C. Cardamone, R. Parente, G. D. Feo, and M. Triggiani, Mast cells as effector cells of innate immunity and regulators of adaptive immunity, Immunol Lett, vol.178, pp.10-14, 2016.

J. Rivera and A. M. Gilfillan, Molecular regulation of mast cell activation, J Allergy Clin Immunol. juin, vol.117, issue.6, pp.1214-1239, 2006.

F. Bérard, P. Saint-mezard, F. Cousin, S. Mecheri, and J. Nicolas,

, Ann Dermatol Venereol. mai, vol.130, issue.1, pp.1-10, 2003.

E. Da-silva, M. C. Jamur, and C. Oliver, Mast cell function: a new vision of an old cell, J Histochem Cytochem Off J Histochem Soc, vol.62, issue.10, pp.698-738, 2014.

A. Nosbaum, F. Augey, J. Nicolas, and F. Bérard,

, Ann Dermatol Venereol, vol.141, issue.3, pp.559-564, 2014.

P. Valent, Mast cell activation syndromes: definition and classification, Allergy. avr, vol.68, issue.4, pp.417-441, 2013.

P. Valent, C. Akin, M. Arock, K. Brockow, J. H. Butterfield et al., Definitions, criteria and global classification of mast cell disorders with special reference to mast cell activation syndromes: a consensus proposal, Int Arch Allergy Immunol, vol.157, issue.3, pp.215-240, 2012.

G. J. Molderings, J. Homann, S. Brettner, M. Raithel, and T. Frieling, Mast cell activation disease: a concise practical guide for diagnostic workup and therapeutic options, Dtsch Med Wochenschr, vol.139, issue.30, pp.1535-1543, 1946.

C. R. Weiler, K. F. Austen, C. Akin, M. S. Barkoff, J. A. Bernstein et al., AAAAI Mast Cell Disorders Committee Work Group Report: Mast Cell Activation Syndrome (MCAS) Diagnosis and Management, J Allergy Clin Immunol. août, p.0091674919311169, 2019.

L. B. Afrin, S. Self, J. Menk, and J. Lazarchick, Characterization of Mast Cell Activation Syndrome, Am J Med Sci. mars, vol.353, issue.3, pp.207-222, 2017.

A. D. Hogan and L. B. Schwartz, Markers of mast cell degranulation, Methods San Diego Calif. sept, vol.13, issue.1, pp.43-52, 1997.

A. I. Petra, S. Panagiotidou, J. M. Stewart, P. Conti, and T. C. Theoharides, Spectrum of mast cell activation disorders, Expert Rev Clin Immunol. juin, vol.10, issue.6, pp.729-768, 2014.

M. Frieri, Mast Cell Activation Syndrome, Clin Rev Allergy Immunol. juin, vol.54, issue.3, pp.353-65, 2018.

J. Cardet, M. C. Castells, and M. J. Hamilton, Immunology and clinical manifestations of nonclonal mast cell activation syndrome, Curr Allergy Asthma Rep. févr, vol.13, issue.1, pp.10-18, 2013.

B. Haenisch, M. M. Nöthen, and G. J. Molderings, Systemic mast cell activation disease: the role of molecular genetic alterations in pathogenesis, heritability and diagnostics, Immunology, vol.137, issue.3, pp.197-205, 2012.

M. Frieri and M. Quershi, Pediatric Mastocytosis: A Review of the Literature, Pediatr Allergy Immunol Pulmonol. 1 déc, vol.26, issue.4, pp.175-80, 2013.

M. Frieri, R. Patel, and J. Celestin, Mast cell activation syndrome: a review, Curr Allergy Asthma Rep. févr, vol.13, issue.1, pp.27-32, 2013.

