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[Recurrent multifocal osteomyelitis in the absence of skin disease]
S Lamparter1, E List-Hellwig, O Basten
1Abteilung für Innere Medizin, Diakonie-Krankenhaus Wehrda, 35041 Marburg. Dr.S.Lamparter@dkh-wehrda.de
History And Admission Findings:
A 64-year-old man complained of pain and a symmetric swelling of the clavicles. There were no fever and chills. 27 years earlier, a resection of the mandible was necessary, due to osteomyelitis. In the past, he complained of recurrent episodes of pain in the sternum and in the thoracic spine. Skin disorders were not reported.
Investigations:
The leucocyte count was within the normal range, however, levels of c-reactive protein and the erythrocyte sedimentation rate were increased. Autoantibodies and blood cultures were negative. On X-ray, osteosclerotic and cystic lesions in both clavicles were found, consistent with osteomyelitis. A bone scintigraphy revealed increased radionuclide activity in the clavicles and the sternum. A bone biopsy sample from the clavicle revealed signs of osteitis with fibrosis together with CD 68 und CD 138 positive cells. These findings indicated the diagnosis of a SAPHO syndrome (Synovitis, Acne, Pustulosis, Hyperostosis, Osteitis) without skin disease.
Treatment And Course:
NSAIDs, bisphosphonates and azithromycin were administered , thereby sufficient pain relief was rapidly achieved.
Conclusion:
SAPHO syndrome is a rare disease complex including osteoarticular disorders which are frequently accompanied by skin disease. However, the diagnosis may be difficult due to a variable clinical manifestation and especially - like in our case - if skin disease is absent. Current treatment options are not evidence-based due to the rarity of the syndrome but include NSAIDs, bisphosphonates and glucocorticoids.
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