Introduction
Pediatric Mastocytosis is a rare disease characterized by the presence of too many mast cells in the skin, and
possibly other tissues. Mast cells are instrumental in mediating anaphylaxis, and children with mastocytosis
are at risk to develop both provoked and unprovoked episodes of anaphylaxis. Pediatric mastocytosis may
involve the bone marrow and the gastrointestinal tract, and symptoms in children can vary greatly from child to
child. Basic treatment includes the avoidance of known triggers, having injectable epinephrine available at all
times, H1 and H2 antihistamines to control itching and gastric acid hyper secretion, and a mast cell stabilizer.
IV steroids may be necessary to treat progressive, severe bullae in infants. Many children may not complain
of specific symptoms, may not be able to identify or localize a symptom, or may have every symptom while
others may have very few. |
