Syringotropic mycosis fungoides: Clinical and histologic features, response to treatment, and outcome in 19 patients - Université Pierre et Marie Curie Accéder directement au contenu
Article Dans Une Revue Journal of The American Academy of Dermatology Année : 2014

Syringotropic mycosis fungoides: Clinical and histologic features, response to treatment, and outcome in 19 patients

Michel Rybojad
Antoine Petit
Olivier Dereure
Pascal Joly
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Résumé

BACKGROUND: A rare variant of mycosis fungoides (MF), syringotropic MF (STMF) is characterized by a particular tropism of the lymphocytic infiltrate for the eccrine structures, and included in the follicular subtype of MF in the World Health Organization-European Organization for Research and Treatment of Cancer classification of cutaneous lymphomas. OBJECTIVE: We sought to determine the clinicopathologic features and disease course of patients with STMF. METHODS: A retrospective study was conducted to identify patients with STMF from 1998 to 2013. RESULTS: Nineteen patients were included: 15 men and 4 women, mean age 55 years (range, 24-86). Most had multiple lesions (n = 16, 84%) with associated alopecia (n = 12, 63%) and/or punctuated aspect (n = 12, 63%). Palms or soles were involved in 10 cases (53%). Folliculotropism was found in 13 cases (68%). After a median follow-up of 70 months (range, 2-140), 3 patients died, 1 from disease-related death. The 5-year overall and disease-specific survival were 100%. The disease-specific survival was significantly higher than in 54 patients with folliculotropic MF without syringotropism (5-year disease-specific survival, 74%; 95% confidence interval, 58%-94%, P = .02). LIMITATIONS: Retrospective setting is a limitation. CONCLUSIONS: In the spectrum of adnexotropic MF, STMF appears as a distinct entity from follicular MF, with peculiar clinical characteristics and natural history.

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Génétique
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Dates et versions

hal-01064589 , version 1 (16-09-2014)

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Adèle De Masson, Maxime Battistella, Marie-Dominique Vignon-Pennamen, Bénédicte Cavelier-Balloy, Frédéric Mouly, et al.. Syringotropic mycosis fungoides: Clinical and histologic features, response to treatment, and outcome in 19 patients. Journal of The American Academy of Dermatology, 2014, 71 (5), pp.926-934. ⟨10.1016/j.jaad.2014.06.033⟩. ⟨hal-01064589⟩
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