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

• Published in: Journal of the American Academy of Dermatology 2014-11, Vol.71 (5), p.926-934
• Main Authors: de Masson, Adèle, MD, Battistella, Maxime, MD, Vignon-Pennamen, Marie-Dominique, MD, Cavelier-Balloy, Bénédicte, MD, Mouly, Frédéric, MD, Rybojad, Michel, MD, Bouaziz, Jean-David, MD, PhD, Petit, Antoine, MD, Saussine, Anne, MD, Ronceray, Sophie, MD, Le Gall, François, MD, Ram-Wolff, Caroline, MD, Assouly, Philippe, MD, Dereure, Olivier, MD, PhD, Joly, Pascal, MD, PhD, Dallot, Alexiane, MD, Dupuy, Alain, MD, PhD, Lebbé, Céleste, MD, PhD, Moulonguet, Isabelle, MD, Rivet, Jacqueline, MD, PhD, Janin, Anne, MD, PhD, Bagot, Martine, MD, PhD
• Format: Article
• Language: English
• Subjects: Adult; Aged; Aged, 80 and over; Alopecia - etiology; cutaneous T-cell lymphoma; Dermatology; Eccrine Glands; Female; follicular; folliculotropism; Genetics; Hair Follicle; Humans; Life Sciences; Lymphocytes - physiology; Male; Middle Aged; mycosis fungoides; Mycosis Fungoides - complications; Mycosis Fungoides - drug therapy; Mycosis Fungoides - pathology; Retrospective Studies; Skin Neoplasms - complications; Skin Neoplasms - drug therapy; Skin Neoplasms - pathology; Survival Rate; syringotropic; syringotropism; Treatment Outcome; Tropism; Young Adult
• ISSN: 0190-9622; 1097-6787
• DOI: 10.1016/j.jaad.2014.06.033

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.