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Pediatric mastocytosis

B V Kettelhut¹, D D Metcalfe

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.

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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.

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