IS Case 289: Clavicular hyperostosis in SAPHO syndrome
Imaging Sciences URMC 2009
Publication Date: 2009-08-05
Clavicular hyperostosis in SAPHO syndrome
This case demonstrates clavicular hyperostosis which can have a number of etiologies, including condensing osteitis of the clavicle, Caffey’s disease, infectious osteomyelitis/spondylitis, osteosarcoma, Ewing sarcoma, Paget's disease, aseptic necrosis of the clavicle, and SAPHO (synovitis, acne, pustulosis, hyperostosis, osteitis) syndrome.
SAPHO syndrome includes a variety of musculoskeletal disorders with the essential component of an inflammatory osteitis, which may or may not be associated with dermatological components usually with negative bacterial cultures. The main target area is the anterior chest wall, with lesser involvement of the spine and peripheral skeleton.
The hyperostosis and osteitis that are seen are manifestations of a chronic inflammatory reaction involving both the cortex and the medullary canal with associated endosteal and periosteal thickening. This results in diffuse cortical thickening and narrowing of the medullary canal with or without areas of osteolysis. Inflammatory enthesopathy is also a feature.
The diagnosis was made in this case based on clinical context, radiologic findings, negative bacterial cultures, and a non-infectious inflammatory pathology of multiple biopsies taken from the site of clavicular hyperostosis.
The prognosis is a chronic course with unpredictable exacerbations and remissions.
Treatment usually consists of non-steroidal anti-inflammatory drugs which are usually effective for the relief of pain.
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