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Gorham disease

Gorham's disease or vanishing bone disease is a poorly understood rare skeletal condition which manifests with massive progressive osteolysis along with a proliferation of thin walled vascular channels. 

The disease starts in one bone but can spread to adjacent bony and soft tissues.

Epidemiology

Gorham's disease is thought to be non hereditary and there is no recognised gender predilection. It can potentially occur in any age group although most reported cases have been in young adults 2.  

Pathology

The osteolysis is thought to be due an increased number of stimulated osteoclasts 3, which is likely secondary to benign vascular proliferation in the affected region 9

Location

Can potentially involve any bone. Reported sites include

  • humerus (first reported case)
  • shoulder girdle
  • pelvis
  • skull 2
  • mandible

Splenic lesions and soft-tissue involvement can be common extra skeletal manifestations 6.

Radiographic features

Plain film and CT
  • intramedullary or subcortical lucent foci may be the earliest manifestation 1
  • this progresses to profound osteolysis with resorption of affected bone and lack of compensatory osteoblastic activity or periosteal reaction
Scintigraphy
  • Tc99m bone scan may initially be positive but later becomes negative with ongoing bone resorption

Etymology

It was first reported by Jackson in 1838 but later defined by Gorham and Stout in 1955.

Differential diagnosis

Imaging differential considerations include:


Related articles

Bone tumours

The differential diagnosis for bone tumours is dependant on the age of the patient, with a very different set of differentials for the paediatric patient.

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