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Pediatric mastocytosis
1Division of Allergy and Clinical Immunology, Children's Hospital Medical Center, Cincinnati, Ohio.
The Journal of Investigative Dermatology
|March 1, 1991
Summary
Pediatric mastocytosis, often appearing in infancy, primarily affects the skin but can involve internal organs. While treatments exist, about half of children with urticaria pigmentosa 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 presents distinct clinical features compared to adult-onset forms.
- Early onset (birth to 15 years) accounts for a significant majority of mastocytosis cases.
Purpose of the Study:
- To describe the clinical presentation and characteristics of pediatric-onset mastocytosis.
- To differentiate pediatric mastocytosis from adult-onset disease.
- To outline typical manifestations, potential organ involvement, and treatment approaches in children.
Main Methods:
- Review of clinical data and literature on pediatric mastocytosis.
- Analysis of typical presentations including cutaneous and systemic involvement.
- Discussion of diagnostic indicators such as elevated plasma histamine levels.
Main Results:
- Pediatric mastocytosis typically manifests with cutaneous lesions like solitary mastocytoma, urticaria pigmentosa, or diffuse cutaneous mastocytosis.
- Bullous eruptions are noted in infants.
- Internal organ involvement (bone marrow, GI tract) is less common than in adults, but elevated plasma histamine levels can occur.
Conclusions:
- Pediatric mastocytosis has a unique presentation, predominantly cutaneous.
- While systemic involvement is less frequent, it can occur.
- Prognosis varies, with potential for symptom resolution by adolescence in some cases, particularly urticaria pigmentosa.