© 2002 Archives of Disease in Childhood
LEADING ARTICLE
Immunology
Paediatric mastocytosis
NIAID/NIH, Bethesda, MD, USA
Correspondence to:
Correspondence to:
Dr M C Carter, NIH/NIAID/LAD, Building 10, Room 11-C206, 10 Center Dr. MSC 1881, Bethesda, MD 20892-1881, USA;
mcarter@niaid.nih.gov
An unusual disease in infants and children
Keywords: mastocytosis; mast cell
Mastocytosis in infants and children is an unusual disease characterised by an excess of mast cells in body tissues. The phenotypic expression of the disease is dependent on the pattern of localisation of the mast cells to specific organs and the release of mast cell mediators. The skin is the most common organ involved in children and may be the only manifestation of the disease. Mastocytosis can present from birth1 to adulthood, with adult onset disease generally being more severe. The cutaneous form was recognised over 100 years ago, but the term mastocytosis, based on the offending cell, was initiated in 1936 by Sezary and Chauvillon.2
Mast cells are most abundant in connective tissues, with a predilection for peripheral nerves, and blood and lymphatic vessels. It is at these sites, under the influence of cytokines (interleukins 3, 4, 5, 6, 9, 10, and 15)3–9 and the principal mast cell
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Arch. Dis. Child. 2002 86: 315.
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