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. 

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

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 oedema
Nuclear medicine
  • increased uptake on Tc-99 m bone scan

The condition was first reported by Ribbing3 in 1949.

Possible imaging differential considerations include

 

 

 

 

 

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Article Information

rID: 51719
Synonyms or Alternate Spellings:
  • Ribbing disease
  • Multiple diaphyseal sclerosis
  • Ribbing's disease

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