Systemic mastocytosis in a 5-year-old child: successful treatment with disodium cromoglycate.
Most clinical signs and symptoms of systemic mastocytosis (SM) are attributed to histamine release. We report here a 5-year-old male child with SM, who suffered from the age of 4 months from disseminated skin lesions, vomiting, diarrhoea, abdominal pain, flushing, tachycardia, hypotension, somnolence, and transient blindness, triggered by heat and egg ingestion. Oral disodium cromoglycate (DSCG) or placebo were started in a single blind trial at a dose of 100 mg/kg/day in four divided doses. The child was studied for 21 months during the administration of three courses of DSCG, each of 6 months' duration, interspersed with three 1-month courses of placebo. During treatment with DSCG all the systemic manifestations improved, and the histaminaemia decreased. During the placebo periods the symptoms, signs, and histaminaemia recurred.
D002675:Child, Preschool; D004205:Cromolyn Sodium / Q000627:therapeutic use*; D006801:Humans; D007073:Immunoglobulin E / Q000276:immunology; D008297:Male; D012882:Skin Tests; D014582:Urticaria Pigmentosa / Q000188:drug therapy*
D007073:Immunoglobulin E; D004205:Cromolyn Sodium