SAPHO syndrome

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Wide spectrum of aseptic neutrophilic dermatoses in association with aseptic osteoarticular lesions that have distinctive histologic and radiographic features.
Important to recognize to avoid unnecessary invasive procedures.
 
Etiology
  • Associated with:
    • Psoriasis
    • Linear IgA Bullous Dermatosis (LABD)
    • Other neutrophilic dermatosis
    • Borreliosis
    • Inflammatory bowel disorder
 
Clinical presentation “Synivitis, acne, pustulosis, hyperostosis, osteitis”
  • Intermittent with periodic exacerbations
  • Osteoarticular lesions
    • Chronic recurrent multifocal “osteomyelitis”
    • Affects bone of anterior chest wall (sternum, clavicles, ribs) and axial skeleton (pelvis and spine)
  • Skin - pustular dermatosis
    • Acne
    • Pustulosis
    • No correlation with bone activity
notion image
 
Labs
  • Elevated inflammatory markers, leukocytosis
 
Diagnosis
  • Can be diagnosed with MRI and bone scintigraphy
 
Treatment
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Isotretinoin for acne may induce flare
  • NSAIDs
  • Intrarticular corticosteroids
  • Systemic corticosteroids
  • Bisphosphonates
  • Conventional immunossupressants
    • Methotrexate
    • Azathioprine
  • TNF inhibitors
  • IL-1 antagonists (anakinra)
  • IL-12/23 inhibitors
  • IL-23 & IL-17 inhibitors
  • JAK inhibitors
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