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SAPHO Syndrome PDF Print E-mail
Frank J. Frassica, MD
Chairman, Department of Orthopedic Surgery
Robert A. Robinson Professor of Orthopedic Surgery
The Johns Hopkins Hospital;
Professor of Oncology
Sidney Kimmel Comprehensive Cancer Center
Baltimore, Maryland


Pretest


I. SAPHO Syndrome

  1. SAPHO syndrome is a term that describes the association between inflammation of bones and joints, and acne or pustulosis:
    1. Synovitis
    2. Acne
    3. Pustulosis
    4. Hyperostosis
    5. Osteitis

II. Diagnostic Criteria

  1. Characteristic pustules or acne in the presence of sterile synovitis, hyperostosis, or osteitis
  2. Sterile synovitis, hyperostosis, or osteitis involving the axial or peripheral skeleton (anterior chest wall, vertebral bodies, sacroiliac joints) with or without characteristic skin lesions

III. Bone and Joint Manifestations

  1. Joints
    1. Synovitis
      1. Sternoclavicular
      2. Costoclavicular
      3. Manubriosternal
    2. Findings
      1. Joint space loss
      2. Erosions
  2. Bone
    1. Hyperostosis - "excessive osteogenesis" - may narrow or obliterate the medullary cavity and enlarge the surface of the bone
      1. Endosteal surface
      2. Medullary cavity
      3. Periosteal surface
    2. Osteitis is inflammation of the bone; clinically the bone is painful and tender

IV. Skin

  1. Acne
  2. Pustulosis
    1. Pustular psoriasis
    2. Palmoplantar pustulosis

V. Laboratory

  1. Variable elevation of serum markers
  2. Biopsy sometimes shows Propionibacterium acnes

VI. Treatment

  1. Nonsteroidal anti-inflammatory agents
  2. Often, no response with antibiotics
  3. Symptomatic

 


Bibliography

Boutin RD, Resnick D. The SAPHO syndrome: An evolving concept for unifying several disorders of bone and skin. Amer J Roentgen. 1998; 170:585-591.

 

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