Published online Nov 2, 2016. doi: 10.5314/wjd.v5.i4.136
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.
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.