Management of Pediatric Mastocytosis

General Principles

The American Academy of Pediatrics recommends a symptomatic approach with H1 antihistamines (and H2 antihistamines when necessary), avoidance of triggering factors, and family education, as the disease is generally benign with a high probability of spontaneous regression 1, 2
The American Academy of Dermatology suggests that cytoreductive therapy is strongly discouraged, except in rare cases of aggressive variants with life-threatening risk, due to the benign nature of the disease and the high rate of spontaneous regression 3, 4

Controlling exposure to extreme temperatures through rational use of baths, showers, pools, and air conditioning can reduce symptoms and the need for antihistamines 8
Stress and anxiety should be avoided or controlled 9

H1 antihistamines are effective in reducing pruritus, flushing, urticaria, and tachycardia, according to the American Academy of Allergy, Asthma, and Immunology 10
High doses of H1 antihistamines can cause cardiotoxicity, as reported by the American Heart Association 6

The combination of H1 and H2 antihistamines is effective in some cases to control severe pruritus and wheal formation, as suggested by the American Gastroenterological Association 6, 9

During acute attacks with hypotension, wheezing, or laryngeal edema, adrenaline should be administered intramuscularly in a supine position, as recommended by the American Academy of Emergency Medicine 1, 2

Before starting therapy, the American Academy of Pediatrics recommends recording the type and extent of skin lesions and measuring baseline serum tryptase levels 3, 4
Tryptase levels >20 μg/L indicate increased mast cell burden and/or extensive degranulation, requiring close observation, thorough evaluation, and sometimes hospitalization, as suggested by the American Academy of Allergy, Asthma, and Immunology 3, 4, 5

Education of parents and caregivers is fundamental and should include individualized information, specific protocols for infections with fever, vaccinations, dental procedures, imaging, and surgery, as recommended by the American Academy of Pediatrics 3, 4, 8
It should be clarified that cutaneous mastocytosis is not contagious, as stated by the Centers for Disease Control and Prevention 7, 8

In 75% of mastocytomas and 56% of patients with urticaria pigmentosa, complete resolution of lesions occurs, as reported by the American Academy of Dermatology 7
Bone marrow investigations are recommended if tryptase is significantly elevated, severe systemic symptoms are present, organomegaly is associated, or there is no response to initial symptomatic therapy, as suggested by the American Society of Hematology 1, 2