1. A 45-year-old female presented with pain and swelling in the right side of the upper jaw and bad breath. Examination found diffuse swelling in the right maxillary region with exposed and inflamed bone. 2. Imaging showed radiolucent and radiopaque areas in the maxilla. Bone scan found increased activity in the maxilla and mandible. 3. Histopathology showed increased bone formation lines, large osteoclasts, and blood vessels. Paget's disease was diagnosed based on these findings.

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 45/F
 C/O Pain, Swelling irt Rt side of the
upper jaw and Halitosis .
 H/o Difficult extraction of teeth on the same side
 O/E Diffuse swelling was present in the right maxillary
region.
 Palpation-swelling had diffuse borders and
firm in consistency
 Intraorally -entire maxillary alveolus was enlarged and
right maxillary alveolus extending from canine to second
molar showed necrotic bone exposed into the oral cavity
With inflammation of the surrounding mucosa.
 Difficulty in walking

3. Generalized radiopacity and hypercementosis
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4. CT scan showed radiolucent and radiopaque areas in the maxilla
Bone scan
• Increased mitotic activity in
maxilla and mandible.
Laboratory investigations
• Ca-9.2 mg/dl
• P-4.3 mg/dl
• ALP-242 IU/L
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 Increased number of resting and reversal lines in sclerotic bone.
 Abnormally large osteoclast is present and many active osteoblasts..
 Marked vascularity.
Histopathology

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7. • B/L SWELLING
• >40 / OLDER
• HYPERCEMENTOSIS
• COTTONWOOL
• A. LINCOLN’S SIGN
• WADDLING GAIT
• MOSAIC PATTERN
• ALP- >200
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8. Paget Disease
(OSTEITIS DEFORMANS)

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• Paget’s Disease is a disease of bone marked by repeated episodes of
increased bone resorption followed by excessive attempts at bone
formation, resulting in weakened and deformed bones of increased mass.

10. Introduction
• Rapid turnover remodeling of bone .
• Elderly--older than 50 years
• Most prevalent in the Britain and New Zealand—Classic paget’s.
• Men : women = 3:2
• Most commonly affected --spine, femurs, skull, pelvis, sternum, and jaws.
• Maxilla 2 : 1 mandible.

11. Etiology
• Classic Paget’s disease
1. Inactivation mutations in the TNFRSF11B gene that encodes osteoprotegerin.
2. Mutations in SQSTM1 (p62), the sequestosome gene that encodes a scaffold protein
for the NFKappaB signaling pathway

12. Virus theory
• Many reports that have described viral particles in the osteoclastic cells of CPDB
• Paramyxoviruses (measles in particular), canine distemper virus and respiratory
syncytial virus.
• Helps in the formation of pagetic osteoclastic cells , increased numbers and
increased nuclei per cell.
• Active virus has not been recovered from Paget bone.

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15. Classic Paget Disease of Bone (CPDB)
• Late adult onset
• Rapid turnover of bone
• Osseous expansion with progressive skeletal deformities
• Tubular bones show bowing and spinal curvature,
• vertebral collapse occur in the later stages of the disease.

16. Simian (monkey like) Stance

17. Paget's disease of bone (osteitis deformans), which has resulted in bowing of the legs
and consequent wearing of the lateral soles & heels of the shoes

18. • All bones of the cranio-facial complex can be affected
• Foramina narrowing  cranial nerve neuropathies  deafness
blurring of the vision, head ache, paresthesia.
• Affected area will often feel warm with visibly enlarged veins /bluish hue
because of the increased vascularity.
• Skull enlargement with “Platybasia”.

19. The optic canals are narrowed, explaining the optic nerve compression and
the visual loss.

20. Leontiasis ossea

21. Dental finding
• Generalized hypercementosis which is most advanced on premolar and
molar teeth.
• Diastemas and lingual inclinations because of dental compensations in
response to the slow expansion
• Denture wearer may present with the classic complaint of a denture that ha
s become too small and may even have had several appliances

22. Paget disease produces a slow expansion of the involve
d bone; in the jaws, it creates
diastemas between teeth and malocclusions. Note the inc
reased vascularity of the area.
.Paget disease will expand the jaws so that dentures will
no longer fit in edentulous
individuals. The tissue will be warm because of the ex
cessive vascularity of pagetic bone.

24. DIAGNOSTIC WORK‐UP
• Elevated serum alkaline phosphatase level.
• Serum calcium and phosphate values are normal.

25. Histopathology
Presence of irregular and Increased number of resting and reversal lines in
sclerotic bone. - Jigsaw puzzle / mosaic pattern
 Abnormally large osteoclast is present and many active osteoblasts..
 Marked vascularity.
Marrow replaced by highly vascular fibrous CT.

26. Marked osteoclastic as well as osteoblastic activity is evi
dent in Paget disease.
An abnormally large osteoclast is present, as are ma
ny active osteoblasts.

27. Histology
Mosaic pattern of resting and reversal lines in
sclerotic bone regions
Normal bone histology

28. Radiographic appearance
• Pagetic bone appears as a mottled mixture of radiopacities and radiolucencies.
• Ratio of these elements depends on the duration/ stage of the disease.
• With progression, diffuse sclerosis is seen radiographically yielding the so-called
‘‘cotton wool’’ appearance of confluent nodular opacifications.
“fluffed, radiodense, cloud‐like aggregation”

29. Lateral skull film shows marked
enlargement of the cranium with new bone formation above
the
outer table of the skull and a patchy, dense, "cotton wool"
appearance.
Periapical film showing the
"cotton wool" appearance of the bone.

30. Osteitis circumscripta
• Osteitis circumscripta-- in early stages of disease, radiolucent ‘‘coin
shaped’’ lesions appear in the flat bones of the skull.

31. Generalized hypercementosis

32. Nuclear medicine bone scan of an elderly male, showing marked uptake in the mandible, especially on the left. The
bearded-appearance is known as the “Abraham Lincoln” sign, after the famous American president.
Black beard or lincoln's sign

33. DIFFERENTIAL DIAGNOSIS
• Radiopaque, painful expansion of the jaws in an adult may be suggestive of
osteomyelitis, particularly chronic diffuse sclerosing osteomyelitis.
• Osteosarcoma is another consideration.
• Fibrous dysplasia may resemble paget disease radiographically but would have been
present from early life.

34. TREATMENT AND PROGNOSIS
• Mild cases require no treatment.
• Paget disease is currently incurable, but the pain and deformity can be controlled
with treatment.
• Anti‐osteoclastic actions of calcitonin or the bisphosphonates.
• 200‐mg oral dose of Disodium Etidronate may be given twice daily.
• prolonged use of etidronate will adversely affect bone mineralization in the rest of
the skeleton.
• Pamidronate is another drug effective in Paget disease.

35. • Edentulous patients -- may require new and larger dentures periodically to
compensate for progressive enlargement of the alveolar processes.
• Dental complications include difficulties in extraction of teeth exhibiting
significant hypercementosis.
• During active disease, pagetoid bone is extremely vascular with multiple
arteriovenous shunts. Oral surgical procedures during this time can result in
extensive hemorrhage.
• During the later sclerotic phase, the bone is hypersensitive to inflammation and
can develop osteomyelitis with minimal provocation.

36. Prognosis
• Osteosarcomas (1% )in CPDB are seen in the elderly with severe advanced
disease
• Only 14% survive beyond 2.5 years.

37. Juvenile Paget disease
• Inherited as an autosomal recessive trait
• Clinically by deformities in the long bones,
• Patho-physiologically by rapid bone turnover
• Begins in infancy or early childhood

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