Pediatric Mastocytosis

  • Nisha Suyien Chandran


Mastocytosis refers to a heterogeneous group of disorders characterized by the abnormal infiltration of mast cells into tissues including skin, bone marrow, liver, spleen, and lymph nodes. Mastocytosis in both adults and children is a clonal mast cell disease due to activating mutations in the KIT gene. Pediatric mastocytosis is typically limited to the skin and has a benign prognosis. Nevertheless, cutaneous mastocytosis in children can be associated with systemic symptoms due to mast cell degranulation and release of mast cell mediators, even when there is no systemic infiltration. Treatment of pediatric mastocytosis is aimed at preventing and controlling skin and systemic mast cell activation symptoms. Therapy is divided into topical and systemic forms, as well as antimediator and cytoreductive therapies; these should be tailored according to the grade of symptom severity in the individual patient.


Mast cells c-KIT Childhood Mastocytoma Urticaria pigmentosa Diffuse cutaneous mastocytosis Tryptase 


  1. 1.
    Kettelhut BV, Metcalfe DD. Pediatric mastocytosis. J Invest Dermatol. 1991;96:15S–8S.CrossRefGoogle Scholar
  2. 2.
    Ben-Amitai D, Metzker A, Cohen HA. Pediatric cutaneous mastocytosis: a review of 180 patients. Isr Med Assoc J. 2005;7:320–2.PubMedGoogle Scholar
  3. 3.
    Hannaford R, Rogers M. Presentation of cutaneous mastocytosis in 173 children. Australas J Dermatol. 2001;42:15–21.CrossRefPubMedGoogle Scholar
  4. 4.
    Valent P, Akin C, Escribano L, Födinger M, Hartmann K, Brockow K, Castells M, Sperr WR, Kluin-Nelemans HC, Hamdy NA, Lortholary O, Robyn J, van Doormaal J, Sotlar K, Hauswirth AW, Arock M, Hermine O, Hellmann A, Triggiani M, Niedoszytko M, Schwartz LB, Orfao A, Horny HP, Metcalfe DD. Standards and standardization in mastocytosis: consensus statements on diagnostics, treatment recommendations and response criteria. Eur J Clin Invest. 2007;37:435–53.CrossRefPubMedGoogle Scholar
  5. 5.
    Middelkamp Hup MA, Heide R, Tank B, Mulder PGH, Oranje AP. Comparison of mastocytosis with onset in children and adults. J Eur Acad Dermatol Venereol. 2002;16:115–20.CrossRefPubMedGoogle Scholar
  6. 6.
    Valent P, Horny HP. Diagnostic criteria and classification of mastocytosis: consensus proposal. Leuk Res. 2001;25:603–25.CrossRefPubMedGoogle Scholar
  7. 7.
    Torrelo A, Alvarez-Twose I, Escribano L. Childhood mastocytosis. Curr Opin Pediatr. 2012;24:480–6.PubMedGoogle Scholar
  8. 8.
    Teodosio C, García-Montero AC, Jara-Acevedo M, et al. Mast cells from different molecular and prognostic subtypes of systemic mastocytosis display distinct immunophenotypes. J Allergy Clin Immunol. 2010;125:719–26.CrossRefPubMedGoogle Scholar
  9. 9.
    Kneilling M, Röcken M. Mast cells: novel clinical perspectives from recent insights. Exp Dermatol. 2009;18:488–96.CrossRefPubMedGoogle Scholar
  10. 10.
    Orfao A, Garcia-Montero AC, Sanchez L, Escribano L, REMA. Recent advances in the understanding of mastocytosis: the role of KIT mutations. Br J Haematol. 2007;138:12–30.CrossRefPubMedGoogle Scholar
  11. 11.
    Ma Y, Zeng S, Metcalfe DD, Akin C, Dimitrijevic S, Butterfield JH, McMahon G, Longley BJ. The c-KIT mutation causing human mastocytosis is resistant to STI571 and other KIT kinase inhibitors; kinases with enzymatic site mutations show different inhibitor sensitivity profiles than wild-type kinases and those with regulatory-type mutations. Blood. 2002;99:1741–4.CrossRefPubMedGoogle Scholar
  12. 12.
