Pagets Disease
Pagets Disease
Pagets Disease
Presented By
Differential Diagnosis
References
Age/Sex: 35/m
Chief complaint:
Patient complains of swollen gums in upper gums since 5 -6 months.
Non Contributory
Provisional diagnosis:
Fibro osseous lesion in relation to anterior region of maxilla.
Differential diagnosis:
AOT
CEOT
Investigations :
Hematologic: RBS- 76 mg/dl, Hb 12.8 gm%, BT 1 45, CT 500 HIV & HBsAg negative
HISTOPATHOLOGIC EXAMINATION
Connective
tissue stroma
shaped bone
fragments without
osteoblastic
rimming. Resorption lacunae are seen
but no
osteoclasts are evident
Irregularly
shaped bone
fragments showing
appositional
and reversal lines depicting
typical
mosaic pattern
FINAL DIAGNOSIS
PAGETS DISEASE
Discussion
Uncoordinated increase in the osteoclastic and osteoblastic activity
of bone cells of older adults producing larger but weaker bones, extensive pain, high levels of serum alkaline phosphatase & urinary hydroxyproline & an increased tendency to develop malignant bone neoplasms.
Was first described by Sir James Paget in 1877. Characterized by excessive and abnormal remodeling of bone
Leads to distortion and weakening of bones. Common in middle aged and elderly patients
Genetic (Sequestrosome 1 gene on chromosome 5 ) Viral infection may be necessary to trigger Inflammatory cause Chronic infection ( Osteitis Deformans) Autoimmune disorders
Hormonal Dysfunction
Prevalence of Paget's disease increases with age. Most commonly diagnosed after 50 years and rarely in people
vascularity.
Skull:
skull
and
enlargement
frontally
bitemporally
(frontal bossing);
Dilated scalp veins; headaches,
If teeth are present, they become loose and migrate, producing some
spacing.
severe as in maxilla.
because the lips are too small to cover the enlarged jaw.
Osteolytic
phase can produce osteoporosis circumscripta (localized cranial osteoporosis) i.e. decreased radiodensity of bone and alteration of trabecular pattern.
Cotton Wool appearance due to patchy areas. Bone softening can produce bowing, platybasia, protrusion
Mixed phase shows generalized bone enlargement. Sclerotic phase shows increased density, trabeculae and cortical
thickening .
a quiescent stage.
Hence, Paget's disease consist of following 3 phases: Lytic Mixed lytic and blastic
by an increased number of overtly large osteoclasts (these abnormal osteoclast consist of as many as 100 nuclei)
appearance of many small irregularly shaped bone fragments that appear to be joined in jigsaw or mosaic pattern with deeply staining hematoxyphilic reversal lines. This pattern is histologic hallmark of Paget's disease.
tissue- this causes increase in the regional blood flow and thus leads to rise in skin temperature seen clinically. centre on blood vessels; the bones are very hard and dense.
Sclerotic / burned out phase Eventually osteoblastic activity diminishes and osteoporotic or burned out phase predominates.
The new bone thus formed is disordered, poorly mineralized and
Histology of Paget's disease shows several bone spicules in highly vascularized connective tissue stroma
Loss
of normal trabeculation and appearance of irregular osteoblastic activity gives rise to typical cotton wool appearance of Paget's bone.
and
PATHOPHYSIOLOGY
Etiology Hormonal Dysfunction Autoimmune States Viral Causation Chronic Infection Genetic Factor (Sequestrosome 1 gene on chromosome 5)
Hyperactivity of osteoclasts Osteoclast destroys lamellar bone Increase extensive resorption osteoblast response
Ca ECF
Compensatory Mechanism
Compression of nerves
Overgrown bone
Bone pain Spinal stenosis Numbness Weakness (Skull) headaches, dizziness, facial droop with vision or hearing impairment
- Hypervascular / Osteolytic Phase: Initial phase of disorder involves bone resorption by osteoclasts. Subsequently there is vigorous osteoblastic response, producing excessive, poorly organized, structurally weak, highly vascular woven bone. Disease causes significant increase in blood flow of involved bones. This increase may be so extensive, due to small arteriovenous shunts, that cardiac output is significantly increased. Degenerative arthritis is assoc. w/ hypervascularity of ends of bone. After some time osteoclastic activity subsides & woven bone is replaced by lamellar bone as the osteoblastic action persists.
