Sclerotic changes of the manubrium sterni

Abstract

Six females with nearly identical sclerotic and hyperostotic changes of the manubrium sterni are reported. Malignancies, bacterial inflammatory processes, and Paget disease, which were first suspected, could be excluded. The youngest patients also had sclerotic changes of other bones, including the lumbar spine, the pubic bone, and the clavicle, and may be classified as having “chronic recurrent multifocal osteomyelitis” (CRMO). The two oldest patients had ossification of the costoclavicular ligament(s) and may be classified as having “inter-sterno-costo-clavicular ossification” (ISCCO). One had only hyperostotic and sclerotic changes as seen in “sterno-costo-clavicular hyperostosis” (SCCH). The pathogenesis of these uncommon diseases is unknown, but they are all frequently associated with pustulosis palmoplantaris and have similar clinical courses and laboratory abnormalities. None of the present patients had HLA-B₂₇. The similarity of the radiological abnormalities of the manubrium sterni suggests that the diseases themselves may be similar, but with different courses depending on age, CRMO being present in children and young adults and ISCCO or SCCH in older adults.