Pediatric Treatment-Resistant Nonbacterial Osteomyelitis of the Mandible Associated with SAPHO Syndrome

SAPHO syndrome is a chronic disease of unknown etiology, which is characterized by synovitis, acne, pustulosis, hyperostosis, and osteitis, however, pediatric osteomyelitis of the jawbone is rarely reported. We present a report of pediatric nonbacterial osteomyelitis of the mandible associated with SAPHO syndrome. A seven-year-old girl presented complaining of pain in, and swelling of, the right retromolar region. Antibiotics had no effect and her past history included linea alba, strabismus, Palmoplantar Pustulosis (PPP) and old fractures of the Thoracic Vertebrae (Th7-9). Together with histological findings, she was diagnosed as suffering from SAPHO syndrome with mandibular osteomyelitis. NSAIDs (Naproxen), Corticosteroids (Dexamethasone), and Methotrexate (MTX) were effective for several months but the effects were transient. As the third line therapy, an antiTNFα agent (infliximab) was administered in addition to MTX. The mandibular symptoms have subsequently been under control for over a year. For treatment of mandible osteomyelitis with SAPHO syndrome, systemic immunosuppressive therapy should be considered, beside continuous oral management.