Telangiectasia macularis eruptiva perstans (TMEP): A form of cutaneous mastocytosis with potential systemic involvement - 14/04/16

Doi : 10.1016/j.jaad.2015.10.050

Maella Severino, MD ^a,^b,ⁿ, Marie-Olivia Chandesris, MD ^h,ⁱ, Stéphane Barete, MD, PhD ^h,^j, Emilie Tournier, MD ^a,^c,^f,^m, Béatrix Sans, MD ^b,^m,ⁿ, Camille Laurent, MD, PhD ^a,^c,^f,^m, Pol André Apoil, MD, PhD ^g,^m, Laurence Lamant, MD, PhD ^a,^c,^f,^m, Claire Mailhol, MD ^d,^m, Michel Laroche, MD, PhD ^e,^m, Sylvie Fraitag, MD ^h,^k, Katia Hanssens, MLT ^l, Patrice Dubreuil, PhD ^l, Olivier Hermine, MD, PhD ^h,ⁱ, Carle Paul, MD, PhD ^a,^b,^m,ⁿ, Cristina Bulai Livideanu, MD ^a,^b,^m,^n,^⁎
^a Paul Sabatier University, Toulouse, France
^b Department of Dermatology, Mastocytosis Competence Center of Midi-Pyrénées, Toulouse University Hospital, Toulouse, France
^c Department of Pathology, Mastocytosis Competence Center of Midi-Pyrénées, Toulouse University Hospital, Toulouse, France
^d Department of Allergy and Pulmonology, Mastocytosis Competence Center of Midi-Pyrénées, Toulouse University Hospital, Toulouse, France
^e Department of Rheumatology, Mastocytosis Competence Center of Midi-Pyrénées, Toulouse University Hospital, Toulouse, France
^f Cancer University Institute of Toulouse Oncopole, Mastocytosis Competence Center of Midi-Pyrénées, Toulouse University Hospital, Toulouse, France
^g Immunology Laboratory, Mastocytosis Competence Center of Midi-Pyrénées, Toulouse University Hospital, Toulouse, France
^h French Reference Center for Mastocytosis, Paris, France
ⁱ Department of Hematology, Necker Hospital, Assistance Publique des Hôpitaux de Paris (AP-HP), Paris, France
^j Unit of Dermatology, AP-HP, Pitié-Salpêtrière Hospital, Sorbonne Universities- Université Pierre et Marie CURIE (UPMC) Paris VI, Paris, France
^k Department of Pathology, Necker-Enfants Malades Hospital, AP-HP, Paris, France
^l 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
^m Mastocytosis Competence Center of Midi-Pyrénées, Toulouse, France
ⁿ Toulouse University Hospital, Toulouse, France

^∗Reprint requests: Cristina Bulai Livideanu, MD, Mastocytosis Competence Center of Midi-Pyrénées, Department of Dermatology, Toulouse University Hospital, 24 Chemin de Pouvourville, 31100, Toulouse, France.Mastocytosis Competence Center of Midi-Pyrénées, Department of Dermatology, Toulouse University Hospital, 24 Chemin de Pouvourville31100, ToulouseFrance

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.

Limitations

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.

Conclusion

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.

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Key words : aggressive systemic mastocytosis, cutaneous mastocytosis, indolent systemic mastocytosis, mast cell activation symptom, mast cell disease, telangiectasia macularis eruptiva perstans

Abbreviations used : IQR, TMEP, WHO

Plan

Methods

Patient population

Diagnostic criteria

Data collection and analysis

Results

Frequency of TMEP

Patient characteristics

Discussion

Conclusion

	Funding sources: None.
	Conflicts of interest: None declared.

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Telangiectasia macularis eruptiva perstans (TMEP): A form of cutaneous mastocytosis with potential systemic involvement - 14/04/16

Abstract

Background

Objective

Methods

Results

Limitations

Conclusion

Plan

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