SAPHO syndrome — SCE Rheumatology MCQ

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EasySpondyloarthropathiesSAPHO syndromeSCE Rheumatology

A 42-year-old woman has a 6-month history of anterior chest-wall pain and a pustular rash affecting her palms and soles. She is afebrile and systemically well. Examination shows tender swelling over the sternoclavicular joints. Bone scintigraphy demonstrates increased tracer uptake involving the manubrium and both sternoclavicular joints. What is the most likely diagnosis?

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Correct answer: A — SAPHO syndrome

The correct answer is SAPHO syndrome: synovitis, acne, pustulosis, hyperostosis and osteitis. Palmoplantar pustulosis combined with chronic painful swelling and osteitis of the sternocostoclavicular region is highly characteristic. Uptake involving the manubrium and sternoclavicular joints may form the classic “bull's-head” scintigraphic pattern. Septic sternoclavicular arthritis usually presents more acutely and may cause fever or systemic illness; it does not explain the characteristic skin disease and bilateral imaging pattern. Psoriatic arthritis can involve the anterior chest wall but this combination is more specific for SAPHO. Tietze syndrome causes focal benign costochondral swelling without pustulosis or osteitis. Osteosarcoma would usually produce a focal destructive bone lesion and would not explain the palmoplantar eruption.

Reference: Rheumatic Disease Clinics of North America. Imaging for Synovitis, Acne, Pustulosis, Hyperostosis, and Osteitis (SAPHO) Syndrome. 2016;42(4):695–710. https://pubmed.ncbi.nlm.nih.gov/27742022/