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Pediatric mastocytosis
1Division of Allergy and Clinical Immunology, Children's Hospital Medical Center, Elland and Bethesda Avenue, Cincinnati, Ohio, USA.
The Journal of Investigative Dermatology
|March 1, 1991
Summary
Pediatric mastocytosis often presents with skin issues like urticaria pigmentosa, differing from adult cases. While internal organ involvement is less common in children, some may see symptoms resolve by adolescence.
Area of Science:
- Pediatric Hematology
- Dermatology
- Allergy and Immunology
Background:
- Mastocytosis is a rare disorder characterized by the abnormal accumulation of mast cells.
- Pediatric-onset mastocytosis accounts for a significant portion of all cases, with onset typically occurring in early childhood.
Purpose of the Study:
- To describe the distinct characteristics of pediatric-onset mastocytosis.
- To outline the typical presentations, potential organ involvement, and treatment strategies for children with mastocytosis.
Main Methods:
- Review of clinical presentations and disease progression in pediatric patients.
- Analysis of common symptoms, affected organs, and therapeutic interventions.
Main Results:
- Pediatric mastocytosis commonly manifests as cutaneous lesions such as solitary mastocytoma or urticaria pigmentosa.
- Internal organ involvement, including bone marrow and gastrointestinal tract, is less frequent in children compared to adults.
- Elevated plasma histamine levels can be observed in pediatric cases.
Conclusions:
- Pediatric mastocytosis presents differently from adult-onset forms, primarily with skin manifestations.
- Treatment often involves antihistamines for symptom management.
- Prognosis varies, with a notable percentage of children experiencing symptom resolution by adolescence.