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Pediatric Skin of Color pp 327–337Cite as

Pediatric Mastocytosis

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Abstract

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.

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References

  1. Kettelhut BV, Metcalfe DD. Pediatric mastocytosis. J Invest Dermatol. 1991;96:15S–8S.

    Article  Google Scholar 

  2. Ben-Amitai D, Metzker A, Cohen HA. Pediatric cutaneous mastocytosis: a review of 180 patients. Isr Med Assoc J. 2005;7:320–2.

    PubMed  Google Scholar 

  3. Hannaford R, Rogers M. Presentation of cutaneous mastocytosis in 173 children. Australas J Dermatol. 2001;42:15–21.

    Article  CAS  PubMed  Google Scholar 

  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.

    Article  CAS  PubMed  Google Scholar 

  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.

    Article  CAS  PubMed  Google Scholar 

  6. Valent P, Horny HP. Diagnostic criteria and classification of mastocytosis: consensus proposal. Leuk Res. 2001;25:603–25.

    Article  CAS  PubMed  Google Scholar 

  7. Torrelo A, Alvarez-Twose I, Escribano L. Childhood mastocytosis. Curr Opin Pediatr. 2012;24:480–6.

    PubMed  Google Scholar 

  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.

    Article  CAS  PubMed  Google Scholar 

  9. Kneilling M, Röcken M. Mast cells: novel clinical perspectives from recent insights. Exp Dermatol. 2009;18:488–96.

    Article  CAS  PubMed  Google Scholar 

  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.

    Article  CAS  PubMed  Google Scholar 

  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.

    Article  CAS  PubMed  Google Scholar 

  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.

    Article  CAS  PubMed  Google Scholar 

  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.

    Article  PubMed Central  CAS  PubMed  Google Scholar 

  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.

    Article  CAS  PubMed  Google Scholar 

  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.

    Article  CAS  PubMed  Google Scholar 

  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.

    Article  CAS  PubMed  Google Scholar 

  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.

    Article  PubMed Central  CAS  PubMed  Google Scholar 

  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.

    Article  PubMed  Google Scholar 

  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.

    Article  PubMed Central  CAS  PubMed  Google Scholar 

  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.

    Article  CAS  PubMed  Google Scholar 

  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.

    Article  PubMed  Google Scholar 

  22. Oku T, Hashizume H, Yokote R, et al. The familial occurrence of bullous mastocytosis (diffuse cutaneous mastocytosis). Arch Dermatol. 1990;126:1478–84.

    Article  CAS  PubMed  Google Scholar 

  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.

    Article  CAS  PubMed  Google Scholar 

  24. Sondergaard J, Absoe-Hansen G. Mastocytosis in childhood. In: Happle R, Grosshans E, editors. Pediatric dermatology. Berlin: Springer-Verlag; 1987. p. 148–54.

    Chapter  Google Scholar 

  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.

    CAS  Google Scholar 

  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.

    Article  CAS  PubMed  Google Scholar 

  27. Stein DH. Mastocytosis: a review. Pediatr Dermatol. 1986;3:365–75.

    Article  CAS  PubMed  Google Scholar 

  28. Schneider I, Schwartz RA. Mast cell disease. Cutis. 1997;50:63–6.

    Google Scholar 

  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.

    Article  PubMed Central  PubMed  Google Scholar 

  30. Tharp MD. Southwestern internal medicine conference. The spectrum of mastocytosis. Am J Med Sci. 1985;289:117–32.

    Article  Google Scholar 

  31. Wolff K, Komar M, Petzelbauer P. Clinical and histopathological aspects of cutaneous mastocytosis. Leuk Res. 2001;25:519–28.

    Article  CAS  PubMed  Google Scholar 

  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.

    Article  CAS  PubMed  Google Scholar 

  33. Verma KK, Bhat R, Singh MK. Bullous mastocytosis treated with oral betamethasone therapy. Indian J Pediatr. 2004;71:261–3.

    Article  PubMed  Google Scholar 

  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.

    Article  PubMed  Google Scholar 

  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.

    Article  CAS  PubMed  Google Scholar 

  36. Rajesh J, Dogra S, Verma S, et al. Diffuse cutaneous mastocytosis: pseudoxanthomatous variant. J Dermatol. 2002;29:354–6.

