Cutaneous Mastocytosis

  • Jason R. Catanzaro
  • Haig TcheurekdjianEmail author


Mastocytosis is a group of disorders characterized by an abnormal accumulation of mast cells in one or more organ systems. Cutaneous mastocytosis, by definition, is the restriction of mast cells to the skin although patients with this disease may have both cutaneous and systemic symptoms. Recognition of ­cutaneous mastocytosis is important, especially in children, as its prognosis is favorable in the majority of cases, and, while there is no cure, treatment options are available for symptomatic relief. Annual visits to monitor for signs of progression to systemic disease as well as patient education about potential triggers and consequences of mast cell degranulation are an important component of management.


Cutaneous mastocytosis Urticaria pigmentosa Mastocytoma Tryptase Telangiectasia macularis eruptiva perstans Darier’s sign 



We gratefully thank Kord Honda, MD, Assistant Professor of Dermatology and Director of Dermatopathology at Case Western Reserve University, for providing the images of mastocytosis histology.


  1. 1.
    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; 7:252–257.PubMedCrossRefGoogle Scholar
  2. 2.
    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; 63:226–232.PubMedCrossRefGoogle Scholar
  3. 3.
    Akay B, Kittler H, Sanli H, Harmankaya K, Anadolu R. Dermatoscopic findings of cutaneous mastocytosis. Dermatology 2009; 226–230.Google Scholar
  4. 4.
    Komarow H, Hu Z, Brittain E, Uzzaman A, Gaskins D, Metcalfe DD. Serum tryptase levels in atopic and nonatopic children. J Allergy Clin Immunol. 2009; 124(4):845–848.PubMedCrossRefGoogle Scholar
  5. 5.
    Heide R, Beishuizen A, De Groot H, Den Hollander JC, Van Doormaal JJ, De Monchy JGR, Pasmans S, Van Gysel D, Oranje AP. Mastocytosis in children: a protocol for management. Pediatr Dermatol. 2008; 25(4):493–500.PubMedCrossRefGoogle Scholar
  6. 6.
    Valent P, Sperr W, Schwartz L, Horny H. Diagnosis and classification of mast cell proliferative disorders: delineation from immunologic diseases and non-mast cell hematopoietic neoplasms. J Allergy Clin Immunol. 2004; 114:3–11.PubMedCrossRefGoogle Scholar

Copyright information

© Springer Science+Business Media, LLC 2010

Authors and Affiliations

  1. 1.Allergy/Immunology Associates, IncClevelandUSA
  2. 2.Department of Medicine and PediatricsCase Western Reserve UniversityClevelandUSA

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