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Classification of Mastocytosis

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Practical Pediatric Dermatology

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Abstract

The term “mastocytosis” denotes a heterogeneous group of disorders, characterized by local or diffuse increased growth and accumulation of mast cells in the skin and/or in internal organs. Most classifications consider cutaneous mastocytosis and systemic mastocytosis as mutually exclusive conditions. However, as bone marrow studies to demonstrate a systemic involvement are not routinely performed nor recommended in children, a classification of pediatric mastocytosis according to the morphological appearance of the lesions can be followed.

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References

  • Bennett JM, Catovsky D, Daniel MT, Flandrin G, Galton DA, Gralnick H, Sultan C, Cox C. The chronic myeloid leukaemias: guidelines for distinguishing chronic granulocytic, atypical chronic myeloid, and chronic myelomonocytic leukaemia. Proposals by the French-American-British Cooperative Leukaemia Group. Br J Haematol. 1994;87(4):746–54.

    Article  CAS  PubMed  Google Scholar 

  • Birt AR, Nickerson M. Generalized flushing of the skin with urticaria pigmentosa. Arch Dermatol. 1959;80:311–7.

    Article  CAS  PubMed  Google Scholar 

  • 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(4):508–16. doi:10.1067/mjd.2003.98.

    Article  PubMed  Google Scholar 

  • Degos R. Urticaria pigmentosa and other types of mastocytosis; attempted classification of cutaneous mastocytoses. Actas Dermosifiliogr. 1955;46(9):759–85.

    CAS  PubMed  Google Scholar 

  • Efrati P, Klajman A, Spitz H. Mast cell leukemia? Malignant mastocytosis with leukemia-like manifestations. Blood. 1957;12(10):869–82.

    CAS  PubMed  Google Scholar 

  • Ehrlich P. Berträge zur Kenntnis der anilifarbungen und ihrer Verwendung in der microscopischem Technik. Arch Mikros Anat. 1877;13:163–77.

    Article  Google Scholar 

  • Ellis JM. Urticaria pigmentosa; a report of a case with autopsy. Arch Pathol. 1949;48(5):426–35.

    CAS  Google Scholar 

  • Harris NL, Jaffe ES, Diebold J, Flandrin G, Muller-Hermelink HK, Vardiman J, Lister TA, Bloomfield CD. The World Health Organization classification of neoplastic diseases of the hematopoietic and lymphoid tissues. Report of the Clinical Advisory Committee meeting, Airlie House, Virginia, November, 1997. Ann Oncol. 1999;10(12):1419–32.

    Article  CAS  PubMed  Google Scholar 

  • Hartmann K, Henz BM. Classification of cutaneous mastocytosis: a modified consensus proposal. Leuk Res. 2002;26(5):483–4; discussion 485–486.

    Article  PubMed  Google Scholar 

  • Heide R, Beishuizen A, De Groot H, Den Hollander JC, Van Doormaal JJ, De Monchy JG, Pasmans SG, Van Gysel D, Oranje AP. Mastocytosis in children: a protocol for management. Pediatr Dermatol. 2008;25(4):493–500. doi:PDE738 [pii] 10.1111/j.1525-1470.2008.00738.x.

  • 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(4):309–15. doi:10.1159/000243808.

    Article  PubMed  Google Scholar 

  • Horan RF, Schneider LC, Sheffer AL. Allergic skin disorders and mastocytosis. JAMA. 1992;268(20):2858–68.

    Article  CAS  PubMed  Google Scholar 

  • Horny HP, Akin C, Metcalfe D. Mastocytosis. In: Swerdlow SH, Campo E, Harris NL, et al., editors. World Health Organization (WHO) classification of tumours of haematopoietic and lymphoid origin, vol. 2. 4th ed. Lyon: IARC Press; 2008. p. 54–63.

    Google Scholar 

  • Kettelhut BV, Metcalfe DD. Pediatric mastocytosis. J Invest Dermatol. 1991;96(3):15S–8.

    Article  Google Scholar 

  • Lennert K, Parwaresch MR. Mast cells and mast cell neoplasia: a review. Histopathology. 1979;3(5):349–65.

    Article  CAS  PubMed  Google Scholar 

  • Leonardi S, Vitaliti G, Pratico AD, La Rosa M. Telangiectasia macularis eruptiva perstans (TMEP) in childhood: a case report and literature review. Allergol Immunopathol (Madr). 2012;40(5):321–3. doi:10.1016/j.aller.2011.05.006.

    Article  CAS  Google Scholar 

  • Longley J, Duffy TP, Kohn S. The mast cell and mast cell disease. J Am Acad Dermatol. 1995;32(4):545–61; quiz 562–544.

    Article  CAS  PubMed  Google Scholar 

  • Marney Jr SR. Mast cell disease. Allergy Proc. 1992;13(6):303–10.

    Article  PubMed  Google Scholar 

  • Metcalfe DD. Classification and diagnosis of mastocytosis: current status. J Invest Dermatol. 1991a;96(3):2S–4.

    Article  Google Scholar 

  • Metcalfe DD. Conclusions. Clinical advances in mastocytosis: an interdisciplinary round table discussion. J Invest Dermatol. 1991b;96(3):S64–5.

    Article  Google Scholar 

  • Munro CS, Farr PM. Solitary mastocytoma causing recurrent blistering in infancy. Arch Dis Child. 1992;67(8):1038–9.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  • Nettleship J, Tay W. Rare forms of urticaria. Br Med J. 1869;2:323–4.

