A Case of Diffuse Sclerotic Osteomyelitis of the Mandible in SAPHO Syndrome Successfully Treated With an Oral Bisphosphonate Administration

Ikeda, Atsushi

A Case of Diffuse Sclerotic Osteomyelitis of the Mandible in SAPHO Syndrome Successfully Treated With an Oral Bisphosphonate Administration

Atsushi Ikeda DDS, Department of Oral and Maxillofacial Reconstructive Surgery, Okayama University, Okayama city, Japan

Eiki Yamachika DDS, PhD, Department of Oral and Maxillofacial Reconstructive Surgery, Okayama University, Okayama city, Japan

Tatsushi Matsumura DDS, PhD, Department of Oral and Maxillofacial Reconstructive Surgery, Okayama University, Okayama city, Japan

Masahide Mizutani DDS, PhD, Department of Oral and Maxillofacial Reconstructive Surgery, Okayama University, Okayama city, Japan

Aya Maeda DDS, PhD, Department of Oral and Maxillofacial Reconstructive Surgery, Okayama University, Okayama city, Japan

Noriko Kishimoto DDS, Department of Oral and Maxillofacial Reconstructive Surgery, Okayama University, Okayama University, Japan

Yuki Arimura DDS, Department of Oral and Maxillofacial Reconstructive Surgery, Okayama University, Okayama city, Japan

Yuuya Yamamoto D.D.S., Department of Oral and Maxillofacial Reconstructive Surgery, Okayama University, Okayama city, Japan

Syouhei Tamura D.D.S., Department of Oral and Maxillofacial Reconstructive Surgery, Okayama University, Okayama city, Japan

Ayumi Uemura D.D.S., Department of Oral and Maxillofacial Reconstructive Surgery, Okayama University, Okayama city, Japan

Seiji Iida DDS, PhD, Department of Oral and Maxillofacial Reconstructive Surgery, Okayama University, Okayama city, Japan

Introduction

SAPHO syndrome, which was proposed in 1987 by Chamot et al¹, is an acronym describing a heterogeneous symptom complex of synovitis, acne, pustulosis, hyperostosis, and osteitis(sterile osteomyelitis). We report the case of Diffuse Sclerotic Osteomyelitis of the mandible (DSOM) in SAPHO syndrome that was successfully treated with an oral bisphosphonate.

Case report

The patient was 78-year old woman who had a chief complaint of pain in the left mandible area. The patient underwent a Computed Tomography (CT). CT showed osteosclerosis lesions in the left mandible and sternocostal area. And an ununiform bone marrow was detected in the left mandible.

Technetium-99m bone scan(^99mTc) showed increased uptake in the mandible, the sternum and the costa. A bone biopsy specimen showed little infiltration of inflammatory cells and no evidence of malignancy. Bacterial cultures remained negative. In addition, because the patient had scale in the soles of feet for palmoplantar pustulosis, the patient was diagnosed as SAPHO syndrome. The treatment with non-steroidal anti-inflammatory drugs (NSAIDs) was started but failed to control symptoms, then therapy

with an oral administration of bisphosphonate (Alendoronate: 35mg/week) was started. Symptoms disappeared after eight months of oral administration and administration of oral bisphosphonate was finished at that time. In addition, ^99mTc showed a decreased uptake in the left part of the mandible. The patient has been obtained good progress without a recurrence at the present and passed for 65 months on complete remission.

Recenly treatment with bisphosphonate of SAPHO patients who is refractory to NSAIDs has been reported². This report showed that administration of oral bisphosphonate had a dramatic and longstanding effect for a patient of DSOM associated with SAPHO syndrome. Further trials of oral bisphosphonate in patients with DSOM and even in patients with SAPHO syndrome are needed to assess its effectiveness.

References

Chamot AM, Benhamou CL, Kahn MF, et al: [Acne-pustulosis-hyperotosis-osteitis syndrome.
Results of a national survey. 85 Cases]. Rev Rhum Mal Osteoartic 54: 187, 1987
Hayem G, Bouchaud-Chabot A, Benal K, et al: SAPHO syndrome: A long-term follow-up study of 120 cases. Semin Arthritis Rheum 29:159, 1999