Table 4.

Major treatment options for symptom control in pediatric mastocytosis.

Systems and Symptoms	First-Line Therapy	Other Therapeutic Options
Skin: pruritus flushing, blistering	HR1-antagonists	HR2-antagonists Oral corticosteroids (short course) Topical corticosteroids (class 1–3, short cycles ± occlusion) Leukotriene antagonist Pimecrolimus cream Topical sodium cromolyn Excision (for mastocytoma) NB-UVB * or PUVA ** Local care (for blistering)
Gastrointestinal: diarrhea, abdominal cramping/pain, reflux, ulceration	HR2-antagonists	Proton pump inhibitors Oral sodium cromolyn Oral corticosteroids
Neuro/psychiatric: headache, poor concentration, cognitive impairment	HR1 and HR2-antagonists	Neuro/psychiatric treatment specific for the individual patient according to symptoms
Cardiovascular: presyncope, syncope, hypotension	HR1 and HR2-antagonists	Oral corticosteroids Epinephrine
Osteopenia/osteoporosis	Calcium, vitamin D3	Treatment specific for the individual patient according to the age and T-score
Anaphylaxis	Epinephrine	Acute anaphylaxis: HR1 and HR2-antagonists Oral corticosteroids Intravenous fluids Prevention of anaphylaxis: Epinephrine auto-injector HR1-antagonists Allergen specific immunotherapy (typically Hymenoptera venom) Omalizumab (for recurrent episodes)

* NB-UVB—narrow-band ultraviolet B; ** PUVA—psoralen plus ultraviolet A.