Journal of the American Academy of Dermatology · 2015
Leonine facies (LF) and mycosis fungoides (MF): A single-center study and systematic review of the literature.
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Brown Danielle Nicole, Wieser Iris, Wang Casey, Dabaja Boutnaina S, Duvic Madeleine
Аннотация
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.
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.
Предметные рубрики MeSH
AdultAgedBiopsy, NeedleCancer Care FacilitiesCohort StudiesCombined Modality TherapyFaciesFemaleFollow-Up StudiesHumansImmunohistochemistryLymphoma, T-Cell, CutaneousMaleMiddle AgedMycosis FungoidesNeoplasm InvasivenessNeoplasm StagingPositron-Emission TomographyRetrospective StudiesRisk AssessmentSkin NeoplasmsSurvival RateTertiary Care CentersTreatment Outcome