SAPHO syndrome

Epidemiology

• bimodal distribution: can manifest in childhood or as an aldult

Pathology

• inflammatory bone disease with or without associated skin disease

Clinical manifestations

• Synovitis
• Acne - commonly involving the face, neck & upper back
• Palmoplantar pustulosis
• Hyperostosis that affects anterior chest wall & sternoclavicular joints
• Osteitis

other

• sterile osteomyeltitis
• serositis or enthesitis
• hydradenitis supprativa
• pustular psoriasis

Diagnostic criteria

• no consensus
• bone or joint symptoms + palmoplantar pustulosis
• quadriceps enthesitis is a minor criterion

Radiology

• X-rays
  • anterior chest wall
    • hyperostosis
    • sclerosis
    • bone hypertrophy
    • sternoclavicular joint, often involved
• thoracic spine
  • spondylodiscitis
  • osteosclerosis
  • paravertebral ossifications
• pelvis: sacroiliac joint involvement
• long bones: distal femur, proximal tibia
• peripheral joints, arthritis in 92% of cases
• in children
  • metaphysis of long bones in the legs (tibia, femur, fibula)
  • clavicle
  • spine

Management

• bisphosphonates may be useful
• treatment as psoratic arthritis may be useful
• antibiotics in treatment of acne may be useful

More general terms

• syndrome