title: Leonine facies (LF) and mycosis fungoides (MF): A single-center study and systematic review of the literature.
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.
  
  
  CONCLUSIONS
  This was a retrospective study with a small sample size.
  
  
  CONCLUSIONS
  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.
authors.affiliation: Baylor College of Medicine, Houston, Texas. Electronic address: danielle.n.brown522@gmail.com.
authors.forename: Danielle Nicole
authors.initials: DN
authors.lastname: Brown
authors.affiliation: Department of Dermatology, University of Texas MD Anderson Cancer Center, Houston, Texas; Department of Dermatology, Paracelsus Medical University Salzburg, Salzburg, Austria.
authors.forename: Iris
authors.initials: I
authors.lastname: Wieser
authors.affiliation: Department of Dermatology, University of Texas MD Anderson Cancer Center, Houston, Texas.
authors.forename: Casey
authors.initials: C
authors.lastname: Wang
authors.affiliation: Department of Radiation Oncology, University of Texas MD Anderson Cancer Center, Houston, Texas.
authors.forename: Boutnaina S
authors.initials: BS
authors.lastname: Dabaja
authors.affiliation: Department of Dermatology, University of Texas MD Anderson Cancer Center, Houston, Texas.
authors.forename: Madeleine
authors.initials: M
authors.lastname: Duvic
mesh_terms: D000328:Adult; D000368:Aged; D001707:Biopsy, Needle; D002173:Cancer Care Facilities; D015331:Cohort Studies; D003131:Combined Modality Therapy; D019066:Facies; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D007150:Immunohistochemistry; D016410:Lymphoma, T-Cell, Cutaneous / Q000175:diagnosis / Q000473:pathology / Q000628:therapy; D008297:Male; D008875:Middle Aged; D009182:Mycosis Fungoides / Q000175:diagnosis / Q000473:pathology* / Q000628:therapy; D009361:Neoplasm Invasiveness / Q000473:pathology; D009367:Neoplasm Staging; D049268:Positron-Emission Tomography / Q000379:methods; D012189:Retrospective Studies; D018570:Risk Assessment; D012878:Skin Neoplasms / Q000175:diagnosis / Q000473:pathology* / Q000628:therapy; D015996:Survival Rate; D062606:Tertiary Care Centers; D016896:Treatment Outcome
keywords: Sézary syndrome; folliculotropic mycosis fungoides; leonine facies; mycosis fungoides