Two cases of SAPHO syndrome / 대한내과학회지

ABSTRACT

We describe two cases of SAPHO syndrome with history of palmoplantar pustulosis and pain on the anterior chest wall and lower back area. The acronym SAPHO (synovitis, acne, pustulosis, hyperostosis and osteitis) syndrome includes a group of disorders characterized by bony lesions commonly involving the anterior chest wall and associated with skin manifestations. The skeletal manifestation is characterized by the association of inflammation and hyperostotic change, in the form of sternocostoclavicular hyperostosis, spondyloarthropathy and chronic recurrent multifocal osteomyelitis. Common cutaneous lesions include palmoplantar pustulosis, pustulotic psoriasis, and severe forms of acne. The pathogenesis remains elusive, but a link with seronegative spondyloarthropathy is probable. To date, the treatment is empirical. Nonsteroidal anti-inflammatory drugs are the first choice, and other drugs including corticosteroid, disease modifying antirheumatic drugs, pamidronate, and infliximab have been tried with some therapeutic benefit. SAPHO syndrome is a condition in the differential diagnosis of infectious or tumorous conditions of the bone. Early and proper diagnosis is important to avoid unnecessary investigations or treatments.