    Longley BJ, Tyrrell L, Lu SZ, Ma YS, Langley K, Ding TG, Duffy T, Jacobs P, Tang LH, Modlin I. Somatic c-KIT activating mutation in urticaria pigmentosa and aggressive mastocytosis: establishment of clonality in a human mast cell neoplasm. Nat Genet. 1996;12:312–4.CrossRefPubMedGoogle Scholar
  13. 13.
    Longley Jr BJ, Metcalfe DD, Tharp M, Wang X, Tyrrell L, Lu SZ, Heitjan D, Ma Y. Activating and dominant inactivating c-KIT catalytic domain mutations in distinct clinical forms of human mastocytosis. Proc Natl Acad Sci USA. 1999;96:1609–14.CrossRefPubMedCentralPubMedGoogle Scholar
  14. 14.
    Longley BJ, Reguera MJ, Ma Y. Classes of c-KIT activating mutations: proposed mechanisms of action and implications for disease classification and therapy. Leuk Res. 2001;25:571–6.CrossRefPubMedGoogle Scholar
  15. 15.
    Longley J. Is mastocytosis a mast cell neoplasia or a reactive hyperplasia? Clues from study of mast cell growth factor. Ann Med. 1994;26:115–6.CrossRefPubMedGoogle Scholar
  16. 16.
    Bodemer C, Hermine O, Palmerini F, Yang Y, Grandpeix-Guyodo C, Leventhal PS, et al. Pediatric mastocytosis is a clonal disease associated with D816 V and other activating c-KIT mutations. J Invest Dermatol. 2010;130:804–15.CrossRefPubMedGoogle Scholar
  17. 17.
    Yanagihori H, Oyama N, Nakamura K, Kaneko F. c-kit mutations in patients with childhood-onset mastocytosis and genotype-phenotype correlation. J Mol Diagn. 2005;7:252–7.CrossRefPubMedCentralPubMedGoogle Scholar
  18. 18.
    Ma D, Stence AA, Bossler AB, Hackman JR, Bellizzi AM. Identification of KIT activating mutations in paediatric solitary mastocytoma. Histopathology. 2014;64:218–25.CrossRefPubMedGoogle Scholar
  19. 19.
    Tang X, Boxer M, Drummond A, et al. A germline mutation in KIT in familial diffuse cutaneous mastocytosis. J Med Genet. 2004;41:e88.CrossRefPubMedCentralPubMedGoogle Scholar
  20. 20.
    Wang HJ, Lin ZM, Zhang J, Yin JH, Yang Y. A new germline mutation in KIT associated with diffuse cutaneous mastocytosis in a Chinese family. Clin Exp Dermatol. 2014;39:146–9.CrossRefPubMedGoogle Scholar
  21. 21.
    Wöhrl S, Moritz KB, Bracher A, Fischer G, Stingl G, Loewe R. A c-kit mutation in exon 18 in familial mastocytosis. J Invest Dermatol. 2013;133(3):839–41.CrossRefPubMedGoogle Scholar
  22. 22.
    Oku T, Hashizume H, Yokote R, et al. The familial occurrence of bullous mastocytosis (diffuse cutaneous mastocytosis). Arch Dermatol. 1990;126:1478–84.CrossRefPubMedGoogle Scholar
  23. 23.
    Kiszewski AE, Durán-Mckinster C, Orozco-Covarrubias L, Gutiérrez-Castrellón P, Ruiz-Maldonado R. Cutaneous mastocytosis in children: a clinical analysis of 71 cases. J Eur Acad Dermatol Venereol. 2004;18:285–90.CrossRefPubMedGoogle Scholar
  24. 24.
    Sondergaard J, Absoe-Hansen G. Mastocytosis in childhood. In: Happle R, Grosshans E, editors. Pediatric dermatology. Berlin: Springer-Verlag; 1987. p. 148–54.CrossRefGoogle Scholar
  25. 25.
    Kettelhut BV, Parker RI, Travis WD, Metcalfe DD. Hematopathology of the bone marrow in paediatric cutaneous mastocytosis: a study of 17 patients. J Clin Pathol. 1989;91:558–62.Google Scholar
  26. 26.