Intermediate Phase: In this phase osteoblastic activity predominates, but osteolytic activity is also present and therefore, and bone structural changes and bone deformity are manifest.
Quiescent stage: Finally, osteoblastic activity diminishes & bone becomes quiescent, with bony sclerosis and no evidence of increased turnover of bone, bone enlargement and widening. Vascular fibrous tissue replaces the marrow. Haversian systems are absent.
activity of the disease. When Paget disease occurs around a joint, secondary osteoarthritis may ensue. Skull involvement may lead to the following:
In
patients with Paget disease who have extensive bony involvement, this increased bone vascularity may cause highoutput cardiac failure and an increased likelihood of bleeding complications following surgery.
serious complications, including nerve root compressions and cauda equina syndrome. Fractures, which are the most common complication of Paget disease, may occur and may have potentially devastating consequences.
formation. Serum calcium & phosphorus, & acid phosphatase are usually normal (unless the patient has been immobilized in which case hypercalemia may occur). uric acid level may be increased. Increased urinary & serum levels of hydroxyproline levels ( elevated urinary hydroxyproline is a reflection of bone lysis and is seen in other conditions such as RA, hyperparathyroidism, and osteomyelitis. Urinary pyridinium / pyridinoline peptides can also be used as markers of increased activity.
The juvenile form of Paget disease differs greatly from the adult
version.
Paget's disease showing Paget cells with atypical round or oval nuclei and abundant basophilic, amphophilic or clear cytoplasm. The Paget cells are present as single cells and located throughout all layers of the epidermis
Paget
cells
with
negative
Paget's disease showing Paget cells with nuclear positivity for androgen receptors in more than 10% of tumor cells.
Paget's disease showing paget cells with nuclear positivity for ARs in almost 100% of tumor cells.
Rare example of mammary Paget's disease showing Paget cells with nuclear positivity for ERs.
Paget's disease showing Paget cells with intense complete membrane staining (3+) for Her2/neu in almost 100% of tumor cells.
Mild injuries may cause acute true pathologic fractures which are
been observed.
Left Ventricular hypertrophy is an associated finding.
the cortical plate enlargement along with mixed radiopacities, in relation to the maxillary right quadrant and may resemble Paget's disease of bone.
Pagets disease of bone may transform into Osteosarcomas. The dysplastic lesion of Pagets is always polyostotic.
Osteogenesis Imperfecta: Also called brittle bone disease, or "Lobstein syndrome, is a genetic
bone disorder.
The patients age (36 years) mitigates against Paget disease, which
This can be associated with either reversible pulpitis or irreversible pulpitis. It can also be associated with areas of inflammation such as periodontitis.
Radiographic Features:
Location: Surrounding the source of inflammation a tooth or an area of inflammation. Edge: Well-localized. Number: May be single or multiple. Staphylococcus aureus culture is always positive. Bone deposits symmetrically and concomitantly.
Surgery
Arthroplasty where the damaged joint is removed and replaced with an artificial joint; the most commonly performed arthoplasties are hip replacements and knee replacements Arthrodesis where the joint is fused into a in a permanent position
Sapp Shafers textbook of Oral Pathology 6th edition. Oral & Maxillofacial Pathology 3rd edition Neville Clinical Outline of Oral Pathology: Diagnosis and TreatmentLewis R. Eversole http://www.wheelessonline.com/ortho/pagets_disease_etiology_a nd_pathogenesis http://emedicine.medscape.com/article/334607-overview#a0104
http://www.wheelessonline.com/ortho/pagets_disease
http://www.dentistry.ucla.edu/pic/visitors/pdb/page1.html#dif_
diag John T. Anderson et al. Osteolytic form of Paget's disease differential diagnosis. The journal of bone and joint surgery. vol. 58-a, no. 7, october 1976 Karpagaselvi Sanjai, Jayalakshmi Kumarswamy, Vinod K Kumar, and Archana Patil. Florid cemento osseous dysplasia in association with dentigerous cyst. J Oral Maxillofac Pathol. 2010 Jul-Dec; 14(2): 6368.
Imperfecta of the Temporal Bone. Radiology 2002; 224:166 170. A. J. Lyons & A. V. Babajews. Gigantiform cementomaan unusual incidental finding. 1986, The British Journal of
findings/apical-findings-inflammatory/sclerosing-osteitis/