Mastocytosis, a heterogeneous mastcell disease, include three different entities:
cutaneous mastocytosis,
systemic mastocytosis (SM) and
mast-cell sarcoma.
Tryptase levels can differentiate
cutaneous mastocytosis from SM. In
mastocytosis, quick onset
drug hypersensitivity reactions (DHRs) that are facilitated by mastcell mediators, are investigated in adults. Due to the limited number of children with mastcell disease and increased serum
tryptase levels, the role of drugs in this age group is less studied. In this review, we critically assessed relevant papers related with immediate DHRs in children with
mastocytosis and discuss practical issues of the management. In childhood
mastocytosis,
anaphylaxis is frequently idiopathic, and elevated level of basal
tryptase, and high burden of disease may increase the risk. Among drugs,
antibiotics,
NSAIDs and
opioids can potentially induce
anaphylaxis, anyway avoidance should be recommended only in case of previous reactions. Moreover, vaccinations are not contraindicated in patients with
mastocytosis. The risk of severe systemic reactions after drugs intake seems to be extremely low and in general lower in children than in adults. Anyway, studies on this topic especially focusing on children, are missing to state final recommendations.