins Medical Institutes, Tucson, AZ 85729, United States
Marshall E Kadin, Department of Dermatology, Boston University and Roger Williams Medical Center, Providence, RI 02908, United States
Gladys H Telang, Department of Dermatology, the Warren Alpert Medical School of Brown University, Providence, RI 02903, United States
Author contributions: Vonderheid EC and Kadin ME designed the report; Telang GH performed the histologic and immunopathologic studies; Vonderheid EC collected the patients’ clinical data; Vonderheid EC, Kadin ME and Telang GH analyzed the data and wrote the paper.
Institutional review board statement: The study was approved by the Johns Hopkins Institutional Review Board.
Informed consent statement: Information about patients was obtained from an approved Cutaneous Lymphoma Registry and informed consent for clinical photography was obtained from all patients.
Conflict-of-interest statement: The authors have no conflicts of interest to declare.
Open-Access: This article is an open-access article which was selected by an in-house editor and fully peer-reviewed by external reviewers. It is distributed in accordance with the Creative Commons Attribution Non Commercial (CC BY-NC 4.0) license, which permits others to distribute, remix, adapt, build upon this work non-commercially, and license their derivative works on different terms, provided the original work is properly cited and the use is non-commercial. See:
Correspondence to: Eric C Vonderheid, MD, Adjunct Professor of Oncology, Sidney Kimmel Cancer Center, Johns Hopkins Medical Institutes, 37580 S. Desert Sun Drive, Tucson, AZ 85729, United States.
Telephone: +1-520-8252699
Received: June 7, 2016
Peer-review started: June 13, 2016
First decision: July 11, 2016
Revised: August 25, 2016
Accepted: October 1, 2016
Article in press: October 9, 2016
Published online: November 2, 2016

Papular mycosis fungoides (MF) is a rare presentation of MF. Six illustrative cases of papular MF were retrospectively reviewed. Five of the cases studied by immunohistochemistry had variable numbers (range: 1%-20%) of CD30+ cells in the dermal infiltrate, a finding that is characteristic of lymphomatoid papulosis but may occasionally occur in typical early MF. Although none of our papular MF patients had progressive disease, lesions with relatively high numbers of CD30+ cells in 3 patients did not respond well to skin-directed treatments used for MF. Interestingly, these patients had evidence of co-existing clonal B cell populations in the blood (one with clonal B cell lymphocytosis and two with B-cell chronic lymphocytic leukemia). We conclude that: (1) papular MF may contain CD30+ cells, thereby causing confusion with lymphomatoid papulosis; and (2) papular MF, like more typical MF, may be associated with clonal B-cell proliferations including chronic lymphocytic leukemia.

Keywords: Mycosis fungoides, Lymphocytosis, Chronic lymphocytic leukemia, Papule, Cutaneous lymphoma

Core tip: Mycosis fungoides presenting with papules as the only clinical manifestation is a rare variant of the disease. To date only 16 cases of papular mycosis fungoides have been described in the literature and none had CD30+ cells. We report 6 additional cases, 5 with 1%-20% CD30+ cells. Three cases had co-existing clonal B cell lymphoproliferation (2 with chronic lymphocytic leukemia). The possible pathogenic relationship between mycosis fungoides and chronic lymphocytic leukemia is discussed.