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 ?
Clinical heterogeneity of SAPHO syndrome: challenging diagnose and treatment.
Mandibular involvement of SAPHO syndrome is clinically rare and has been reported to comprise 10% of this entity [1].
SAPHO syndrome is a constellation of multiple symptoms and signs including synovitis, acne, pustulosis, hyperostosis, and osteitis.
They also recognized a range of associated disorders, including SAPHO syndrome (synovitis, acne, pustulosis, hyperostosis, and osteitis), PAPA syndrome (pyogenic arthritis, pyoderma gangrenosum, and acne), and PASH (pyoderma gangrenosum, acne, and hidradenitis suppurativa).
SAPHO syndrome is suspected when a patient presents with a pustular skin disease in association with rheumatic pain but our patient had not any bony involvement and internal organs like the hematopoietic system [17].
To the Editor: SAPHO syndrome is a heterogeneous disorder characterized by synovitis, acne, pustulosis, hyperostosis, and osteitis.
Spondylodiscitis in SAPHO syndrome is sometimes difficult to distinguish from infectious spondylodiscitis.
Gerard, "Genetic susceptibility factors in a cohort of 38 patients with SAPHO syndrome: a study of PSTPIP2, NOD2, and LPIN2 genes," Journal of Rheumatology, vol.
CRMO is widely believed to be a pediatric equivalent of SAPHO syndrome. (4) SAPHO syndrome (synovitis, acne, pustulosis, hyperostosis and osteitis) is an inflammatory bone disorder that commonly presents with skin manifestations.
Insidious onset of intermittent pain and swelling in the bone and joints (bilateral and symmetrical) are characteristic features of the SAPHO syndrome. The most commonly affected bone sites are the metaphysis of the tubular bones, the flat bones, and the axial skeleton (spine, ribs, pelvis, sternum, and clavicle).
(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.
Reported risk factors for PSO include intravenous drug abuse with or without antecedent history of blunt trauma, acquired immunodeficiency syndrome, hemoglobinopathies and SAPHO syndrome (synovitis, acne, pustulosis, hyperostosis and osteomyelitis) [4,11].