Hereditary multiple diaphyseal sclerosis
Hereditary multiple diaphyseal sclerosis, also known as Ribbing disease, is a rare sclerosing dysplasia.
It is more common in females and typically occurs in middle-aged individuals1-2.
It may be painful or asymptomatic.
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.
- intramedullary sclerosis2
- cortical thickening and sclerosis at the periosteal and endosteal surface1
- similar to radiograph findings
- no periosteal reaction or soft tissue component 2
- 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 oedema
- increased uptake on Tc-99 m bone scan
History and etymology
The condition was first reported by Ribbing3 in 1949.
Possible imaging differential considerations include
- 1. Ihde LL, Forrester DM, Gottsegen CJ, Masih S, Patel DB, Vachon LA, White EA, Matcuk GR. Sclerosing bone dysplasias: review and differentiation from other causes of osteosclerosis. Radiographics : a review publication of the Radiological Society of North America, Inc. 31 (7): 1865-82. doi:10.1148/rg.317115093 - Pubmed
- 2, Mukkada PJ, Franklin T, Rajeswaran R, Joseph S. Ribbing disease. The Indian journal of radiology & imaging. 20 (1): 47-9. doi:10.4103/0971-3026.59754 - Pubmed
- 3. Ribbing S. Hereditary, multiple, diaphyseal sclerosis. Acta radiologica. 31 (5-6): 522-36. Pubmed