2015 Volume 53 Issue 3 Pages 427-434
SAPHO syndrome is a rare condition characterized by aseptic inflammatory lesions of the bone and skin. In the oral and maxillofacial regions, the mandible is most often affected. However, involvement is more typically seen in the anterior chest wall, axial skeleton, and extremities. Mandibular bone lesions in this syndrome are often indistinguishable from more common types of osteomyelitis, which are caused by severe dental infections. Therefore, dentists may have difficulty in promptly distinguishing this condition from others.
We treated a 7-year-old girl diagnosed with SAPHO syndrome who had noninfectious osteomyelitis of the mandible and palmoplantar pustulosis. Pain and swelling developed on the right side of the face at 2 months after dental treatment. The initial clinical and radiographic findings suggested infectious osteomyelitis resulting from severe dental caries or a periapical infection. Antibiotics were administered, but were ineffective and she was referred to our clinic. Our initial examination found no infectious lesions in the intraoral region. The patient had a history of pustules and erythema of the palms and soles, which occurred along with the mandibular osteomyelitic symptoms. She was referred to the pediatrics department. Computed tomography, positron emission tomography, and magnetic resonance imaging revealed an osteolytic lesion with a periosteal reaction that was limited to the right mandibular ramus. Biopsy specimens of bone and skin lesions showed inflammatory tissues that were compatible with osteomyelitis and palmoplantar pustulosis, respectively. Cultures did not identify any infectious agents or strains. Based on the clinical manifestations, radiologic imaging, and histopathological findings, we made a diagnosis of SAPHO syndrome with noninfectious osteomyelitis of the mandible and palmoplantar pustulosis. The patient was administered nonsteroidal anti-inflammatory drugs, which improved clinical symptoms in both bone and skin.
The prognosis of SAPHO syndrome is generally good and pharmacologic therapy is usually effective. However, as the disease progresses, patients often suffer from deformities or growth defects with hyperostosis of the affected bones. SAPHO syndrome should be considered when pediatric dentists encounter children with jawbone inflammation with an unknown etiology.