    Article  PubMed  Google Scholar 

  37. Hartmann K, Henz BM. Classification of cutaneous mastocytosis: a modified consensus proposal. Leuk Res. 2002;26:483–4.

    Article  PubMed  Google Scholar 

  38. Azaña JM, Torrelo A, Mediero IG, Zambrano A. Urticaria pigmentosa: a review of 67 pediatric cases. Pediatr Dermatol. 1994;11:102–6.

    Article  PubMed  Google Scholar 

  39. Berry T, Strand M, Smidt AC, Torrelo A. Localized mastocytosis of the vulva. Pediatr Dermatol. 2014;31:111–3.

    Article  PubMed  Google Scholar 

  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.

    Article  PubMed  Google Scholar 

  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.

    Article  PubMed  Google Scholar 

  42. Tsutsumi R, Watanabe T, Yoshida Y, Yamamoto O. Epidermal nevus syndrome with cutaneous mastocytosis. J Dermatol. 2012;39:1055–7.

    Article  PubMed  Google Scholar 

  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.

    Article  CAS  PubMed  Google Scholar 

  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. 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.

    Article  PubMed Central  CAS  PubMed  Google Scholar 

  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. 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.

    Article  CAS  PubMed  Google Scholar 

  48. Brockow K, Ring J. Extensive blistering is a predictor for severe complications in children with mastocytosis. Allergy. 2012;67:1323–4.

    Article  PubMed  Google Scholar 

  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.

    Article  PubMed  Google Scholar 

  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. 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.

    Article  PubMed  Google Scholar 

  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.

    Article  PubMed  Google Scholar 

  53. Murphy M, Walsh D, Drumm B, Watson R. Bullous mastocytosis: a fatal outcome. Pediatr Dermatol. 1999;16:452–5.

    Article  CAS  PubMed  Google Scholar 

  54. Welch EA, Alper JC, Bogaars H, et al. Treatment of bullous mastocytosis with disodium cromoglycate. J Am Acad Dermatol. 1983;9:349–53.

    Article  CAS  PubMed  Google Scholar 

  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. Cardet JC, Akin C, Lee MJ. Mastocytosis: update on pharmacotherapy and future directions. Expert Opin Pharmacother. 2013;14:2033–45.

    Article  CAS  PubMed  Google Scholar 

  57. Worobec AS. Treatment of systemic mast cell disorders. Hematol Oncol Clin North Am. 2000;14:659–87.

    Article  CAS  PubMed  Google Scholar 

  58. Marone G, Spadaro G, Granata F, et al. Treatment of mastocytosis: pharmacologic basis and current concepts. Leuk Res. 2001;25:583–94.

    Article  CAS  PubMed  Google Scholar 

  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.

    Article  CAS  PubMed  Google Scholar 

  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.

    CAS  PubMed  Google Scholar 

  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.

    Article  CAS  PubMed  Google Scholar 

  62. Gasior-Chrzan B, Falk ES. Systemic mastocytosis treated with histamine H1 and H2 receptor antagonists. Dermatologica. 1992;184:149–52.

    Article  CAS  Google Scholar 

  63. Soter NA, Austen KF, Wasserman SI. Oral disodium cromoglycate in the treatment of systemic mastocytosis. N Engl J Med. 1979;301:465–9.

    Article  CAS  PubMed  Google Scholar 

  64. Horan RF, Sheffer AL, Austen KF. Cromolyn sodium in the management of systemic mastocytosis. J Allergy Clin Immunol. 1990;85:852–5.

    Article  CAS  PubMed  Google Scholar 

  65. Czarnetzki BM, Behrendt H. Urticaria pigmentosa: clinical picture and response to oral disodium cromoglycate. Br J Dermatol. 1981;105:563–7.

    Article  CAS  PubMed  Google Scholar 

  66. Welch EA, Alper JC, Bogaars H, Farrell DS. Treatment of bullous mastocytosis with disodium cromoglycate. J Am Acad Dermatol. 1983;9:349–53.

    Article  CAS  PubMed  Google Scholar 

  67. Tolar J, Tope WD, Neglia JP. Leukotriene-receptor inhibition for the treatment of systemic mastocytosis. N Engl J Med. 2004;350:735–6.

