SAPHO syndrome with mandibular manifestation.

To cite: Baba A, Ojiri H, Takahashi S, et al. BMJ Case Rep Published online: [please include Day Month Year] doi:10.1136/bcr-2015213401 DESCRIPTIONF A 47-year-old man was referred to our hospital due to symptoms of trismus and exacerbation of palmoplantar pustulosis. He had been presenting, for 20 years, with refractory and recurrent mandibular osteomyelitis accompanied by palmoplantar pustulosis and anterior chest pain—symptomatic treatment and surgery had been given at each presentation. When the mandibular lesion got worse this time, he also had anterior chest pain. He had experienced similar episodes before this event. MRI (STIR: short tau inversion recovery) showed high signal intensity in the right ramus of the mandible, with perilesional soft tissue swelling (figure 1). These findings indicated active mandibular osteomyelitis. SAPHO syndrome was suggested by the clinical course and history. Bone scintigraphy using 99mTc-methylenediphosphonate (MDP) was performed and increased tracer uptake was seen in the right ramus of the mandible, greater wing of sphenoid bone, sternum and the sternocostoclavicular joints (figure 2). These characteristic clinical and radiological findings were compatible with the diagnosis of SAPHO syndrome involving the mandibular region. The term SAPHO syndrome was coined in 1987 to describe an association of characteristic bone, joint and skin lesions. ‘SAPHO’ is an acronym for synovitis, acne, palmoplantar pustulosis, hyperostosis and osteitis. Its aetiology remains unknown. 2 The sternocostoclavicular, sternoclavicular, costoclavicular and manubriosternal joints are affected. Bone scintigraphy using 99mTc-MDP is known to be important for diagnosis of this disease. SAPHO syndrome in the mandibular region is clinically rare and has been reported to be 10% of this entity. SAPHO syndrome in the mandibular region is seen as recurrent mandibular osteomyelitis, mainly involving the body of the mandible. In patients with clinical and radiological evidence of SAPHO syndrome, when recurrent mandibular Figure 1 MRI (short tau inversion recovery) showed high signal intensity in the right ramus of the mandible, with perilesional soft tissue swelling.