SAPHO syndrome


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SAPHO syndrome

Synovitis Acne Palmoplantar Pustulosis Hyperostosis Osteitis. A rare association of severe acne and arthralgia/arthritis, which is more common in men.
 
Clinical findings
The acne affects the face, upper back and trunk, and is accompanied by fever and weight loss. The pain usually affects the hips, knees, shoulders, and occasionally other sites. The arthritis usually affects the lower limbs; it may be acute and polyarticular.
 
Lab
Increased erythrocyte sedimentation rate (ESR), increased leukocytes, decreased serum complement, negative ANA, negative rheumatoid factors, presence of circulating immune complexes.
 
Management
NSAIDs for pain, arthritis; corticosteroids for severe pain and recalcitrant acne.

SAPHO syndrome

A syndrome characterized by synovitis, acne, pustulosis, hyperostosis, osteitis
References in periodicals archive ?
Spondylodiscitis in SAPHO syndrome is sometimes difficult to distinguish from infectious spondylodiscitis.
Thus, the diagnosis of SAPHO syndrome was established.
5) Biopsy of the disk space in patients with SAPHO syndrome reveals chronic sterile nonspecific inflammation.
In our patient, the diagnosis of spondylodiscitis related SAPHO syndrome was established based on the medical history of palmoplantar pustulosis and computed tomography-guided biopsy which showed negative results for metastatic tumor or infection associated with hyperostosis, erosions involving sternum and medial end of clavicle and the presence of human leukocyte antigen B27.
Insidious onset of intermittent pain and swelling in the bone and joints (bilateral and symmetrical) are characteristic features of the SAPHO syndrome.
SAPHO syndrome shows no clear association with either HLA-B27 (13) or HLA-Cw6, (13) but does exhibit correlation with HLA-B39 (12) and HLA-B61.
1) The SAPHO syndrome could represent a form of "reactive osteitis" to these bacterial triggering agents.
18) As well, bisphosphonate therapy may be beneficial in conditions that mimic osteonecrosis such as SAPHO syndrome (synovitis, acne, pustulosis, hyperostosis and osteitis) of the mandible, in which the histologic appearance may resemble that of osteonecrosis.
Successful treatment of SAPHO syndrome with zoledronic acid.
He was forced to leave the navy at the age of 30 and now, at 63, takes painkillers every day to deal with the debilitating effects of SAPHO Syndrome, which effects the muscular and skeletal systems.
The ex-Royal Engineer, of Johnstone, Renfrewshire, has Sapho Syndrome, an immune system disorder.
Acne fulminans is a component of SAPHO syndrome (acronym for Synovitis, Acne, Pustulosis, Hyperostosis and Osteitis).