    Lange M, Niedoszytko M, Renke J, Gleń J, Nedoszytko B. Clinical aspects of paediatric mastocytosis: a review of 101 cases. J Eur Acad Dermatol Venereol. 2013;27:97–102.CrossRefPubMedGoogle Scholar
  27. 27.
    Stein DH. Mastocytosis: a review. Pediatr Dermatol. 1986;3:365–75.CrossRefPubMedGoogle Scholar
  28. 28.
    Schneider I, Schwartz RA. Mast cell disease. Cutis. 1997;50:63–6.Google Scholar
  29. 29.
    Castells M, Metcalfe DD, Escribano L. Diagnosis and treatment of cutaneous mastocytosis in children: practical recommendations. Am J Clin Dermatol. 2011;12:259–70.CrossRefPubMedCentralPubMedGoogle Scholar
  30. 30.
    Tharp MD. Southwestern internal medicine conference. The spectrum of mastocytosis. Am J Med Sci. 1985;289:117–32.CrossRefGoogle Scholar
  31. 31.
    Wolff K, Komar M, Petzelbauer P. Clinical and histopathological aspects of cutaneous mastocytosis. Leuk Res. 2001;25:519–28.CrossRefPubMedGoogle Scholar
  32. 32.
    Oranje AP, Soekanto W, Sukardi A, Vuzevski VD, van der Willigen A, Afiani HM. Diffuse cutaneous mastocytosis mimicking staphylococcal scalded-skin syndrome: report of three cases. Pediatr Dermatol. 1991;8:147–51.CrossRefPubMedGoogle Scholar
  33. 33.
    Verma KK, Bhat R, Singh MK. Bullous mastocytosis treated with oral betamethasone therapy. Indian J Pediatr. 2004;71:261–3.CrossRefPubMedGoogle Scholar
  34. 34.
    Heide R, Zuidema E, Beishuizen A, Den Hollander JC, Van Gysel D, Seyger MM, Pasmans SG, Kakourou T, Oranje AP. Clinical aspects of diffuse cutaneous mastocytosis in children: two variants. Dermatology. 2009;219:309–15.CrossRefPubMedGoogle Scholar
  35. 35.
    Husak R, Blume-Peytavi U, Pfrommer C, et al. Nodular and bullous cutaneous mastocytosis of the xanthelasmoid type: case report. Br J Dermatol. 2001;144:355–8.CrossRefPubMedGoogle Scholar
  36. 36.
    Rajesh J, Dogra S, Verma S, et al. Diffuse cutaneous mastocytosis: pseudoxanthomatous variant. J Dermatol. 2002;29:354–6.CrossRefPubMedGoogle Scholar
  37. 37.
    Hartmann K, Henz BM. Classification of cutaneous mastocytosis: a modified consensus proposal. Leuk Res. 2002;26:483–4.CrossRefPubMedGoogle Scholar
  38. 38.
    Azaña JM, Torrelo A, Mediero IG, Zambrano A. Urticaria pigmentosa: a review of 67 pediatric cases. Pediatr Dermatol. 1994;11:102–6.CrossRefPubMedGoogle Scholar
  39. 39.
    Berry T, Strand M, Smidt AC, Torrelo A. Localized mastocytosis of the vulva. Pediatr Dermatol. 2014;31:111–3.CrossRefPubMedGoogle Scholar
  40. 40.
    Sun Q, Zhou C, Hwang SK, Zhang J, Du J, Dai L. Intertriginous cutaneous mastocytosis in a 16-year-old boy. Int J Dermatol. 2014;53:e332–4.CrossRefPubMedGoogle Scholar
  41. 41.
    Verma R, Vasudevan B, Vijendran P, Mitra D. A rare case of bullous mastocytosis in a child associated with bathing trunk nevus. Int J Dermatol. 2014;53:761–3.CrossRefPubMedGoogle Scholar
  42. 42.
    Tsutsumi R, Watanabe T, Yoshida Y, Yamamoto O. Epidermal nevus syndrome with cutaneous mastocytosis. J Dermatol. 2012;39:1055–7.CrossRefPubMedGoogle Scholar
  43. 43.
