Sterno-kosto-klavikuläre Hyperostose - ein bisher nicht beschriebenes Krankheitsbild

Abstract

Persistent pulling pain in the sternum, clavicles and both first ribs, especially during cold and wet weather, necessitated medical investigations and treatment in 3 patients. Externally a cylindrical or spindle-shaped swelling of the clavicles was noticeable and two patients had signs of superior caval obstruction in addition. X-rays showed hyperostosis of the sternal and middle part of the clavicle, synostosis of the sternoclavicular joints with involvement of the two first ribs as well as a thickening and increase in breadth of the sternum. In one patient there were additional swellings of the first to fourth ribs bilaterally. During the period of observation there was no radiological progression of the bone disease. Apart from a consistently raised erythrocyte sedimentation rate blood chemistry was normal. The sterno-costo-clavicular hyperostosis led to bilateral occlusion of the subclavian veins with resulting upper venous congestion in two patients. Histology of the biopsy samples showed a characteristic hyperostotic spongiosclerosis in all three cases. The cause of this sterno-costo-clavicular hyperostosis is unknown. Clinical, radiological and histological findings indicate that it is a uniform disease which has not previously been described.

The psychological burden of an initially unexplained illness: patients with sternocostoclavicular hyperostosis before and after delayed diagnosis

“…SCCH was first described as a separate entity in 1974 in Japan and in 1975 in Germany [4,5]. In 1987, Chamot et al coined the term SAPHO (Synovitis, Acne, Pustulosis, Hyperostosis, Osteitis) for a syndrome in which SCCH was associated with generalized joint and skin manifestations [6].…”

Evolution of chronic recurrent multifocal osteitis toward spondylarthropathy over the long term

“…Sonozaki et al (7) proposed the name of pustulotic arthro-osteitis for the osteoarticular manifestations associated with PPP. This dermatosis is frequently associated with anterior thoracic involvement that, in its most advanced form, is sternocostoclavicular hyperostosis (SCCH), another pathologic manifestation described by Sonozaki et al (13) and then Kohler et al (14).…”

SAPHO Syndrome

“…Arthroosteitis of the anterior chest wall or sternocostoclavicular hyperostosis (SCCH) was first described by Kohler etal. [11] in 1975. It is a benign diathe sis of unknown etiology, characterized by hyperostosis and soft tissue ossification between the clavicles, anterior por tion of the upper ribs and manubrium, with variable hyper ostosis or ankylosis in the spine and sacroiliac joints.…”