SAPHO syndrome of the right temporomandibular bone and joint in a 25-year-old man

Abstract

SAPHO syndrome is an association of specific bone, joint, and skin lesions. The acronym SAPHO refers to synovitis, acne, palmoplantar pustulosis, hyperostosis, and osteitis. However, not all these clinical manifestations need to be present to establish the diagnosis. It is a rare disease in which genetic predisposition or an autoimmune response to a microorganism in the skin mimicking a normal bone or joint molecular structure has been proposed. We present a 25-year-old man who presented with 2-year history of difficulty in opening the mouth with associated pain in the right jaw. He also developed progressive right hearing impairment and multiple acne-like rashes on his face 10 months prior to presentation. Examination of the right jaw revealed a tender firm immobile swelling in the region of the temporomandibular joint which was slightly warm to touch. CECT revealed cortical bone erosions of the right temporal squama and condyle with diffuse endosteal sclerosis and hyperostosis of the mandibular condyle and ramus. The temporal squama was partially resorbed with areas of bone remodeling. The right temporomandibular joint was also involved in the sclerotic changes with marked narrowing of its joint space. Based on the clinical and radiological features, a diagnosis of SAPHO syndrome was established. It is essential to make such diagnosis in order to avoid unnecessary and futile treatment.