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.


CONCLUSIONS
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.
Marzano
Angelo V
AV
Institute of Dermatological Sciences of the University of Milan and IRCCS Ospedale Maggiore of Milan, Italy. elvio.alessi@unimi.it
Borghi
Alessandro
A
Facchetti
Maura
M
Alessi
Elvio
E
D000328:Adult; D000368:Aged; D018801:CD2 Antigens / Q000032:analysis; D017252:CD3 Complex / Q000032:analysis; D018450:Disease Progression; D005260:Female; D006801:Humans; D007057:Ichthyosis / Q000175:diagnosis* / Q000276:immunology; D007150:Immunohistochemistry; D017493:Leukocyte Common Antigens / Q000032:analysis; D008297:Male; D008875:Middle Aged; D009182:Mycosis Fungoides / Q000175:diagnosis* / Q000276:immunology; D012189:Retrospective Studies
D018801:CD2 Antigens; D017252:CD3 Complex; D017493:Leukocyte Common Antigens