    Article  Google Scholar 

  • 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(2):147–51.

    Article  CAS  PubMed  Google Scholar 

  • Requena L. Erythrodermic mastocytosis. Cutis. 1992;49(3):189–92.

    CAS  PubMed  Google Scholar 

  • Sangster A. An anomalous mottled rash, accompanied by pruritus, factitious urticaria and pigmentation, ‘urticaria pigmentosa (?)’. Trans Clin Soc London. 1878;11:161–3.

    Google Scholar 

  • Selye H. Diseases. In: Selye H, editor. The mast cells. London: Butterworth; 1965. p. 262–75.

    Google Scholar 

  • Sézary A, Levy-Coblentz G, Chauvillon P. Dermographisme et mastocytose. Bull Soc Fr Dermatol Syphiligr. 1936;43:359–61.

    Google Scholar 

  • Soter NA. The skin in mastocytosis. J Invest Dermatol. 1991;96(3):32S–8; discussion 38S–39S.

    Article  Google Scholar 

  • Soter NA. Mastocytosis and the skin. Hematol Oncol Clin North Am. 2000;14(3):537–55, vi.

    Article  CAS  PubMed  Google Scholar 

  • Stein DH. Mastocytosis: a review. Pediatr Dermatol. 1986;3(5):365–75.

    Article  CAS  PubMed  Google Scholar 

  • Tefferi A, Vardiman JW. Classification and diagnosis of myeloproliferative neoplasms: the 2008 World Health Organization criteria and point-of-care diagnostic algorithms. Leukemia. 2008;22(1):14–22. doi:10.1038/sj.leu.2404955.

    Article  CAS  PubMed  Google Scholar 

  • Torrelo A, Alvarez-Twose I, Escribano L. Childhood mastocytosis. Curr Opin Pediatr. 2012;24(4):480–6. doi:10.1097/MOP.0b013e328355b248.

    PubMed  Google Scholar 

  • Travis WD, Li CY, Bergstralh EJ, Yam LT, Swee RG. Systemic mast cell disease. Analysis of 58 cases and literature review. Medicine (Baltimore). 1988;67(6):345–68.

    Article  CAS  Google Scholar 

  • Unna PG. Beitrage zur Anatomie und Pathogenese der Urticaria simplex und pigmentosa. Mschr Prakt Dermatol. 1887;6:1.

    Google Scholar 

  • Valent P, Akin C, Arock M, Brockow K, Butterfield JH, Carter MC, Castells M, Escribano L, Hartmann K, Lieberman P, Nedoszytko B, Orfao A, Schwartz LB, Sotlar K, Sperr WR, Triggiani M, Valenta R, Horny HP, Metcalfe DD. Definitions, criteria and global classification of mast cell disorders with special reference to mast cell activation syndromes: a consensus proposal. Int Arch Allergy Immunol. 2012;157(3):215–25. doi:10.1159/000328760.

    Article  PubMed  Google Scholar 

  • Valent P, Akin C, Escribano L, Fodinger 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(6):435–53. doi:10.1111/j.1365-2362.2007.01807.x.

    Article  CAS  PubMed  Google Scholar 

  • Valent P, Horny HP, Escribano L, Longley BJ, Li CY, Schwartz LB, Marone G, Nunez R, Akin C, Sotlar K, Sperr WR, Wolff K, Brunning RD, Parwaresch RM, Austen KF, Lennert K, Metcalfe DD, Vardiman JW, Bennett JM. Diagnostic criteria and classification of mastocytosis: a consensus proposal. Leuk Res. 2001a;25(7):603–25.

    Article  CAS  PubMed  Google Scholar 

  • Valent P, Horny HP, Li CY, Longley BJ, Metcalfe D, Parwaresch MR, Bennett JM. Mastocytosis (mast cell disease). In: Jaffe ES, Harris NL, Stein H, Vardiman J, editors. World Health Organization (WHO) classification of tumours: pathology and genetics, Tumours of haematopoietic and lymphoid tissues, vol. 1. Lyon: IARC Press; 2001b. p. 291–302.

    Google Scholar 

  • Van Gysel D, van Schaik RH, Oranje AP. Mastocytosis. In: Irvine AD, Hoeger PH, Yan AC, editors. Harper’s textbook of paediatric dermatology, vol. 1. 3rd ed. West Sussex: Wiley-Blackwell; 2011. p. 75.71–15.

    Google Scholar 

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Authors and Affiliations

  1. Department of Pediatrics, O. L. Vrouw Hospital, Moorselbaan, Aalst, Belgium

    Dirk Van Gysel MD, PhD

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  1. Dirk Van Gysel MD, PhD
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Correspondence to Dirk Van Gysel MD, PhD .

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Editors and Affiliations

  1. Rotterdam, The Netherlands

    Arnold P. Oranje

  2. Farwaniya, Kuwait

    Nawaf Al-Mutairi

  3. Department of Dermatology, Henry Ford Hospital, Detroit, Michigan, USA

    Tor Shwayder

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Van Gysel, D. (2016). Classification of Mastocytosis. In: Oranje, A., Al-Mutairi, N., Shwayder, T. (eds) Practical Pediatric Dermatology. Springer, Cham. https://doi.org/10.1007/978-3-319-32159-2_20

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  • DOI: https://doi.org/10.1007/978-3-319-32159-2_20

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