Hereditary multiple diaphyseal sclerosis, also known as Ribbing disease, is a rare sclerosing dysplasia.
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Epidemiology
It is more common in females and typically occurs in middle-aged individuals1-2.
Clinical presentation
It may be painful or asymptomatic.
Pathology
Location
Ribbing disease predominantly involves the diaphysis of long tubular bones, most commonly the tibia and femur2. The epiphyses are spared1. The skull is generally not involved1.
It can be unilateral or bilateral 1-2.
Radiographic features
Plain radiograph
- intramedullary sclerosis2
- cortical thickening and sclerosis at the periosteal and endosteal surface1
CT
- similar to radiograph findings
- no periosteal reaction or soft tissue component 2
MRI
- areas of sclerosis correspond to low signal intensity on T1WI 2
- short inversion time inversion recovery (STIR) may show high signal intensity due to marrow edema
Nuclear medicine
- increased uptake on Tc-99 m bone scan
History and etymology
The condition was first reported by Ribbing3 in 1949.
Differential diagnosis
Possible imaging differential considerations include
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