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Pediatric mastocytosis
1Division of Allergy and Immunology, Children's Hospital Medical Center, Cincinnati, Ohio.
Insights
Pediatric mastocytosis, a rare condition involving mast cell hyperplasia, often presents in infancy. Early recognition and symptomatic treatment are key, with prognosis linked to disease severity and skin involvement.
Area of Science:
- Pediatric Allergy and Immunology
- Dermatology
- Hematology
Background:
- Mastocytosis is an uncommon pediatric disorder characterized by mast cell hyperplasia.
- It involves the release of mast cell mediators, primarily affecting the skin.
- The condition typically manifests within the first two years of life.
Purpose of the Study:
- To provide allergists with guidance on recognizing and managing pediatric-onset mastocytosis.
- To review the presentation, symptoms, and complications of mastocytosis in children.
Main Methods:
- Literature review of Index Medicus from 1985 to present.
- Keywords used: mastocytosis, pediatrics, cutaneous.
- Included English-language studies on human disease.
Main Results:
- The most common presentations are solitary mastocytoma and urticaria pigmentosa.
- Pruritus is the most frequent initial symptom.
- Severe cases can lead to bullae and gastrointestinal bleeding due to elevated histamine levels.
Conclusions:
- Treatment for pediatric mastocytosis is primarily symptomatic.
- Prognosis correlates with disease severity; less extensive skin involvement offers a better outlook for resolution 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.