SAPHO Syndrome

Abstract

Four women with pustulosis palmoplantaris (PPP) and associated sternocostoclavicular hyperostosis are described. The connection between the two diseases is not coincidental: it is encountered in 9.4% of PPP patients, and was reported in the French literature as part of the SAPHO (synovitis acne pustulosis hyperostosis osteomyelitis) syndrome. This syndrome is linked to the spondyloarthropathies, having an increased prevalence of HLA B27 and occurrence of sacroiliitis. None of our patients had HLA B27, but HLA A26 was found in the 3 patients examined. No conclusion can be drawn from this finding, however, since this antigen is found in 21.7% of Ashkenazi Jews. Recognition of this syndrome by dermatologists will improve diagnosis and treatment.

Pustulotic Arthro-Osteitis (Sonozaki Syndrome): A Case Report and Review of Literature

“…Many clinical features of SAPHO syndrome are similar to PAO. Differential diagnosis is difficult, but PAO is distinguished from SAPHO syndrome by the absence of acne and hyperostosis [7,8]. In our patient, there was no anterior thoracic joint hyperostosis or no acne lesions characteristic of SAPHO syndrome.…”

“…PPP is a sterile lesion without any association with Propionibacterium acnes. The characteristic skeletal features are hyperostosis at the anterior thoracic joints and less often at the pelvic bones, spine, and peripheral joints [7,13]. The characteristic "bull' s head" sign of SAPHO syndrome by bone scintigraphy (MDP Tc-99m) can also be seen in PAO.…”

“…Another disease for which differential diagnosis should be considered is the synovitis, acne, pustulosis, hyperostosis, and osteitis (SAPHO) syndrome. PAO is distinguished from SAPHO syndrome by the absence of hyperostosis and the difference in skin manifestations [7,8].…”

SAPHO syndrome with ankylosis of the temporomandibular joint

“…Chamot and Kahn 8 suggested an increased frequency of HLA-B27 (30%) in patients with hyperostosis or involvement of the anterior thorax. According to these spots, radiological examination reveals signs of osteolysis or hyperostosis 4,26 . According to these spots, radiological examination reveals signs of osteolysis or hyperostosis 4,26 .…”

SAPHO: The Impossible Acronym

“…The excellent paper of Brandsen et al [1] dealing with this French-born new concept, the SAPHO [2], deserves some comments. This acronym -Syndrome -Acne -Pus tulosis -Hyperostosis -Osteitis -emphasizes the occur rence simultaneous or not of cutaneous and osteoarticular disorders and the relationship of chronic, aseptic, neutro philic, skin-limited, and painful rheumatic diseases.…”