Abstract
Synovitis, acne, pustulosis, hyperostosis, and osteitis (SAPHO) syndrome is a rare disorder that classically involves the musculoskeletal system, i.e. bone, joint and skin. The exact pathogenesis of this syndrome is unknown. However, autoimmunity, infections, immune malfunction and genetic factors are attributed to its pathophysiology. Bone and joint involvements are the hallmark of SAPHO syndrome and not necessarily require cutaneous involvement at the time of diagnosis. X-ray of the affected joints could show osteitis with sclerosis. Anterior chest wall involvement particularly ''bullhead appearance'' seen on bone scan is a striking feature of the syndrome. Erythrocyte sedimentation rate (ESR) is usually elevated amongst the majority of patients. Diagnosis of SAPHO is always challenging and often delayed because of a multitude of symptoms. The mainstay of treatment is control of pain and inflammation with both non-steroidal anti-inflammatory drugs (NSAIDs) and rescue courses of systemic steroids. If failed to control symptoms with first-line agents and in those with severe disease, disease-modifying anti-inflammatory drugs (DMARDs) may be needed eventually. Despite a chronic inflammatory condition, it remains stable in the majority of cases. Here in this case report, we reiterate the importance of early recognition, timely diagnosis and prompt treatment initiation.
Highlights
It was in 1987 that a group of French authors coined the acronym SAPHO, a rare disorder of unknown aetiology [1]
Bone and joint involvements are the hallmark of SAPHO syndrome and not necessarily require cutaneous involvement at the time of diagnosis
Diagnostic criteria for SAPHO syndrome diagnosis Inclusion Bone-joint involvement associated with palmoplantar pustulosis (PPP) and psoriasis vulgaris Bone-joint involvement associated with severe acne Isolated sterile* hyperostosis/osteitis Chronic recurrent multifocal osteomyelitis Bone-joint involvement associated with chronic bowel diseases Exclusion Infectious osteitis Tumoural condition of the bone Non-inflammatory condensing lesions of the bone
Summary
It was in 1987 that a group of French authors coined the acronym SAPHO (synovitis, acne, pustulosis, hyperostosis, osteitis), a rare disorder of unknown aetiology [1]. Both X-ray (Figure 2b) and CT of the pelvis (Figure 2c) showed multiple areas of sclerosis of the left side of the sacrum These bony changes suggested the diagnosis of SAPHO syndrome. Her whole-body scan (Figure 3) showed classical “bullhead” appearance and sclerosis of sternoclavicular, manubriosternal and left sacroiliac joints, which matched the areas of sclerosis on CT. These findings were not suggestive of metastatic disease. On the basis, her clinical presentation, baseline blood investigations and supportive radiological evidence, diagnosis of SAPHO syndrome was made
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