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Pediatric mastocytosis
1Division of Allergy and Clinical Immunology, Children's Hospital Medical Center, Cincinnati, Ohio.
Insights
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.
Abstract:
The onset of mastocytosis occurs between birth and 2 years of age in approximately 55% of all cases; an additional 10% develop the disease before the age of 15 years. Mastocytosis in these age groups differs in many respects from mastocytosis that has its onset in adulthood. The typical presentation of pediatric-onset mastocytosis consists of cutaneous manifestations: either a solitary mastocytoma, urticaria pigmentosa, or, less commonly, diffuse cutaneous mastocytosis. Particularly in infants, bullous eruptions may occur. Mastocytosis in infants and children may involve internal organs, including the bone marrow and the gastrointestinal tract, although such manifestations appear to be less common in children than in adults. Plasma histamine levels may be elevated in pediatric-onset mastocytosis. Treatment usually involves the use of H1 and H2 antihistamines to control itching and to control the hypersecretion of gastric acid that may occur. The prognosis for children with mast cell disease is variable; approximately half of the children with urticaria pigmentosa may experience resolution of lesions and symptoms by adolescence.