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Paget’s Disease of the Bone

Paget’s disease, is an osteolytic disease that causes bone to develop too fast. According to the National Institute of Health, it “is the second most common bone disease after osteoporosis.” However, in Asia and Africa, the disease is not as prevalent.

The exact cause of the disease is still being studied, but genetics factors are suspected to play a significant role as the disease has been linked to several genes. It also appears to largely in people over the age of 55. However, there have been rare instances when it presented in younger individuals.

During an excavation of Norton Priory, 130 skeletons were recovered. The church was used for burial from the 13th- 15th centuries. Of those 130, six of them showed evidence of Paget’s, which is roughly a 19% rate of occurrence. This is quite high. All six were male and over the age of 45.

Sir James Paget was the British surgeon who first surgeon to record it, and believed it was the product of chronic inflammation. In his article, Paget noted that his patient, Mr. Stanley, was in pretty good health when he saw him 1856. However, he walked “stiffly” (38). When he saw Mr. Stanley three years later, he noted that his “left tibia had become larger, and had a well marked anterior curve” (38).

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Mr. Stanley Plate from On a Form of Chronic Inflammation of Bones (Osteitis Deformans) J. Paget

We now understand that Mr. Stanley was developing Paget’s.

Osteoblasts, osteocytes, and osteoclasts are responsible producing and maintaining our bones throughout our lifetime. Our bones are always doing their thing- helping us grow, strengthen, and heal. New bone tissue replaces old bone tissue regularly.  If something disrupts the process of laying down and maintaining bone, this could be bad.

Paget’s disease is a disorder that affects that bone remodeling process. The bones that are affected will begin producing new bone faster than it should, and the new bone is usually not quality. The bones will begin to bow or bend, compress, fracture and misshapen. Essentially, it is increased bone growth and irregular bone remodeling.

Pagets4_CR

Paget’s Disease of the Bone seen in lower leg Image from: med.stanford.ed

This image shows an individual’s lower leg that has been affected by Paget’s. The extreme bowing of the tibia is being caused by the pressure of supporting the body on weaker, softened bones. The bone is also enlarged, which is one of the early symptoms. However, it can take months or years to diagnose.

 

 

 

 

 

 

 

Paget’s can also impact the craniofacial bones, causing Leontiasis Ossea, or Lion Face Syndrome. The overgrowth of bone around the skull can result in hearing and sight loss, headaches, and other impairments.

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Magnetic resonance imaging in Paget disease shows thickening of bone around the skull Image via 

 

The skull below shows bone overgrowth of the frontal, maxillary, madibular and zygomatic regions of the face, which would have created a lion like appearance.

lionFace.jpg

 Image via medicalmuseion

Resources:
Paget J. On a Form of Chronic Inflammation of Bones (Osteitis Deformans). Medico-Chirurgical Transactions. 1877;60:37-64.9.
Magnetic resonance imaging in Paget disease of the skull.
R T Tjon-A-ThamJ L BloemT H FalkeO L BijvoetV K GohelB I HarinckG B Ziedses des Plantes 

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