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American Journal of Clinical Dermatology

, Volume 12, Issue 4, pp 259–270 | Cite as

Diagnosis and Treatment of Cutaneous Mastocytosis in Children

Practical Recommendations
  • Mariana Castells
  • Dean D. Metcalfe
  • Luis Escribano
Review Article

Abstract

Cutaneous mastocytosis in children is a generally benign disease that can present at birth and is often associated with mast cell mediator-related symptoms including pruritus, flushing, and abdominal pain with diarrhea. The most common form of presentation is urticaria pigmentosa, also referred to as maculopapular mastocytosis. Flares of lesions are induced by triggers such as physical stimuli, changes in temperature, anxiety, medications, and exercise. The skin lesions are typically present on the extremities. Symptoms respond to topical and systemic anti-mediator therapy including antihistamines and cromolyn sodium. Remission at puberty is seen in a majority of cases. Progression to systemic mastocytosis with involvement of extracutaneous organs is not common.

The cause of cutaneous mastocytosis is unknown and familial cases are rare. Mutations of c-kit have been observed in the skin of those affected. The diagnosis is established on clinical grounds and the findings on skin biopsy. Bone marrow studies are recommended if there is suspicion of progression of disease to an adult form, if cytoreductive therapy is contemplated, or if skin lesions remain present and/or tryptase levels remain elevated after puberty. The use of chemotherapy, including kinase inhibitors, is strongly discouraged unless severe hematologic disease is present, since malignant evolution is extremely rare.

Keywords

Mast Cell Tryptase Mastocytosis Systemic Mastocytosis Cromolyn Sodium 
These keywords were added by machine and not by the authors. This process is experimental and the keywords may be updated as the learning algorithm improves.

Notes

Acknowledgments

This work was in part supported by the Division of Intramural Research, National Institute of Allergy and Infectious Diseases/National Institutes of Health (NIAID/NIH) and in part by a grant from The Mastocytosis Society towards funding the Mastocytosis Registry at BWH, headed by Dr Mariana Castells. Research funding was received from the Foundation for Research of Castilla La Mancha (FISCAM 2007/36, FISCAM 2008/46), the Ministry of Health (FIS PS09/00032), and the Spanish Mastocytosis Foundation (FEM 2010).

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Copyright information

© Adis Data Information BV 2011

Authors and Affiliations

  • Mariana Castells
    • 1
  • Dean D. Metcalfe
    • 2
  • Luis Escribano
    • 3
  1. 1.Department of Medicine, Division of Rheumatology, Immunology, and Allergy, Brigham & Women’s HospitalHarvard Medical SchoolBostonUSA
  2. 2.Laboratory of Allergic DiseasesNational Institute of Allergy and Infectious Diseases/National Institutes of Health (NIAID/NIH)BethesdaUSA
  3. 3.Mastocytosis Institute of Castilla La ManchaHospital Virgen del ValleToledoSpain

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