J. Vitte, L. Amadei, M. Gouitaa, S. Mezouar, L. Zieleskiewicz et al., Paired acute-baseline serum tryptase levels in perioperative anaphylaxis: An observational study, Allergy. 21 mars, 2019.
URL : https://hal.archives-ouvertes.fr/hal-02159236

R. L. Baretto, S. Beck, J. Heslegrave, C. Melchior, O. Mohamed et al., Validation of international consensus equation for acute serum total tryptase in mast cell activation: A perioperative perspective, Allergy. déc, vol.72, issue.12, pp.2031-2035, 2017.

M. Nassiri, O. Eckermann, M. Babina, G. Edenharter, and M. Worm, Serum levels of 9?,11?-PGF 2 and cysteinyl leukotrienes are useful biomarkers of anaphylaxis, J Allergy Clin Immunol. janv, vol.137, issue.1, pp.312-314, 2016.

D. Schryver, S. Halbrich, M. Clarke, A. , L. Vieille et al., Tryptase levels in children presenting with anaphylaxis: Temporal trends and associated factors, J Allergy Clin Immunol. avr, vol.137, issue.4, pp.1138-1180, 2016.

D. D. Metcalfe, The Treatment of Mastocytosis: An Overview, J Invest Dermatol. mars, vol.96, issue.3, pp.55-64, 1991.

K. Hartmann and D. D. Metcalfe, Pediatric mastocytosis, Hematol Oncol Clin North Am. juin, vol.14, issue.3, pp.625-665, 2000.

P. Valent, C. Akin, P. Bonadonna, K. Hartmann, K. Brockow et al., Proposed Diagnostic Algorithm for Patients with Suspected Mast Cell Activation Syndrome, J Allergy Clin Immunol Pract. avr, vol.7, issue.4, pp.1125-1133, 2019.

M. Lange, A. Zawadzka, S. Schrörs, J. S?omka, H. ?ugowska-umer et al., The role of serum tryptase in the diagnosis and monitoring of pediatric mastocytosis: a single-center experience, Adv Dermatol Allergol, vol.4, pp.306-318, 2017.

M. J. Hamilton, Nonclonal Mast Cell Activation Syndrome: A Growing Body of Evidence, Immunol Allergy Clin North Am, vol.38, issue.3, pp.469-81, 2018.

S. Barete, Les mastocytoses. Ann Dermatol Vénéréologie, vol.141, pp.698-714, 2014.

N. Klaiber, S. Kumar, and A. Irani, Mastocytosis in Children. Curr Allergy Asthma Rep, vol.17, issue.11, p.80, 2017.

C. Caffarelli, F. Paravati, E. Hachem, M. Duse, M. Bergamini et al., Management of chronic urticaria in children: a clinical guideline, Ital J Pediatr. déc, vol.45, issue.1, p.101, 2019.

R. Confino-cohen, G. Chodick, V. Shalev, M. Leshno, O. Kimhi et al., Chronic urticaria and autoimmunity: Associations found in a large population study, J Allergy Clin Immunol. mai, vol.129, issue.5, pp.1307-1320, 2012.

U. Müller, A. Helbling, T. Hunziker, B. Wüthrich, A. Pécoud et al., Mastocytosis and atopy: a study of 33 patients with urticaria pigmentosa, Allergy. nov, vol.45, issue.8, pp.597-603, 1990.

A. Kiszewski, C. Duran-mckinster, L. Orozco-covarrubias, P. Gutierrez-castrellon, and R. Ruiz-maldonado, Cutaneous mastocytosis in children: a clinical analysis of 71 cases, J Eur Acad Dermatol Venereol. mai, vol.18, issue.3, pp.285-90, 2004.

C. Méni, J. Bruneau, S. Georgin-lavialle, L. Saché-de-peufeilhoux, L. Damaj et al., Paediatric mastocytosis: a systematic review of 1747 cases, Br J Dermatol. mars, vol.172, issue.3, pp.642-51, 2015.

M. Maurer, K. Weller, C. Bindslev-jensen, A. Giménez-arnau, P. J. Bousquet et al., Unmet clinical needs in chronic spontaneous urticaria. A GA2LEN task force report1: Unmet clinical needs in chronic urticaria, Allergy. mars, vol.66, issue.3, pp.317-347, 2011.

