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[Marshall syndrome].

Pascal Pillet1, Marion Bailhache1

  • 1Département de pédiatrie, hôpital des Enfants-groupe hospitalier Pellegrin, CHU Bordeaux, Bordeaux, France.

La Revue Du Praticien
|February 14, 2024
PubMed
Summary
This summary is machine-generated.

Marshall syndrome, also known as periodic fever, aphthous stomatitis, pharyngitis, and adenitis (PFAPA) syndrome, is a common autoinflammatory disease in children. Correct diagnosis and management, including corticosteroid therapy, improve quality of life and avoid unnecessary antibiotics.

Keywords:
Familial Mediterranean FeverHereditary Autoinflammatory Diseases

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Area of Science:

  • Pediatric Rheumatology
  • Autoinflammatory Diseases
  • Clinical Immunology

Context:

  • Marshall syndrome, or PFAPA syndrome, is the most common autoinflammatory disease.
  • It typically manifests in children aged 1-5 years.
  • Characterized by recurrent episodes of fever, aphthous stomatitis, pharyngitis, and adenitis.

Purpose:

  • To outline the diagnostic criteria for Marshall syndrome (PFAPA).
  • To discuss current management strategies, including corticosteroid therapy and surgical options.
  • To emphasize the importance of accurate diagnosis for appropriate patient care.

Summary:

  • Marshall syndrome (PFAPA) presents with periodic fever, aphthous stomatitis, pharyngitis, and adenitis.
  • Diagnosis relies on specific clinical criteria, differentiating it from infectious causes.
  • While febrile seizures can impact quality of life, the condition generally has a benign prognosis and often resolves spontaneously.

Impact:

  • Accurate diagnosis of PFAPA syndrome prevents unnecessary antibiotic use.
  • Effective management, primarily with corticosteroids, alleviates acute symptoms.
  • Understanding the generally benign and self-limiting nature of PFAPA reassures families and guides appropriate care.