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Other Proliferative Disorders of the Skin

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Skin Tumors and Reactions to Cancer Therapy in Children

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Abstract

Manifestations of mastocytosis and Langerhans cell histiocytosis (LCH) range from relatively benign cutaneous proliferations of mast cells and histiocytes, respectively, to severe, life-threatening multi-organ disease. This chapter will address each condition’s pathogenesis, natural history, clinical features, treatments, and potential complications—essential information for medical professionals caring for children. Both mastocytosis and LCH are rare disorders, and medical professionals must be prepared to appropriately counsel and refer patients to appropriate specialists to prevent serious systemic complications.

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Recommended Reading

  • Bodemer C, Hermine O, PalmĂ©rini F, Yang Y, Grandpeix-Guyodo C, Leventhal PS, et al. Pediatric mastocytosis is a clonal disease associated with D816V and other activating c-KIT mutations. J Invest Dermatol. 2010;130(3):804–15.

    Article  CAS  PubMed  Google Scholar 

  • Campo E, Swerdlow SH, Harris NL, Pileri S, Stein H, Jaffe ES. The 2008 WHO classification of lymphoid neoplasms and beyond: evolving concepts and practical applications. Blood. 2011;117(19):5019–32.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  • Hartmann K, Escribano L, Grattan C, Brockow K, Carter MC, Alvarez-Twose I, et al. Cutaneous manifestations in patients with mastocytosis: consensus report of the European competence network on mastocytosis; the American Academy of Allergy, Asthma & Immunology; and the European academy of Allergology and clinical immunology. J Allergy Clin Immunol. 2016;137(1):35–45.

    Article  PubMed  Google Scholar 

  • Haupt R, Minkov M, Astigarraga I, Schäfer E, Nanduri V, Jubran R, Euro Histio Network, et al. Langerhans cell histiocytosis (LCH): guidelines for diagnosis, clinical work-up, and treatment for patients till the age of 18 years. Pediatr Blood Cancer. 2013;60(2):175–84.

    Article  PubMed  Google Scholar 

  • Lee JW, Shin HY, Kang HJ, Kim H, Park JD, Park KD, et al. Clinical characteristics and treatment outcome of Langerhans cell histiocytosis: 22 years’ experience of 154 patients at a single center. Pediatr Hematol Oncol. 2014;31(3):293–302.

    Article  PubMed  Google Scholar 

  • Matito A, Morgado JM, Sanchez-Lopez P, Alvarez-Twose I, Sanchez-Munoz L, Orfao A, Escribano L. Management of Anesthesia in adult and pediatric Mastocytosis: a study of the Spanish network on Mastocytosis (REMA) based on 726 anesthetic procedures. Int Arch Allergy Immunol. 2015;167(1):47–56.

    Article  CAS  PubMed  Google Scholar 

  • Meni C, Bruneau J, Georgin-Lavialle S, Le Sache de Peufeilhoux L, Damaj G, Hadj-Rabia S, et al. Paediatric mastocytosis: a systematic review of 1747 cases. Br J Dermatol. 2015;172(3):642–51.

    Article  CAS  PubMed  Google Scholar 

  • Simko SJ, Garmezy B, Abhyankar H, Lupo PJ, Chakraborty R, Lim KP, et al. Differentiating skin-limited and multisystem Langerhans cell histiocytosis. J Pediatr. 2014;165(5):990–6.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  • Theoharides TC, Valent P, Akin C. Mast cell s, Mastocytosis, and related disorders. N Engl J Med. 2015;373(2):163–72.

    Google Scholar 

  • Uzzaman A, Maric I, Noel P, Kettelhut BV, Metcalfe DD, Carter MC. Pediatric-onset mastocytosis: a long term clinical follow-up and correlation with bone marrow histopathology. Pediatr Blood Cancer. 2009;53(4):629–34.

    Article  PubMed  PubMed Central  Google Scholar 

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Authors and Affiliations

  1. Department of Dermatology, Emory University, 1525 Clifton Road, 3rd Floor, Atlanta, GA, 30322, USA

    Emily A. Gurnee M.D. & Leslie P. Lawley M.D.

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  1. Emily A. Gurnee M.D.
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  2. Leslie P. Lawley M.D.
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Correspondence to Leslie P. Lawley M.D. .

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  1. Harvard Medical School, Boston Children’s Hospital, Boston, Massachusetts, USA

    Jennifer T. Huang

  2. Washington University School of Medicine, St. Louis, Missouri, USA

    Carrie C. Coughlin

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Gurnee, E.A., Lawley, L.P. (2018). Other Proliferative Disorders of the Skin. In: Huang, J., Coughlin, C. (eds) Skin Tumors and Reactions to Cancer Therapy in Children. Springer, Cham. https://doi.org/10.1007/978-3-319-66200-8_4

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