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Paediatric mastocytosis: a systematic review of 1747 cases

C Méni1, J Bruneau, S Georgin-Lavialle

  • 1Service de Dermatologie de l'Adulte et de l'Enfant, Faculté de Médecine et AP-HP, Hôpital Necker-Enfants Malades, Centre de Référence des Mastocytoses, CEREMAST, Université Paris Descartes, Paris Sorbonne Cité, 149 Rue de Sèvres, 75743, Paris Cedex 15, France.

The British Journal of Dermatology
|February 10, 2015
PubMed

Insights

Paediatric mastocytosis, previously thought benign, shows unpredictable progression. This review of 1747 cases reveals common presentations and variable outcomes, including rare fatalities, highlighting the need for careful monitoring.

Area of Science:

  • Dermatology
  • Pediatrics
  • Hematology

Background:

  • Paediatric mastocytosis was historically viewed as a benign condition with spontaneous regression.
  • The unpredictable clinical course and evolution of paediatric mastocytosis necessitate further characterization.

Purpose of the Study:

  • To elucidate the clinical characteristics, presentation patterns, and natural history of paediatric mastocytosis.
  • To provide a comprehensive overview of paediatric mastocytosis based on a large cohort of published cases.

Main Methods:

  • A systematic literature review was conducted, analyzing 1747 cases of paediatric mastocytosis.
  • Data were extracted from studies published between 1950 and April 2014, focusing on demographics, clinical presentation, genetic mutations, and outcomes.

Main Results:

  • Lesions typically appeared before age 2 (90%), with urticaria pigmentosa being the most common form (75%).
  • The KIT D816V mutation was identified in 34% of tested patients. Complete or partial regression occurred in 67% of cases, with stabilization in 27%.
  • A concerning 2.9% of patients experienced a fatal outcome.

Conclusions:

  • Paediatric mastocytosis exhibits diverse presentations and variable prognoses, challenging the notion of universal benignity.
  • While many cases regress, the potential for severe outcomes underscores the importance of vigilant clinical observation and management.
  • Understanding the genetic landscape, such as KIT D816V mutations, may offer insights into disease behavior and therapeutic targets.

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