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Pediatric mastocytosis
1Division of Allergy and Immunology, Children's Hospital Medical Center, Cincinnati, Ohio.
Annals of Allergy
|September 1, 1994
Summary
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.