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Ichthyosiform mycosis fungoides.

Angelo V Marzano1, Alessandro Borghi, Maura Facchetti

  • 1Institute of Dermatological Sciences of the University of Milan and IRCCS Ospedale Maggiore of Milan, Italy. elvio.alessi@unimi.it

Dermatology (Basel, Switzerland)
|April 9, 2002
PubMed
Summary

Ichthyosiform mycosis fungoides (MF) is a rare skin cancer variant. This study found it typically has a benign course and often presents with follicular MF lesions, not classic MF.

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Area of Science:

  • Dermatology
  • Oncology
  • Pathology

Background:

  • Mycosis fungoides (MF) is a T-helper lymphocyte skin cancer with diverse presentations.
  • Ichthyosis-like presentation is an atypical, rarely reported variant of MF.
  • Only one case of ichthyosiform MF had been previously documented.

Purpose of the Study:

  • To describe the clinical, pathological, and molecular features of four patients with ichthyosiform MF.
  • To characterize the clinical course of this rare MF variant.

Main Methods:

  • Retrospective case series analysis.
  • Review of clinical, pathological, immunohistochemical, and molecular genetic data.

Main Results:

  • Four patients with ichthyosiform MF were identified, representing 1.8% of all MF cases.
  • No patients progressed to systemic disease within a median follow-up of 10 years.
  • Associated follicular MF (FMF) lesions were present in 3/4 cases; classic MF lesions were absent in all.

Conclusions:

  • Ichthyosiform MF is a rare variant of mycosis fungoides.
  • This variant typically follows an indolent clinical course.
  • Ichthyosiform MF is associated with FMF lesions but not classic MF lesions.

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