Anaphylaxis and epinephrine autoinjector use in pediatric patients with cutaneous mastocytosis

Abstract

Background/Objectives Mastocytosis is a complex disorder presenting with a broad clinical spectrum. In this study, we aimed to evaluate the frequency of systemic symptoms, necessity of the usage of epinephrine autoinjectors (EAI), and factors affecting the use of EAI among pediatric patients with cutaneous mastocytosis (CM). Methods The study population was composed of 53 patients with CM. The clinical data were collected from the medical files. A questionnaire about the patient's anaphylaxis experiences and treatment attitudes toward EAI was performed. Results Thirty‐three of 53 patients were male (62.3%), and the median age of the study participants was 80 months. Anaphylaxis was reported in two patients (3.7%). One of the patients had drug‐induced anaphylaxis, and the other had venom‐induced anaphylaxis. Three patients (5.6%) reported a personal history of EAI use due to systemic symptoms. Patients with higher serum tryptase level and cases of familial mastocytosis had more systemic symptoms (P = .012 and P = .010, respectively). The patient rate of compliance with EAI for prescription filling and carrying the EAI was 88.7% and 79.2% respectively. 41.5% of parents were hesitant to use EAI when necessary, although they were trained for the use of it by medical staff. Conclusions The rate of anaphylaxis in patients with CM in the current study wassignificant and justifies prescribing EAI. Detailed education and counseling on EAI usage are needed to reduce parental hesitancy.

Cutaneous mastocytosis in childhood

“…In individual cases of severe CM, omalizumab has also been reported to have a positive effect in children [ 34 ]. If risk factors for anaphylactic reactions have been identified, the family must be provided with an emergency kit including an adrenaline auto-injector [ 35 ]. In these cases, anaphylaxis training is also indicated .…”

Updates in diagnosis and management of paediatric mastocytosis

“…Increased serum tryptase levels are associated with systemic symptoms in paediatric mastocytosis [26]. Triggers include mechanical or physical stimuli, infections, teething and allergens, particularly hymenoptera venom stings [27 ▪ ]. Major drugs that can trigger mast cell activation include analgesics such as aspirin and other NSAIDs, opioids, muscle relaxants, cough suppressants, contrast media and quinolone antibiotics.…”

“…Premedication with H1/H2 antihistamines 1–2 h prior to anaesthesia should be considered. Epinephrine autoinjectors should be prescribed to children with extensive skin lesions, highly elevated tryptase and/or a history of systemic reactions [27 ▪ ]. In a cohort of 53 patients with paediatric cutaneous mastocytosis, two experienced anaphylaxis [27 ▪ ].…”

“…Epinephrine autoinjectors should be prescribed to children with extensive skin lesions, highly elevated tryptase and/or a history of systemic reactions [27 ▪ ]. In a cohort of 53 patients with paediatric cutaneous mastocytosis, two experienced anaphylaxis [27 ▪ ]. Parents were hesitant to use epinephrine auto-injectors despite their indication [27 ▪ ].…”

“…In a cohort of 53 patients with paediatric cutaneous mastocytosis, two experienced anaphylaxis [27 ▪ ]. Parents were hesitant to use epinephrine auto-injectors despite their indication [27 ▪ ].…”