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SAPHO (synovitis, acne, pustulosis, hyperostosis, and osteitis) syndrome

Authoring team

  • is a rare disorder characterized by cutaneous and osteoarticular manifestations that include acne, palmoplantar pustulosis, psoriasis, arthralgia, chest pain, and enthesitis
    • skin manifestations may be evident at the time of presentation with osteoarticular symptoms, but may have occurred years earlier or may develop later

    • laboratory evaluation is nonspecific, and magnetic resonance imaging is the modality of choice for evaluating osteoarticular manifestations

    • bone biopsies are performed to rule out malignancy and infection and primarily exhibit a sterile neutrophilic inflammatory infiltrate early in the disease; late-stage findings include enlarged sclerotic trabeculae with increased osteocytes and marrow fibrosis

  • generally considered to be a rare condition, possibly due to being underdiagnosed - a prevalence estimate of <1/10,000 is frequently mentioned

  • SAPHO syndrome may present at any age but is most commonly seen in children and young to middle-aged adults - there may be a female preponderance

  • bony involvement is most notable at the sternoclavicular joint
    • anterior chest wall is affected in 63% to 73% of patients with SAPHO syndrome
      • in early disease, radiography is often normal
      • early lesions, when present, are osteolytic and may be accompanied by endosteal or periosteal reaction. As the disease progresses, the lesions become sclerotic
      • pathognomonic bull's head sign can be seen with whole-body scintigraphy and refers to increased radiotracer uptake in the sternoclavicular joints and sternum-the manubrium represents the skull of the bull, and the sternoclavicular joints are the horns (1)
        • unlike plain radiography, whole-body scintigraphy and whole-body magnetic resonance imaging may identify subclinical disease, although magnetic resonance imaging is preferred because it has similar sensitivity without the radiation exposure
        • bone biopsy is also necessary to rule out malignancy and osteomyelitis (1)

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