Sapho syndrome

a group of disorders whose most common manifestation is osteitis of the anterior chest wall; the name is an acronym referring to the major findings of synovitis, acne, pustulosis, hyperostosis and osteitis.

SAPHO syndrome comprises a spectrum of disorders that share some clinical, radiologic and pathologic characteristics. At one end of the spectrum is chronic recurrent multifocal osteomyelitis, and at the other end is sternocostoclavicular hyperostosis. Between these two extremes are less well defined varieties of musculoskeletal manifestations associated with pustulosis palmaris et plantaris, perhaps best described as pustulotic arthro osteitis. Other cutaneous lesions associated with this syndrome are acne conglobata and psoriasis.

Involvement of the sternum, clavicles and anterior portions of the ribs occurs in both adult patients and children with chronic recurrent multifocal osteomyelitis; in both situations the patients may or may not have associated pustulosis palmaris et plantaris. In other sites subchondral lesions may be evident, particularly in the discovertebral junction and symphysis pubis but also in the sacroiliac joints.

On radiographs, bone sclerosis is a dominant abnormality. Sclerosis may simulate that of osteitis condensans ilii, osteitis pubis, idiopathic hemispherical sclerosis of a vertebral body, or condensing osteitis of the clavicle.

DR