G. Molderings, U. Kolck, C. Scheurlen, M. Brüss, T. Frieling et al., Die systemische Mastzellerkrankung mit gastrointestinal betonter Symptomatik -eine Checkliste als Diagnoseinstrument, Dtsch Med Wochenschr. sept, vol.131, issue.38, pp.2095-100, 2006.

G. J. Molderings, B. Haenisch, M. Bogdanow, R. Fimmers, and M. M. Nöthen, Familial Occurrence of Systemic Mast Cell Activation Disease. Wallace GR, éditeur. PLoS ONE, vol.8, p.76241, 2013.

S. Broesby-olsen, M. Carter, H. F. Kjaer, C. G. Mortz, M. B. Møller et al., Pediatric Expression of Mast Cell Activation Disorders, Immunol Allergy Clin North Am, vol.38, issue.3, pp.365-77, 2018.

A. Tefferi, R. L. Levine, K. Lim, A. -. Wahab, O. Lasho et al., Frequent TET2 mutations in systemic mastocytosis: clinical, KITD816V and FIP1L1-PDGFRA correlates, Leukemia. mai, vol.23, issue.5, pp.900-904, 2009.

M. Lange, M. Niedoszytko, J. Renke, J. Gle?, and B. Nedoszytko, Clinical aspects of paediatric mastocytosis: a review of 101 cases: Clinical aspects of paediatric mastocytosis, J Eur Acad Dermatol Venereol. janv, vol.27, issue.1, pp.97-102, 2013.

M. J. Hamilton, J. L. Hornick, C. Akin, M. C. Castells, and N. J. Greenberger, Mast cell activation syndrome: a newly recognized disorder with systemic clinical manifestations, J Allergy Clin Immunol. juill, vol.128, issue.1, pp.147-152, 2011.

K. Brockow, C. Jofer, H. Behrendt, and J. Ring, Anaphylaxis in patients with mastocytosis: a study on history, clinical features and risk factors in 120 patients: Anaphylaxis in patients with mastocytosis, Allergy. 4 janv, vol.63, issue.2, pp.226-258, 2008.

A. Matito and M. Carter, Cutaneous and Systemic Mastocytosis in Children: A Risk Factor for Anaphylaxis?, Curr Allergy Asthma Rep. mai, vol.15, issue.5, p.22, 2015.

I. Alvarez-twose, S. Vañó-galván, L. Sánchez-muñoz, J. M. Morgado, A. Matito et al., Increased serum baseline tryptase levels and extensive skin involvement are predictors for the severity of mast cell activation episodes in children with mastocytosis, Allergy. juin, vol.67, issue.6, pp.813-834, 2012.

M. Barnes, L. Van, L. Delong, and L. P. Lawley, Severity of Cutaneous Findings Predict the Presence of Systemic Symptoms in Pediatric Maculopapular Cutaneous Mastocytosis, Pediatr Dermatol. mai, vol.31, issue.3, pp.271-276, 2014.

D. González-de-olano, B. De-la-hoz-caballer, N. López, R. , S. Muñoz et al., Prevalence of allergy and anaphylactic symptoms in 210 adult and pediatric patients with mastocytosis in Spain: a study of the Spanish network on mastocytosis (REMA), Clin Exp Allergy. 28 août, vol.0, issue.0, p.070831211107005, 2007.

N. Russell, S. Jennings, B. Jennings, V. Slee, L. Sterling et al., The Mastocytosis Society Survey on Mast Cell Disorders: Part 2-Patient Clinical Experiences and Beyond, J Allergy Clin Immunol Pract. avr, vol.7, issue.4, pp.1157-1165, 2019.

G. Barbara, V. Stanghellini, D. Giorgio, R. Cremon, C. Cottrell et al., Activated mast cells in proximity to colonic nerves correlate with abdominal pain in irritable bowel syndrome, Gastroenterology. mars, vol.126, issue.3, pp.693-702, 2004.