    Article  CAS  PubMed  Google Scholar 

  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.

    Article  PubMed  Google Scholar 

  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.

    Article  CAS  PubMed  Google Scholar 

  70. Mackey S, Pride HB, Tyler WB. Diffuse cutaneous mastocytosis: treatment with oral psoralen plus UV-A. Arch Dermatol. 1996;132:1429–30.

    Article  CAS  PubMed  Google Scholar 

  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.

    Article  CAS  PubMed  Google Scholar 

  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.

    Article  CAS  PubMed  Google Scholar 

  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.

    Article  CAS  PubMed  Google Scholar 

  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.

    Article  CAS  PubMed  Google Scholar 

  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.

    Article  CAS  PubMed  Google Scholar 

  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. 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.

    Article  PubMed  Google Scholar 

  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.

    Article  CAS  PubMed  Google Scholar 

  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.

    Article  PubMed Central  CAS  PubMed  Google Scholar 

  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.

    Article  CAS  PubMed  Google Scholar 

  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.

    Article  CAS  PubMed  Google Scholar 

  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.

    Article  PubMed  Google Scholar 

  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.

    Article  PubMed  Google Scholar 

  84. Klein NJ, Misseldine S. Anesthetic considerations in pediatric mastocytosis: a review. J Anesth. 2013;27:588–98.

    Article  PubMed  Google Scholar 

  85. Laxenaire MC, Mertes PM. Anaphylaxis during anaesthesia. Results of a two-year survey in France. Br J Anaesth. 2001;87:549–58.

    Article  CAS  PubMed  Google Scholar 

  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. 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. Greenhawt M, Akin C. Mastocytosis and allergy. Curr Opin Allergy Clin Immunol. 2007;7:387–92.

    Article  PubMed  Google Scholar 

  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.

    Article  CAS  PubMed  Google Scholar 

  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.

    Article  PubMed Central  PubMed  Google Scholar 

  91. Ahmad N, Evans P, Lloyd-Thomas AR. Anesthesia in children with mastocytosis – a case based review. Paediatr Anaesth. 2009;19:97–107.

    Article  PubMed  Google Scholar 

  92. Vaughan ST, Jones GN. Systemic mastocytosis presenting as profound cardiovascular collapse during anaesthesia. Anaesthesia. 1998;53:804–7.

    Article  CAS  PubMed  Google Scholar 

  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.

    Article  CAS  PubMed  Google Scholar 

  94. Roberts 2nd LJ. Recurrent syncope due to systemic mastocytosis. Hypertension. 1984;6:285–94.

    PubMed  Google Scholar 

  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.

    Article  PubMed  Google Scholar 

  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.

    Article  CAS  PubMed  Google Scholar 

  97. Goldfinger MM, Sandadi J. Undiagnosed systemic mastocytosis in a teenager revealed during general anesthesia. Paediatr Anaesth. 2010;20:290–1.

    Article  PubMed  Google Scholar 

  98. Chaar CI, Bell RL, Duffy TP, Duffy AJ. Guidelines for safe surgery in patients with systemic mastocytosis. Am Surg. 2009;75:74–80.

    PubMed  Google Scholar 

  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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  1. Division of Dermatology, University Medicine Cluster, National University Hospital, 1E Kent Ridge Road, NUHS Tower Block, Level 10, 119228, Singapore, Singapore

    Nisha Suyien Chandran M.R.C.P. (UK)

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  1. Nisha Suyien Chandran M.R.C.P. (UK)
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Correspondence to Nisha Suyien Chandran M.R.C.P. (UK) .

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Editors and Affiliations

  1. Department of Dermatology, Mt. Sinai St. Luke’s-Roosevelt Hospital and Beth Israel Medical centers, New York, New York, USA

    Nanette B. Silverberg

  2. Department of Pediatric Dermatology, National Institute of Pediatrics, Mexico City, Mexico

    Carola Durán-McKinster

  3. Department of Dermatology, Changi General Hospital, Singapore, Singapore

    Yong-Kwang Tay

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Chandran, N.S. (2015). Pediatric Mastocytosis. In: Silverberg, N., Durán-McKinster, C., Tay, YK. (eds) Pediatric Skin of Color. Springer, New York, NY. https://doi.org/10.1007/978-1-4614-6654-3_37

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