    Kim CR, Kim HJ, Jung MY, Lee JH, Park JH, Lee DY, Lee JH, Yang JM. Cutaneous mastocytosis associated with congenital alopecia. Am J Dermatopathol. 2012;34:529–32.CrossRefPubMedGoogle Scholar
  44. 44.
    Vano-Galvan S, Alvarez-Twose I, De las Heras E, Morgado JM, Matito A, Sánchez-Muñoz L, Plana MN, Jaén P, Orfao A, Escribano L. Dermoscopic features of skin lesions in patients with mastocytosis. Arch Dermatol. 2011;147:932–40.Google Scholar
  45. 45.
    Alvarez-Twose I, Vano-Galvan S, Sanchez-Munoz L, Morgado JM, Matito A, Torrelo A, Jaen P, Schwartz LB, Orfao A, Escribano L. Increased serum baseline tryptase levels and extensive skin involvement are predictors for the severity of mast cell activation episodes in children with mastocytosis. Allergy. 2012;67:813–21.CrossRefPubMedCentralPubMedGoogle Scholar
  46. 46.
    Van Gysel D, van Schaik RHN, Oranje AP. Mastocytosis. In: Irvine AD, Hoeger PH, Yan AC, editors. Harper’s textbook of pediatric dermatology, vol. 1. 3rd ed. West Sussex: Wiley-Blackwell; 2011. p. 75.1–75.15.Google Scholar
  47. 47.
    Heide R, van Doorn K, Mulder PG, van Toorenenbergen AW, Beishuizen A, de Groot H, Tank B, Oranje AP. Serum tryptase and SCORMA (SCORing MAstocytosis) index as disease severity parameters in childhood and adult cutaneous mastocytosis. Clin Exp Dermatol. 2009;34:462–8.CrossRefPubMedGoogle Scholar
  48. 48.
    Brockow K, Ring J. Extensive blistering is a predictor for severe complications in children with mastocytosis. Allergy. 2012;67:1323–4.CrossRefPubMedGoogle Scholar
  49. 49.
    Escribano L, Alvarez-Twose I, Sánchez-Muñoz L, Garcia-Montero A, Núñez R, Almeida J, Jara-Acevedo M, Teodósio C, García-Cosío M, Bellas C, Orfao A. Prognosis in adult indolent systemic mastocytosis: a long-term study of the Spanish network on mastocytosis in a series of 145 patients. J Allergy Clin Immunol. 2009;124:514–21.CrossRefPubMedGoogle Scholar
  50. 50.
    Van Gysel D, Oranje AP, Vermeiden I, de Lijster de Raadt J, Mulder PG, van Toorenenbergen AW. Value of urinary N-methylhistamine measurements in childhood mastocytosis. J Am Acad Dermatol. 1996;35:556–8.Google Scholar
  51. 51.
    Barnes M, Van L, DeLong L, Lawley LP. Severity of cutaneous findings predict the presence of systemic symptoms in pediatric maculopapular cutaneous mastocytosis. Pediatr Dermatol. 2014;31:271–5.CrossRefPubMedGoogle Scholar
  52. 52.
    Brockow K, Akin C, Huber M, Metcalfe DD. Assessment of the extent of cutaneous involvement in children and adults with mastocytosis: relationship to symptomatology, tryptase levels, and bone marrow pathology. J Am Acad Dermatol. 2003;48:508–16.CrossRefPubMedGoogle Scholar
  53. 53.
    Murphy M, Walsh D, Drumm B, Watson R. Bullous mastocytosis: a fatal outcome. Pediatr Dermatol. 1999;16:452–5.CrossRefPubMedGoogle Scholar
  54. 54.
    Welch EA, Alper JC, Bogaars H, et al. Treatment of bullous mastocytosis with disodium cromoglycate. J Am Acad Dermatol. 1983;9:349–53.CrossRefPubMedGoogle Scholar
  55. 55.
    Escribano L, García-Belmonte D, Hernández-González A. Successful management of a case of diffuse cutaneous mastocytosis with recurrent anaphylactoid episodes and hypertension [abstract]. J Allergy Clin Immunol. 2004;113: S335.Google Scholar
  56. 56.
    Cardet JC, Akin C, Lee MJ. Mastocytosis: update on pharmacotherapy and future directions. Expert Opin Pharmacother. 2013;14:2033–45.CrossRefPubMedGoogle Scholar
  57. 57.
