A 41-year-old Caucasian female patient presented with diffuse pustulous skin lesions and polyarthralgias. The patient had no other relevant past medical history. The histological study of the skin lesions revealed a polymorphonuclear infiltrate in the dermis. Other laboratory tests were unremarkable. 

CT AXIAL PELVIS

CT AXIAL CLAVICLE

CT CORONAL LOWER LUMBAR

Tc-99m methylene diphosphonate bone scintigraphy
What is the likely diagnosis?

ANSWER


SAPHO syndrome

The main findings in the CT study were sclerosis, irregularities, and hyperostosis of the manubrium and medial ends of both clavicles; asymmetric sacroiliitis (Fig. 2) and sclerosis and erosion of the superior-lateral corner of L4 vertebral body .
Bone scintingraphy revealed intense radiotracer uptake in both clavicle medial ends and sternoclavicular joints. There was also radiotracer uptake in the sacroiliac joints (more pronounced on the right).

SAPHO is an acronym that refers to an uncommon syndrome, composed of the combination of synovitis, acne, pustulosis, hyperostosis and osteitis. It affects more often young adults, with a female preponderance.
The causes are unknown, although some authors support its integration in seronegative spondyloarthtropaties which is supported by the increasead prevalence of the HLA B27 allele, occasional presence of sacroiliitis, inflammatory bowel disease and psoriasis.The most common dermatologic manifestations are palmoplantar pustulosis, severe acne, and psoriasis.
Characteristic radiographic findings include hyperostosis, which includes endosteal and periosteal proliferation and enthesopatic ossification. In association there are mixed areas of osteolysis. Adjacent joints show manifestations of arthritis, namely erosions and joint space narrowing. CT can depict these alterations in greater detail.


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14 comments

  1. Anonymous Says:
  2. sweet's syndrome

     
  3. Anonymous Says:
  4. Ankylosing spondylitis

     
  5. Anonymous Says:
  6. dr.house : SAPHO syndorme

     
  7. Anonymous Says:
  8. wegener granulomatosis

     
  9. Anonymous Says:
  10. ankspon likely tho age doent fit !! sweets syn shud also present wid fever !!

     
  11. Anonymous Says:
  12. dermatomiositis?

     
  13. Anonymous Says:
  14. ostheosarcoma

     
  15. Anonymous Says:
  16. system eritematous lupus?

     
  17. Anonymous Says:
  18. histiocytosis

     
  19. MosaadMD Says:
  20. celiac disease

     
  21. MosaadMD Says:
  22. celiac disease

     
  23. Anonymous Says:
  24. sarcoidosis

     
  25. Monica Says:
  26. SAPHO syndrome (synovitis, acne, pustulosis, hyperostosis, osteitis)that involves mainly the axial skeleton and sternal-clavicular joints

     
  27. JRG Says:
  28. SAPHO syndrom.

     

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