D. Levy, Migraine pain, meningeal inflammation, and mast cells, Curr Pain Headache Rep. juin, vol.13, issue.3, pp.237-277, 2009.

L. B. Afrin, D. Pöhlau, M. Raithel, B. Haenisch, F. L. Dumoulin et al., Mast cell activation disease: An underappreciated cause of neurologic and psychiatric symptoms and diseases, Brain Behav Immun, vol.50, pp.314-335, 2015.

D. Kempuraj, S. Mentor, R. Thangavel, M. E. Ahmed, G. P. Selvakumar et al., Mast Cells in Stress, Pain, Blood-Brain Barrier, Neuroinflammation and Alzheimer's Disease. Front Cell Neurosci, vol.13, p.54, 2019.

S. Wirz and G. J. Molderings, A Practical Guide for Treatment of Pain in Patients with Systemic Mast Cell Activation Disease, Pain Physician, vol.20, issue.6, pp.849-61, 2017.

M. P. Rogers, K. Bloomingdale, B. J. Murawski, N. A. Soter, P. Reich et al., Mixed organic brain syndrome as a manifestation of systemic mastocytosis, Psychosom Med. août, vol.48, issue.6, pp.437-484, 1986.

O. Hermine, O. Lortholary, P. S. Leventhal, A. Catteau, F. Soppelsa et al., Case-Control Cohort Study of Patients' Perceptions of Disability in Mastocytosis. Soyer HP, éditeur, PLoS ONE. 28 mai, vol.3, issue.5, p.2266, 2008.
URL : https://hal.archives-ouvertes.fr/hal-00284181

S. Georgin-lavialle, R. Gaillard, D. Moura, and O. Hermine, Mastocytosis in adulthood and neuropsychiatric disorders, Transl Res. août, vol.174, pp.77-85, 2016.

T. C. Theoharides, Autism Spectrum Disorders and Mastocytosis, Int J Immunopathol Pharmacol, vol.22, issue.4, pp.859-65, 2009.

E. Dere, A. Zlomuzica, D. Souza-silva, M. A. Ruocco, L. A. Sadile et al., Neuronal histamine and the interplay of memory, reinforcement and emotions, Behav Brain Res. déc, vol.215, issue.2, pp.209-229, 2010.

A. I. Petra, S. Panagiotidou, J. M. Stewart, P. Conti, and T. C. Theoharides, Spectrum of mast cell activation disorders, Expert Rev Clin Immunol. juin, vol.10, issue.6, pp.729-768, 2014.

Z. Ibrahim, P. Bongrand, P. Carayon, and J. Vitte, Détermination du taux sérique de tryptase dans une population pédiatrique, Rev Fr Allergol, vol.49, issue.7, pp.524-531, 2009.

M. C. Carter, S. T. Clayton, H. D. Komarow, E. H. Brittain, L. M. Scott et al., Assessment of clinical findings, tryptase levels, and bone marrow histopathology in the management of pediatric mastocytosis, J Allergy Clin Immunol. déc, vol.136, issue.6, pp.1673-1679, 2015.

R. Heide, M. Hup, P. Mulder, and A. P. Oranje, Clinical Scoring of Cutaneous Mastocytosis, Acta Derm Venereol. 1 sept, vol.81, issue.4, pp.273-279, 2001.

D. Grossin and S. Daens, Le Syndrome d'Ehlers-Danlos et le Syndrome d'Activation Mastocytaire : le SAMED ?

S. Daens, D. Grossin, T. Hermanns-lê, D. Peeters, and D. Manicourt,

, Rev Med Liege. févr, vol.73, issue.2, pp.61-65, 2018.

B. Tinkle, M. Castori, B. Berglund, H. Cohen, R. Grahame et al., Hypermobile Ehlers-Danlos syndrome (a.k.a. Ehlers-Danlos syndrome Type III and Ehlers-Danlos syndrome hypermobility type): Clinical description and natural history, Am J Med Genet C Semin Med Genet. mars, vol.175, issue.1, pp.48-69, 2017.