    Worobec AS. Treatment of systemic mast cell disorders. Hematol Oncol Clin North Am. 2000;14:659–87.CrossRefPubMedGoogle Scholar
  58. 58.
    Marone G, Spadaro G, Granata F, et al. Treatment of mastocytosis: pharmacologic basis and current concepts. Leuk Res. 2001;25:583–94.CrossRefPubMedGoogle Scholar
  59. 59.
    Escribano L, Akin C, Castells M, Schwartz LB. Current options in the treatment of mast cell mediator-related symptoms in mastocytosis. Inflamm Allergy Drug Targets. 2006;5:61–77.CrossRefPubMedGoogle Scholar
  60. 60.
    Debeuckelaere S, Schoors DF, Devis G. Systemic mast cell disease: a review of the literature with special focus on the gastrointestinal manifestations. Acta Clin Belg. 1991;46:226–32.PubMedGoogle Scholar
  61. 61.
    Fenske NA, Lober CW, Pautler SE. Congenital bullous urticaria pigmentosa: treatment with concomitant use of H1- and H2-receptor antagonists. Arch Dermatol. 1985;121:115–8.CrossRefPubMedGoogle Scholar
  62. 62.
    Gasior-Chrzan B, Falk ES. Systemic mastocytosis treated with histamine H1 and H2 receptor antagonists. Dermatologica. 1992;184:149–52.CrossRefGoogle Scholar
  63. 63.
    Soter NA, Austen KF, Wasserman SI. Oral disodium cromoglycate in the treatment of systemic mastocytosis. N Engl J Med. 1979;301:465–9.CrossRefPubMedGoogle Scholar
  64. 64.
    Horan RF, Sheffer AL, Austen KF. Cromolyn sodium in the management of systemic mastocytosis. J Allergy Clin Immunol. 1990;85:852–5.CrossRefPubMedGoogle Scholar
  65. 65.
    Czarnetzki BM, Behrendt H. Urticaria pigmentosa: clinical picture and response to oral disodium cromoglycate. Br J Dermatol. 1981;105:563–7.CrossRefPubMedGoogle Scholar
  66. 66.
    Welch EA, Alper JC, Bogaars H, Farrell DS. Treatment of bullous mastocytosis with disodium cromoglycate. J Am Acad Dermatol. 1983;9:349–53.CrossRefPubMedGoogle Scholar
  67. 67.
    Tolar J, Tope WD, Neglia JP. Leukotriene-receptor inhibition for the treatment of systemic mastocytosis. N Engl J Med. 2004;350:735–6.CrossRefPubMedGoogle Scholar
  68. 68.
    Turner PJ, Kemp AS, Rogers M, Mehr S. Refractory symptoms successfully treated with leukotriene inhibition in a child with systemic mastocytosis. Pediatr Dermatol. 2012;29:222–3.CrossRefPubMedGoogle Scholar
  69. 69.
    Kinsler VA, Hawk JL, Atherton DJ. Diffuse cutaneous mastocytosis treated with psoralen photochemotherapy: case report and review of the literature. Br J Dermatol. 2005;152:179–80.CrossRefPubMedGoogle Scholar
  70. 70.
    Mackey S, Pride HB, Tyler WB. Diffuse cutaneous mastocytosis: treatment with oral psoralen plus UV-A. Arch Dermatol. 1996;132:1429–30.CrossRefPubMedGoogle Scholar
  71. 71.
    Godt O, Proksch E, Streit V, Christophers E. Short- and long-term effectiveness of oral and bath PUVA therapy in urticaria pigmentosa and systemic mastocytosis. Dermatology. 1997;195:35–9.CrossRefPubMedGoogle Scholar
  72. 72.
    Czarnetzki BM, Rosenbach T, Kolde G, Frosch PJ. Phototherapy of urticaria pigmentosa: clinical response and changes of cutaneous reactivity, histamine and chemotactic leukotrienes. Arch Dermatol Res. 1985;277:105–13.CrossRefPubMedGoogle Scholar
  73. 73.
