title: Telangiectasia macularis eruptiva perstans (TMEP): A form of cutaneous mastocytosis with potential systemic involvement.
abstract: BACKGROUND
  Telangiectasia macularis eruptiva perstans (TMEP) has not been fully characterized.
  
  
  OBJECTIVE
  We sought to estimate the frequency and clinical characteristics of TMEP in a cohort of adult patients with cutaneous mastocytosis, and to assess the presence of systemic involvement.
  
  
  METHODS
  We included all consecutive patients evaluated for cutaneous mastocytosis in 2 centers: the Mastocytosis Competence Center of the Midi-Pyrénées from May 2006 to December 2013, and the French Reference Center for Mastocytosis from January 2008 to September 2013. Skin phenotype, histopathology, presence of KIT mutation in the skin, and assessment of systemic involvement according to World Health Organization (WHO) criteria were prospectively investigated.
  
  
  RESULTS
  Of 243 patients with cutaneous mastocytosis, 34 (14%) were given a diagnosis of TMEP. The diagnosis of systemic mastocytosis was established in 16 patients (47%) with TMEP. Three patients (9%) had aggressive systemic mastocytosis (C-findings according to WHO). In all, 32 patients (94%) exhibited at least 1 mast cell activation-related symptom.
  
  
  CONCLUSIONS
  Patient recruitment was undertaken at 2 referral centers with expertise in the diagnosis and treatment of mastocytosis so that the clinical findings and incidence of systemic involvement may be overestimated in comparison with the overall population of patients with TMEP.
  