C. Hamonet, Syndrome d'Ehlers-Danlos (SED) type III (hypermobile): validation d'une échelle clinique somatosensorielle (ECSS-62), à propos de 626 cas, 28 févr, pp.405-420, 2017.

F. Malfait, C. Francomano, P. Byers, J. Belmont, B. Berglund et al., The 2017 international classification of the Ehlers-Danlos syndromes, Am J Med Genet C Semin Med Genet. mars, vol.175, issue.1, pp.8-26, 2017.
URL : https://hal.archives-ouvertes.fr/hal-01709259

P. Beighton, A. De-paepe, B. Steinmann, P. Tsipouras, and R. J. Wenstrup, Ehlers-Danlos syndromes: revised nosology, Villefranche, 1997. Ehlers-Danlos National Foundation (USA) and Ehlers-Danlos Support Group (UK), Am J Med Genet. 28 avr, vol.77, issue.1, pp.31-38, 1998.

R. Freeman, W. Wieling, F. B. Axelrod, D. G. Benditt, E. Benarroch et al., Consensus statement on the definition of orthostatic hypotension, neurally mediated syncope and the postural tachycardia syndrome, Clin Auton Res. avr, vol.21, issue.2, pp.69-72, 2011.

R. Bonamichi-santos, K. Yoshimi-kanamori, P. Giavina-bianchi, and M. V. Aun, Association of Postural Tachycardia Syndrome and Ehlers-Danlos Syndrome with Mast Cell Activation Disorders, Immunol Allergy Clin North Am. août, vol.38, issue.3, pp.497-504, 2018.

D. Wallman, J. Weinberg, and A. D. Hohler, Ehlers-Danlos Syndrome and Postural Tachycardia Syndrome: A relationship study, J Neurol Sci. mai, vol.340, issue.1-2, pp.99-102, 2014.

S. L. Seneviratne, A. Maitland, and L. Afrin, Mast cell disorders in Ehlers-Danlos syndrome, Am J Med Genet C Semin Med Genet, vol.175, issue.1, pp.226-262, 2017.

I. Cheung and P. Vadas, A New Disease Cluster: Mast Cell Activation Syndrome, Postural Orthostatic Tachycardia Syndrome, and Ehlers-Danlos Syndrome, J Allergy Clin Immunol. févr, vol.135, issue.2, p.65, 2015.

J. J. Lyons, Hereditary Alpha Tryptasemia, Immunol Allergy Clin North Am. août, vol.38, issue.3, pp.483-95, 2018.

T. A. Doherty and A. A. White, Postural orthostatic tachycardia syndrome and the potential role of mast cell activation, Auton Neurosci. déc, vol.215, pp.83-91, 2018.

G. J. Molderings, B. Haenisch, S. Brettner, J. Homann, M. Menzen et al., Pharmacological treatment options for mast cell activation disease, Naunyn Schmiedebergs Arch Pharmacol. juill, vol.389, issue.7, pp.671-94, 2016.

F. Siebenhaar, A. Förtsch, K. Krause, K. Weller, M. Metz et al., Rupatadine improves quality of life in mastocytosis: a randomized, double-blind, placebo-controlled trial, Allergy. juill, vol.68, issue.7, pp.949-52, 2013.

N. De-silva, H. Damayanthi, A. Rajapakse, C. Rodrigo, and S. Rajapakse, Leukotriene receptor antagonists for chronic urticaria: a systematic review, Allergy Asthma Clin Immunol, vol.10, issue.1, p.24, 2014.

R. Asero, A. Tedeschi, and M. Cugno, Heparin and Tranexamic Acid Therapy May Be Effective in Treatment-Resistant Chronic Urticaria with Elevated D-Dimer: A Pilot Study, Int Arch Allergy Immunol, vol.152, issue.4, pp.384-393, 2010.