    Abdullah AN, Keczkes K. Cutaneous and ocular side-effects of PUVA photochemotherapy: a 10-year follow-up study. Clin Exp Dermatol. 1989;14:421–4.CrossRefPubMedGoogle Scholar
  74. 74.
    Stern RS, Nichols KT, Vakeva LH. Malignant melanoma in patients treated for psoriasis with methoxsalen (psoralen) and ultraviolet A radiation (PUVA): the PUVA follow-up study. N Engl J Med. 1997;336:1041–5.CrossRefPubMedGoogle Scholar
  75. 75.
    Carter MC, Robyn JA, Bressler PB, Walker JC, Shapiro GG, Metcalfe DD. Omalizumab for the treatment of unprovoked anaphylaxis in patients with systemic mastocytosis. J Allergy Clin Immunol. 2007;119:1550–1.CrossRefPubMedGoogle Scholar
  76. 76.
    Douglass JA, Carroll K, Voskamp A, Bourke P, Wei A, O’Hehir RE. Omalizumab is effective in treating systemic mastocytosis in a nonatopic patient. Allergy. 2010;65:926–7.Google Scholar
  77. 77.
    Matito A, Blázquez-Goñi C, Morgado JM, Alvarez-Twose I, Mollejo M, Sánchez-Muñoz L, Escribano L. Short-term omalizumab treatment in an adolescent with cutaneous mastocytosis. Ann Allergy Asthma Immunol. 2013;111:425–6.CrossRefPubMedGoogle Scholar
  78. 78.
    Molderings GJ, Raithel M, Kratz F, Azemar M, Haenisch B, Harzer S, Homann J. Omalizumab treatment of systemic mast cell activation disease: experiences from four cases. Intern Med. 2011;50:611–5.CrossRefPubMedGoogle Scholar
  79. 79.
    Vega-Ruiz A, Cortes JE, Sever M, Manshouri T, Quintás-Cardama A, Luthra R, Kantarjian HM, Verstovsek S. Phase II study of imatinib mesylate as therapy for patients with systemic mastocytosis. Leuk Res. 2009;33:1481–4.CrossRefPubMedCentralPubMedGoogle Scholar
  80. 80.
    Lim KH, Pardanani A, Butterfield JH, Li CY, Tefferi A. Cytoreductive therapy in 108 adults with systemic mastocytosis: outcome analysis and response prediction during treatment with interferon-alpha, hydroxyurea, imatinib mesylate or 2-chlorodeoxyadenosine. Am J Hematol. 2009;84:790–4.CrossRefPubMedGoogle Scholar
  81. 81.
    Ma Y, Zeng S, Metcalfe DD, Akin C, Dimitrijevic S, Butterfield JH, McMahon G, Longley BJ. The c-KIT mutation causing human mastocytosis is resistant to STI571 and other KIT kinase inhibitors; kinases with enzymatic site mutations show different inhibitor sensitivity profiles than wild-type kinases and those with regulatory-type mutations. Blood. 2002;99:1741–4.CrossRefPubMedGoogle Scholar
  82. 82.
    Agarwala MK, George R, Mathews V, Balasubramanian P, Thomas M, Nair S. Role of imatinib in the treatment of pediatric onset indolent systemic mastocytosis: a case report. J Dermatolog Treat. 2013;24:481–3.CrossRefPubMedGoogle Scholar
  83. 83.
    Morren MA, Hoppé A, Renard M, Debiec Rychter M, Uyttebroeck A, Dubreuil P, Martin L. Imatinib mesylate in the treatment of diffuse cutaneous mastocytosis. J Pediatr. 2013;162:205–7.CrossRefPubMedGoogle Scholar
  84. 84.
    Klein NJ, Misseldine S. Anesthetic considerations in pediatric mastocytosis: a review. J Anesth. 2013;27:588–98.CrossRefPubMedGoogle Scholar
  85. 85.
    Laxenaire MC, Mertes PM. Anaphylaxis during anaesthesia. Results of a two-year survey in France. Br J Anaesth. 2001;87:549–58.CrossRefPubMedGoogle Scholar
  86. 86.
    Mertes PM, Tajima K, Regnier-Kimmoun MA, Lambert M, Lohom G, Gueant-Rodriguez RM, Malinovsky JM. Perioperative anaphylaxis. Med Clin North Am. 2010;94:761–89, xi.Google Scholar
  87. 87.