  
  CONCLUSIONS
  TMEP accounts for about 14% of patients with cutaneous mastocytosis. The disease manifests as mast cell activation symptoms in almost all patients and can be associated with systemic involvement in about 50% of cases.
authors.affiliation: Paul Sabatier University, Toulouse, France; Department of Dermatology, Mastocytosis Competence Center of Midi-Pyrénées, Toulouse University Hospital, Toulouse, France; Toulouse University Hospital, Toulouse, France.
authors.forename: Maella
authors.initials: M
authors.lastname: Severino
authors.affiliation: French Reference Center for Mastocytosis, Paris, France; Department of Hematology, Necker Hospital, Assistance Publique des Hôpitaux de Paris (AP-HP), Paris, France.
authors.forename: Marie-Olivia
authors.initials: MO
authors.lastname: Chandesris
authors.affiliation: French Reference Center for Mastocytosis, Paris, France; Unit of Dermatology, AP-HP, Pitié-Salpêtrière Hospital, Sorbonne Universities- Université Pierre et Marie CURIE (UPMC) Paris VI, Paris, France.
authors.forename: Stéphane
authors.initials: S
authors.lastname: Barete
authors.affiliation: Paul Sabatier University, Toulouse, France; Department of Pathology, Mastocytosis Competence Center of Midi-Pyrénées, Toulouse University Hospital, Toulouse, France; Cancer University Institute of Toulouse Oncopole, Mastocytosis Competence Center of Midi-Pyrénées, Toulouse University Hospital, Toulouse, France; Mastocytosis Competence Center of Midi-Pyrénées, Toulouse, France.
authors.forename: Emilie
authors.initials: E
authors.lastname: Tournier
authors.affiliation: Department of Dermatology, Mastocytosis Competence Center of Midi-Pyrénées, Toulouse University Hospital, Toulouse, France; Mastocytosis Competence Center of Midi-Pyrénées, Toulouse, France; Toulouse University Hospital, Toulouse, France.
authors.forename: Béatrix
authors.initials: B
authors.lastname: Sans
authors.affiliation: Paul Sabatier University, Toulouse, France; Department of Pathology, Mastocytosis Competence Center of Midi-Pyrénées, Toulouse University Hospital, Toulouse, France; Cancer University Institute of Toulouse Oncopole, Mastocytosis Competence Center of Midi-Pyrénées, Toulouse University Hospital, Toulouse, France; Mastocytosis Competence Center of Midi-Pyrénées, Toulouse, France.
authors.forename: Camille
authors.initials: C
authors.lastname: Laurent
authors.affiliation: Immunology Laboratory, Mastocytosis Competence Center of Midi-Pyrénées, Toulouse University Hospital, Toulouse, France; Mastocytosis Competence Center of Midi-Pyrénées, Toulouse, France.
authors.forename: Pol André
authors.initials: PA
authors.lastname: Apoil
authors.affiliation: Paul Sabatier University, Toulouse, France; Department of Pathology, Mastocytosis Competence Center of Midi-Pyrénées, Toulouse University Hospital, Toulouse, France; Cancer University Institute of Toulouse Oncopole, Mastocytosis Competence Center of Midi-Pyrénées, Toulouse University Hospital, Toulouse, France; Mastocytosis Competence Center of Midi-Pyrénées, Toulouse, France.
authors.forename: Laurence
authors.initials: L
authors.lastname: Lamant
authors.affiliation: Department of Allergy and Pulmonology, Mastocytosis Competence Center of Midi-Pyrénées, Toulouse University Hospital, Toulouse, France; Mastocytosis Competence Center of Midi-Pyrénées, Toulouse, France.
authors.forename: Claire
authors.initials: C
authors.lastname: Mailhol
authors.affiliation: Department of Rheumatology, Mastocytosis Competence Center of Midi-Pyrénées, Toulouse University Hospital, Toulouse, France; Mastocytosis Competence Center of Midi-Pyrénées, Toulouse, France.
authors.forename: Michel
authors.initials: M
authors.lastname: Laroche
authors.affiliation: French Reference Center for Mastocytosis, Paris, France; Department of Pathology, Necker-Enfants Malades Hospital, AP-HP, Paris, France.
authors.forename: Sylvie
authors.initials: S
authors.lastname: Fraitag
authors.affiliation: Centre de Recherche en Cancérologie de Marseille (CRCM) (Signaling, Hematopoiesis, and Mechanism of Oncogenesis), Inserm, U1068, Institut Paoli-Calmettes, Aix-Marseille University, UM105, Centre national de la recherche scientifique (CNRS), Unité mixte de recherche (UMR) 7258, Marseille, France.
authors.forename: Katia
authors.initials: K
authors.lastname: Hanssens
authors.affiliation: Centre de Recherche en Cancérologie de Marseille (CRCM) (Signaling, Hematopoiesis, and Mechanism of Oncogenesis), Inserm, U1068, Institut Paoli-Calmettes, Aix-Marseille University, UM105, Centre national de la recherche scientifique (CNRS), Unité mixte de recherche (UMR) 7258, Marseille, France.
authors.forename: Patrice
authors.initials: P
authors.lastname: Dubreuil
authors.affiliation: French Reference Center for Mastocytosis, Paris, France; Department of Hematology, Necker Hospital, Assistance Publique des Hôpitaux de Paris (AP-HP), Paris, France.
authors.forename: Olivier
authors.initials: O
authors.lastname: Hermine
authors.affiliation: Paul Sabatier University, Toulouse, France; Department of Dermatology, Mastocytosis Competence Center of Midi-Pyrénées, Toulouse University Hospital, Toulouse, France; Mastocytosis Competence Center of Midi-Pyrénées, Toulouse, France; Toulouse University Hospital, Toulouse, France.
authors.forename: Carle
authors.initials: C
authors.lastname: Paul
authors.affiliation: Paul Sabatier University, Toulouse, France; Department of Dermatology, Mastocytosis Competence Center of Midi-Pyrénées, Toulouse University Hospital, Toulouse, France; Mastocytosis Competence Center of Midi-Pyrénées, Toulouse, France; Toulouse University Hospital, Toulouse, France. Electronic address: livideanu.c@chu-toulouse.fr.
authors.forename: Cristina
authors.initials: C
authors.lastname: Bulai Livideanu
mesh_terms: D000328:Adult; D000367:Age Factors; D001707:Biopsy, Needle; D015331:Cohort Studies; D018450:Disease Progression; D005260:Female; D005602:France; D006403:Hematologic Tests; D006801:Humans; D007150:Immunohistochemistry; D008297:Male; D034721:Mastocytosis, Systemic / Q000453:epidemiology / Q000473:pathology* / Q000503:physiopathology; D008875:Middle Aged; D011379:Prognosis; D012017:Referral and Consultation; D012189:Retrospective Studies; D018570:Risk Assessment; D012720:Severity of Illness Index; D012737:Sex Factors; D013684:Telangiectasis / Q000473:pathology* / Q000503:physiopathology; D014582:Urticaria Pigmentosa / Q000453:epidemiology / Q000473:pathology* / Q000503:physiopathology
keywords: aggressive systemic mastocytosis; cutaneous mastocytosis; indolent systemic mastocytosis; mast cell activation symptom; mast cell disease; telangiectasia macularis eruptiva perstans