title: Syringotropic mycosis fungoides: clinical and histologic features, response to treatment, and outcome in 19 patients.
abstract: 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).
  
  
  CONCLUSIONS
  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.
authors.affiliation: Service de Dermatologie, Hôpital Saint-Louis, Assistance Publique-Hôpitaux de Paris (AP-HP), Paris, France; Institut National de la Santé et de la Recherche Médicale (INSERM)-Unité Mixte de Recherche (UMR)-S976, Paris, France; Université Paris VII Sorbonne Paris Cité, Paris, France.
authors.forename: Adèle
authors.initials: A
authors.lastname: de Masson
authors.affiliation: Laboratoire de Pathologie, Hôpital Saint-Louis, Assistance Publique-Hôpitaux de Paris (AP-HP), Paris, France; Université Paris VII Sorbonne Paris Cité, Paris, France; INSERM UMR-S928, Paris, France.
authors.forename: Maxime
authors.initials: M
authors.lastname: Battistella
authors.affiliation: Laboratoire de Pathologie, Hôpital Saint-Louis, Assistance Publique-Hôpitaux de Paris (AP-HP), Paris, France.
authors.forename: Marie-Dominique
authors.initials: MD
authors.lastname: Vignon-Pennamen
authors.affiliation: Laboratoire de Pathologie, Hôpital Saint-Louis, Assistance Publique-Hôpitaux de Paris (AP-HP), Paris, France.
authors.forename: Bénédicte
authors.initials: B
authors.lastname: Cavelier-Balloy
authors.affiliation: Service de Dermatologie, Hôpital Saint-Louis, Assistance Publique-Hôpitaux de Paris (AP-HP), Paris, France.
authors.forename: Frédéric
authors.initials: F
authors.lastname: Mouly
authors.affiliation: Service de Dermatologie, Hôpital Saint-Louis, Assistance Publique-Hôpitaux de Paris (AP-HP), Paris, France.
authors.forename: Michel
authors.initials: M
authors.lastname: Rybojad
authors.affiliation: Service de Dermatologie, Hôpital Saint-Louis, Assistance Publique-Hôpitaux de Paris (AP-HP), Paris, France; Institut National de la Santé et de la Recherche Médicale (INSERM)-Unité Mixte de Recherche (UMR)-S976, Paris, France; Université Paris VII Sorbonne Paris Cité, Paris, France.
authors.forename: Jean-David
authors.initials: JD
authors.lastname: Bouaziz
authors.affiliation: Service de Dermatologie, Hôpital Saint-Louis, Assistance Publique-Hôpitaux de Paris (AP-HP), Paris, France.
authors.forename: Antoine
authors.initials: A
authors.lastname: Petit
authors.affiliation: Service de Dermatologie, Hôpital Saint-Louis, Assistance Publique-Hôpitaux de Paris (AP-HP), Paris, France.
authors.forename: Anne
authors.initials: A
authors.lastname: Saussine
authors.affiliation: Laboratoire de Pathologie, Hôpital Pontchaillou, Rennes, France.
authors.forename: Sophie
authors.initials: S
authors.lastname: Ronceray
authors.affiliation: Laboratoire de Pathologie, Hôpital Pontchaillou, Rennes, France.
authors.forename: François
authors.initials: F
authors.lastname: Le Gall
authors.affiliation: Service de Dermatologie, Hôpital Saint-Louis, Assistance Publique-Hôpitaux de Paris (AP-HP), Paris, France.
authors.forename: Caroline
authors.initials: C
authors.lastname: Ram-Wolff
authors.affiliation: Service de Dermatologie, Hôpital Saint-Louis, Assistance Publique-Hôpitaux de Paris (AP-HP), Paris, France.
authors.forename: Philippe
authors.initials: P
authors.lastname: Assouly
authors.affiliation: Service de Dermatologie, Hôpital Saint-Eloi, Montpellier, France.
authors.forename: Olivier
authors.initials: O
authors.lastname: Dereure
authors.affiliation: Service de Dermatologie, Hôpital Charles-Nicolle, Rouen, France.
authors.forename: Pascal
authors.initials: P
authors.lastname: Joly
authors.affiliation: Service de Dermatologie, Hôpital Robert-Ballanger, Aulnay, France.
authors.forename: Alexiane
authors.initials: A
authors.lastname: Dallot
authors.affiliation: Service de Dermatologie, Hôpital Pontchaillou, Rennes, France.
authors.forename: Alain
authors.initials: A
authors.lastname: Dupuy
authors.affiliation: Service de Dermatologie, Hôpital Saint-Louis, Assistance Publique-Hôpitaux de Paris (AP-HP), Paris, France; Institut National de la Santé et de la Recherche Médicale (INSERM)-Unité Mixte de Recherche (UMR)-S976, Paris, France; Université Paris VII Sorbonne Paris Cité, Paris, France.
authors.forename: Céleste
authors.initials: C
authors.lastname: Lebbé
authors.affiliation: Service de Dermatologie, Hôpital Saint-Louis, Assistance Publique-Hôpitaux de Paris (AP-HP), Paris, France.
authors.forename: Isabelle
authors.initials: I
authors.lastname: Moulonguet
authors.affiliation: Laboratoire de Pathologie, Hôpital Saint-Louis, Assistance Publique-Hôpitaux de Paris (AP-HP), Paris, France.
authors.forename: Jacqueline
authors.initials: J
authors.lastname: Rivet
authors.affiliation: Laboratoire de Pathologie, Hôpital Saint-Louis, Assistance Publique-Hôpitaux de Paris (AP-HP), Paris, France; Université Paris VII Sorbonne Paris Cité, Paris, France; INSERM UMR-S928, Paris, France.
authors.forename: Anne
authors.initials: A
authors.lastname: Janin
authors.affiliation: Service de Dermatologie, Hôpital Saint-Louis, Assistance Publique-Hôpitaux de Paris (AP-HP), Paris, France; Institut National de la Santé et de la Recherche Médicale (INSERM)-Unité Mixte de Recherche (UMR)-S976, Paris, France; Université Paris VII Sorbonne Paris Cité, Paris, France. Electronic address: martine.bagot@sls.aphp.fr.
authors.forename: Martine
authors.initials: M
authors.lastname: Bagot
mesh_terms: D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D000505:Alopecia / Q000209:etiology*; D004439:Eccrine Glands; D005260:Female; D018859:Hair Follicle; D006801:Humans; D008214:Lymphocytes / Q000502:physiology; D008297:Male; D008875:Middle Aged; D009182:Mycosis Fungoides / Q000150:complications / Q000188:drug therapy / Q000473:pathology*; D012189:Retrospective Studies; D012878:Skin Neoplasms / Q000150:complications / Q000188:drug therapy / Q000473:pathology*; D015996:Survival Rate; D016896:Treatment Outcome; D018523:Tropism; D055815:Young Adult
keywords: cutaneous T-cell lymphoma; follicular; folliculotropism; mycosis fungoides; syringotropic; syringotropism