    Gonzalez de Olano D, de la Hoz Caballer B, Nunez Lopez R, Sanchez Munoz L, Cuevas Agustin M, Dieguez MC, Alvarez Twose I, Castells MC, Escribano Mora L. 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. 2007;37:1547–55.Google Scholar
  88. 88.
    Greenhawt M, Akin C. Mastocytosis and allergy. Curr Opin Allergy Clin Immunol. 2007;7:387–92.CrossRefPubMedGoogle Scholar
  89. 89.
    Brockow K, Jofer C, Behrendt H, Ring J. Anaphylaxis in patients with mastocytosis: a study on history, clinical features and risk factors in 120 patients. Allergy. 2008;63:226–32.CrossRefPubMedGoogle Scholar
  90. 90.
    Carter MC, Uzzaman A, Scott LM, Metcalfe DD, Quezado Z. Pediatric mastocytosis: routine anesthetic management for a complex disease. Anesth Analg. 2008;107:422–7.CrossRefPubMedCentralPubMedGoogle Scholar
  91. 91.
    Ahmad N, Evans P, Lloyd-Thomas AR. Anesthesia in children with mastocytosis – a case based review. Paediatr Anaesth. 2009;19:97–107.CrossRefPubMedGoogle Scholar
  92. 92.
    Vaughan ST, Jones GN. Systemic mastocytosis presenting as profound cardiovascular collapse during anaesthesia. Anaesthesia. 1998;53:804–7.CrossRefPubMedGoogle Scholar
  93. 93.
    Desborough JP, Taylor I, Hattersley A, Garden A, Wolff A, Bloom SR, Morgan M. Massive histamine release in a patient with systemic mastocytosis. Br J Anaesth. 1990;65:833–6.CrossRefPubMedGoogle Scholar
  94. 94.
    Roberts 2nd LJ. Recurrent syncope due to systemic mastocytosis. Hypertension. 1984;6:285–94.PubMedGoogle Scholar
  95. 95.
    Florian S, Krauth MT, Simonitsch-Klupp I, Sperr WR, Fritsche-Polanz R, Sonneck K, Fodinger M, Agis H, Bohm A, Wimazal F, Horny HP, Valent P. Indolent systemic mastocytosis with elevated serum tryptase, absence of skin lesions, and recurrent severe anaphylactoid episodes. Int Arch Allergy Immunol. 2005;136:273–80.CrossRefPubMedGoogle Scholar
  96. 96.
    Tirel O, Chaumont A, Ecoffey C. Circulatory arrest in the course of anesthesia for a child with mastocytosis. Ann Fr Anesth Reanim. 2001;20:874–5.CrossRefPubMedGoogle Scholar
  97. 97.
    Goldfinger MM, Sandadi J. Undiagnosed systemic mastocytosis in a teenager revealed during general anesthesia. Paediatr Anaesth. 2010;20:290–1.CrossRefPubMedGoogle Scholar
  98. 98.
    Chaar CI, Bell RL, Duffy TP, Duffy AJ. Guidelines for safe surgery in patients with systemic mastocytosis. Am Surg. 2009;75:74–80.PubMedGoogle Scholar
  99. 99.
    Valent P, Escribano L, Broesby-Olsen S, Hartmann K, Grattan C, Brockow K, Niedoszytko M, Nedoszytko B, Oude Elberink JN, Kristensen T, Butterfield JH, Triggiani M, Alvarez-Twose I, Reiter A, Sperr WR, Sotlar K, Yavuz S, Kluin-Nelemans HC, Hermine O, Radia D, van Doormaal JJ, Gotlib J, Orfao A, Siebenhaar F, Schwartz LB, Castells M, Maurer M, Horny HP, Akin C, Metcalfe DD, Arock M. Proposed diagnostic algorithm for patients with suspected mastocytosis: a proposal of the European competence network on mastocytosis. Allergy. 2014;69:1267–74. doi: 10.1111/all.12436.

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© Springer Science+Business Media New York 2015

Authors and Affiliations

  1. 1.Division of DermatologyUniversity Medicine Cluster, National University HospitalSingaporeSingapore

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