Ichthyosiform mycosis fungoides

BACKGROUND: Mycosis fungoides (MF) is a skin malignancy of T helper lymphocytes with a wide clinical spectrum. Among the atypical variants of MF, there is an ichthyosis-like presentation. However, to date, only 1 case of ichthyosiform MF has been reported. OBJECTIVE: Our goal was to summarize the clinical characteristics and course, and the pathological, immunohistochemical and molecular genetic findings on 4 patients with ichthyosiform MF. METHODS: A retrospective study was conducted. RESULTS: The 4 patients represented 1.8% of the 221 patients with MF seen by us since 1975. None progressed to systemic disease in up to 12 years (median, 10 years) after the onset of the cutaneous manifestations. Interestingly, skin lesions typical of so-called follicular MF (FMF) were associated in 3 of 4 cases, whereas cutaneous manifestations of classic MF were absent in all 4 patients. CONCLUSION: Ichthyosiform MF represents a rare variant within the clinicopathologic spectrum of MF usually featuring a benign course and a tendency to be associated with lesions of FMF but not with lesions of classic MF.