Leonine Facies (LF) and Mycosis Fungoides (MF): A Single-Center Study and Systematic Review of the Literature  

Oleh:

Brown, Danielle Nicole ; Wieser, Iris ; Wang, Casey ; Dabaja, Boutnaina S. ; Duvic, Madeleine

Jenis: Article from Journal - ilmiah internasional
Dalam koleksi: JAAD: Journal of the American Academy of Dermatology (keterangan: ada di ClinicalKey) vol. 73 no. 06 (Dec. 2015), page 976-986.
Topik: Folliculotropic Mycosis Fungoides; Leonine Facies; Mycosis Fungoides; Sezary Syndrome

Ketersediaan

Perpustakaan FK Nomor Panggil: J15.K Non-tandon: 1 (dapat dipinjam: 0) Tandon: tidak ada

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Isi artikel

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.

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