Leonine facies (LF) and mycosis fungoides (MF): A single-center study and systematic review of the literature

J Am Acad Dermatol. 2015 Dec;73(6):976-86. doi: 10.1016/j.jaad.2015.09.017. Epub 2015 Oct 21.

Abstract

Background: Leonine facies (LF) is defined as displaying facial features similar to that of a lion with prominent convexities and furrowed creases. LF develops in a very small population of patients with cutaneous T-cell lymphoma.

Objective: We aimed to study the clinicopathologic features and overall prognosis associated with LF in patients with mycosis fungoides and Sézary syndrome.

Methods: We conducted a single-center retrospective study, reviewing 1338 patients with mycosis fungoides seen from 1987 to 2015 at a tertiary referral center for cutaneous T-cell lymphoma, and a systematic review of 14 patients in the literature.

Results: We identified 10 patients with mycosis fungoides who developed LF. Folliculotropism was seen in all patients with LF who had facial biopsy specimens. Radiation was a beneficial therapy. Complete remission was achieved in 1 patient and overall 5-year survival was 26%. Systematic review of 10 additional patients showed that all patients with LF, including ours, had stage-IV disease and some degree of blood involvement, but not all met criteria for Sézary syndrome.

Limitations: This was a retrospective study with a small sample size.

Conclusion: LF is associated with stage-IV cutaneous T-cell lymphoma, is often accompanied by folliculotropism and blood involvement, and can be treated with local electron beam therapy.

Keywords: Sézary syndrome; folliculotropic mycosis fungoides; leonine facies; mycosis fungoides.

Publication types

  • Review
  • Systematic Review

MeSH terms

  • Adult
  • Aged
  • Biopsy, Needle
  • Cancer Care Facilities
  • Cohort Studies
  • Combined Modality Therapy
  • Facies*
  • Female
  • Follow-Up Studies
  • Humans
  • Immunohistochemistry
  • Lymphoma, T-Cell, Cutaneous / diagnosis
  • Lymphoma, T-Cell, Cutaneous / pathology
  • Lymphoma, T-Cell, Cutaneous / therapy
  • Male
  • Middle Aged
  • Mycosis Fungoides / diagnosis
  • Mycosis Fungoides / pathology*
  • Mycosis Fungoides / therapy
  • Neoplasm Invasiveness / pathology
  • Neoplasm Staging
  • Positron-Emission Tomography / methods
  • Retrospective Studies
  • Risk Assessment
  • Skin Neoplasms / diagnosis
  • Skin Neoplasms / pathology*
  • Skin Neoplasms / therapy
  • Survival Rate
  • Tertiary Care Centers
  • Treatment Outcome