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[Pediatric cutaneous mastocytosis].

A Van Rymenam1, J P Sacré1, B Dezfoulian2

  • 1Service de Pédiatrie, CHU Liège, Belgique.

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|October 8, 2020
PubMed
Summary

Mastocytosis, rare diseases of mast cell accumulation, are mostly skin-related in children with good prognoses. Treatment focuses on preventing triggers and managing symptoms with antihistamines.

Keywords:
Darier’s signPediatricsTryptaseCutaneous mastocytosis

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Area of Science:

  • Dermatology
  • Hematology
  • Pediatrics

Background:

  • Mastocytosis encompasses rare diseases defined by mast cell accumulation in various organs.
  • These conditions are categorized into systemic (10%) and cutaneous (90%) forms.
  • Cutaneous mastocytosis predominantly affects pediatric populations and typically resolves favorably over time.

Observation:

  • Diagnostic criteria for cutaneous mastocytosis, in the absence of systemic signs, include Darier's sign and skin biopsy findings.
  • Darier's sign is a key diagnostic indicator.
  • Skin histology confirms the characteristic mast cell infiltration.

Findings:

  • Cutaneous mastocytosis is the predominant form, accounting for 90% of cases.
  • Pediatric patients with cutaneous mastocytosis generally experience a favorable prognosis.
  • Systemic mastocytosis represents a smaller subset of cases (10%).

Implications:

  • Early diagnosis of cutaneous mastocytosis is crucial for appropriate management.
  • Preventive strategies, such as avoiding degranulation triggers, are essential.
  • Symptomatic treatment, primarily using antihistamine agents, helps manage patient discomfort.