B V Kettelhut1, D D Metcalfe. 1. Division of Allergy and Immunology, Children's Hospital Medical Center, Cincinnati, Ohio.
Abstract
OBJECTIVE: The information presented will aid the practicing allergist in the recognition and management of pediatric-onset mastocytosis. DATA SOURCES: Index Medicus from 1985 to present with keywords: mastocytosis; pediatrics; cutaneous. Limited to English language and to human disease. STUDY SELECTION: Information relative to mastocytosis in the pediatric age group to adulthood was reviewed. RESULTS: Mastocytosis in children is an uncommon disease and is characterized by mast cell hyperplasia and release of mast cell mediators, particularly in the skin. It generally presents during the first 2 years of life. The most common manifestation is a solitary mastocytoma, with urticaria pigmentosa being the next most frequent manifestation. The most common initial presenting symptom of pediatric mastocytosis is pruritus. Complications of severe mastocytosis include formation of bullae and gastrointestinal bleeding attributed to high levels of circulating plasma histamine, which in turn stimulates gastric acid secretion. CONCLUSION: Treatment of pediatric mastocytosis is largely symptomatic. Prognosis seems to be somewhat related to the severity of the disease, with children with less extensive skin involvement tending to have the best chance to have resolution of the disease by adulthood.
OBJECTIVE: The information presented will aid the practicing allergist in the recognition and management of pediatric-onset mastocytosis. DATA SOURCES: Index Medicus from 1985 to present with keywords: mastocytosis; pediatrics; cutaneous. Limited to English language and to human disease. STUDY SELECTION: Information relative to mastocytosis in the pediatric age group to adulthood was reviewed. RESULTS:Mastocytosis in children is an uncommon disease and is characterized by mast cell hyperplasia and release of mast cell mediators, particularly in the skin. It generally presents during the first 2 years of life. The most common manifestation is a solitary mastocytoma, with urticaria pigmentosa being the next most frequent manifestation. The most common initial presenting symptom of pediatric mastocytosis is pruritus. Complications of severe mastocytosis include formation of bullae and gastrointestinal bleeding attributed to high levels of circulating plasma histamine, which in turn stimulates gastric acid secretion. CONCLUSION: Treatment of pediatric mastocytosis is largely symptomatic. Prognosis seems to be somewhat related to the severity of the disease, with children with less extensive skin involvement tending to have the best chance to have resolution of the disease by adulthood.