Leonine facies (LF) and mycosis fungoides (MF): A single-center study and systematic review of the literature - 12/11/15
, Iris Wieser, MD b, d, Casey Wang, MD b, Boutnaina S. Dabaja, MD c, Madeleine Duvic, MD bAbstract |
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.
Le texte complet de cet article est disponible en PDF.Key words : folliculotropic mycosis fungoides, leonine facies, mycosis fungoides, Sézary syndrome
Abbreviations used : BSA, CT, CTCL, FMF, LCT, LDH, LF, MF, PET, SS, TSEB
Plan
| Funding sources: None. |
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| Disclosure: Dr Duvic received clinical trial support for patients in trial and consulted on protocol design (Eisai-E7777 and bexarotene). Ms Brown and Drs Wieser, Wang, and Dabaja have no conflicts of interest to declare. |
Vol 73 - N° 6
P. 976-986 - décembre 2015 Retour au numéro
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