Paget’s disease of bone is a condition characterized by abnormal and anarchic resorption and deposition of bone, resulting in distortion and weakening of the affected bones
Paget disease is a chronic bone disorder that typically results in enlarged, deformed bones due to excessive breakdown and formation of bone tissue that can cause bones to weaken and may result in bone pain, arthritis deformities or fractures.
Paget disease is a chronic bone disorder that typically results in enlarged, deformed bones due to excessive breakdown and formation of bone tissue that can cause bones to weaken and may result in bone pain, arthritis deformities or fractures.
MPD is an uncommon skin malignancy characterized by a chronic eczema-like lesion of the nipple and adjacent areolar skin. Paget cells originate in either invasive intraduct carcinoma or ductal carcinoma in-situ (DCIS) of in the deeper breast tissue.
Extramammary Paget disease (EMPD). A similar condition that involves the skin of the anogenital regions of female and male where there are an abundance of apocrine glands.
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Paget's (PAJ-its) disease of bone interferes with your body's normal recycling process, in which new bone tissue gradually replaces old bone tissue. Over time, bones can become fragile and misshapen. The pelvis, skull, spine and legs are most commonly affected.
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2. What is Paget’s disease?
Paget’s disease of bone is a
condition characterized by
abnormal and anarchic resorption
and deposition of bone, resulting in
distortion and weakening of the
affected bones
3. Bone consists of
Osteoblasts: Bone forming cells
Osteoclasts: Bone breaking cells
Osteocytes: Present in the matrix
Matrix: Collagen, Calcium, Osteocalcin
6. Clinical & Radiographic Features
Pain in or over a bone
Joint pain and limited mobility
The shape of the bone may change
-the bone in the legs may bend or bow out
-the bone in the skull may get bigger
Maxillary disease is more common than mandibular
Lionlike facial deformity (leontiasis ossea)
Patchy sclerotic areas of Skull RG (cotton wool)
Bone Scan of Mandible may show Codylar to
condylar uptake(Black beard or Lincoln’s sign)
7. Clinical & Radiographic Features
Bone breaks more easily
In late stages hipjoint may damage
Hearing problems may occur because the bone
expands and heart problems may also occur
COTTON WOOL APPEARANCE
8. Histopathological Features
Uncontrolled alternating resorption and formation of bone
Basophilic reversal lines- indicate the junction between
alternating resorptive and formative phases result in a
jigsaw puzzle, or mosaic appearance of the bone
REVERSAL LINES
10. What causes Paget’s disease
Unknown; evidence favors an infection of
genetically predisposed individuals with a
measles virus of the paramyxovirus family
Studies have demonstrated that abnormal virus-
infected osteoclasts generate IL-6, a resorptive
cytokine from the bone marrow of patients with
Paget’s disease
11. How common is Paget’s disease
The exact number of Paget’s disease is not known
Men are more likely to be affected than woman
It usually affects people over the age of 40 years
It’s estimated to affect 3% of people over the age of
40
12. Epidemiology
Prevalence
High: United Kingdom, United States, Australia,
New Zealand, Western Europe
Low: Scandinavia, China, Japan, India
Family History: positive in as many as 40% of patients
13. Treatment
At this time there is no cure for Paget’s
disease. Therefore treatment is designed to
control the symptoms and change the rate of
bone growth.
When symptomatic, bone pain is noted most
frequently and often may be controlled by
acetaminophen or nonsteroidal antiinfl
ammatory drugs (NSAIDs).
16. Treatment
Surgery
1. Fractures: to allow fractures to heal in better
position
2. Bone deformity: cutting and realigning of the
affected bones
3. Severe degenerative arthritis: joint replacement of
the hips, knees