A follow-up study of 13 patients with pustulosis palmoplantaris (PPP) and skeletal disease is reported. A prolonged and fluctuating course occurred in all patients. Nine patients had anterior chest wall involvement with erosions or ankylosis of the sternoclavicular, first sternocostal and/or manubriosternal joint together with sclerosis and often hyperostosis of adjacent bones, ossification/calcification of the first costal cartilage, and in 7 patients of the costoclavicular ligament. Spinal involvement in the form of spondylodiscitis, sclerosis of vertebral bodies, syndesmophytes, paravertebral ossifications and/or spondyloarthritis occurred in 11 patients, three of whom also had involvement of the sacroiliac joints. One patient had sclerosis of a pubic and ischial bone. Peripheral arthritis or tenosynovitis occurred in 5 patients, two of whom had signs of enthesopathy, and one also erosions. The presence of a distinct PPP syndrome is suggested.