M. Maurer, K. Rosén, H. Hsieh, S. Saini, C. Grattan et al., Omalizumab for the Treatment of Chronic Idiopathic or Spontaneous Urticaria, N Engl J Med. 7 mars, vol.368, issue.10, pp.924-959, 2013.

G. J. Molderings, M. Raithel, F. Kratz, M. Azemar, B. Haenisch et al., Omalizumab Treatment of Systemic Mast Cell Activation Disease: Experiences from Four Cases, Intern Med, vol.50, issue.6, pp.611-616, 2011.

T. Hinojosa, D. J. Lewis, R. Vangipuram, L. Safeer, U. N. Mui et al., The efficacy of omalizumab in Cutaneous Mastocytosis: A case series, Dermatol Ther. mai, vol.32, issue.3, p.12848, 2019.

M. Picard, P. Giavina-bianchi, V. Mezzano, and M. Castells, Expanding Spectrum of Mast Cell Activation Disorders: Monoclonal and Idiopathic Mast Cell Activation Syndromes, Clin Ther. mai, vol.35, issue.5, pp.548-62, 2013.

D. Cuvillo, A. Sastre, J. Montoro, J. Jáuregui, I. Ferrer et al., Use of antihistamines in pediatrics, J Investig Allergol Clin Immunol, vol.17, issue.2, pp.28-40, 2007.

F. Simons, Behalf of the Early Prevention of Asthma in Atopic Children (EPAAC) Study Group*. Safety of levocetirizine treatment in young atopic children: An 18-month study, Pediatr Allergy Immunol. sept, vol.18, issue.6, pp.535-577, 2007.

T. W. De-vries and F. Van-hunsel, Adverse drug reactions of systemic antihistamines in children in the Netherlands: Table 1. Arch Dis Child, vol.101, pp.968-70, 2016.

M. Staevska, T. A. Popov, T. Kralimarkova, C. Lazarova, S. Kraeva et al., The effectiveness of levocetirizine and desloratadine in up to 4 times conventional doses in difficult-to-treat urticaria, J Allergy Clin Immunol. mars, vol.125, issue.3, pp.676-82, 2010.

M. G. Haarman, F. Van-hunsel, and T. W. De-vries, Adverse drug reactions of montelukast in children and adults, Pharmacol Res Perspect, vol.5, issue.5, p.341, 2017.

R. Lemal, G. Fouquet, L. Terriou, M. Vaes, C. B. Livideanu et al., Omalizumab Therapy for Mast Cell-Mediator Symptoms in Patients with ISM, CM, MMAS, and MCAS, J Allergy Clin Immunol Pract. avr, p.2213219819303228, 2019.
URL : https://hal.archives-ouvertes.fr/hal-02299382

. Lange, ANNEXES Annexe 1. Critères diagnostiques de Mastocytose systémique d' après Barete, p.89

, Critère majeur

, Infiltrats denses et multifocaux de mastocytes au niveau de la moelle osseuse ou d'autres organes extracutanés

, Critères mineurs

, Présence de mastocytes atypiques de forme allongés dans la moelle (>25%)

, Coexpression du CD2 et/ou du CD25 par les mastocytes médullaires, sanguins ou d'autres organes extracutanés

, Mutation du C-KIT sur le codon 816 dans la moelle osseuse, le sang ou d'autres organes extracutanés

, Tryptase sérique > 20µg/L

, Le diagnostic est établi en présence d'un critère majeur et un critère mineur ou de trois critères mineurs

, Symptômes associés à la mastocytose selon le score AFIRMM

, Chaque handicap

, Le score AFIRMM a ensuite été calculé comme suit: où n est le numéro du symptôme, Grade est la gravité auto-évaluée du symptôme (0-4), et Weight est la pondération

, Le score AFIRMM peut aller d'un minimum de 0 (aucun handicap) jusqu'à un maximum de 760

, Annexe 3. Score SCORMA d'après Heide et al.(60)