Pagets Disease

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A Case Report

Presented By

Dr. Kush Pathak

Case description Discussion Pathophysiology Lab Investigations

Differential Diagnosis
References

Age/Sex: 35/m
Chief complaint:
Patient complains of swollen gums in upper gums since 5 -6 months.

History of present illness:


Swelling present since 5-6 months associated with mobility of teeth. Pain was not present in the beginning but at present since one month, pain is mild and continuous.

Past medical history: Personal history: Family history:

Non Contributory

General physical examination:


Pt. was cooperative, moderately built, moderately nourished & presented with a normal gait. Pallor was seen

Extra - Oral Examination:


Diffuse swelling present in relation to premaxillary region with obliteration of nasopalatine fold & elevation of ala of nose of left side seen.

Intra - Oral Examination:


Hard tissue examination: stains ++ gen. severe attrition gr. I mobility irt 11, 12, 13, 21, 23 gr. III mobility irt 22

Soft tissue examination:


Obliteration of ala of nose seen. Lips are incompetent

Exophytic lesion firm to hard present in relation to premaxillary region


excluding the anterior part of hard palate.

Tenderness on palpation seen.

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

Cotton Wool appearance due to patchy areas.

Generalized bone enlargement. Increased density, trabeculae and cortical thickening .

HISTOPATHOLOGIC EXAMINATION

Connective

tissue stroma

Stroma composed of irregularly

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.

It is also called osteitis deformans.

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

Etiology of Paget's disease is still unknown. But possible etiology includes:

Genetic (Sequestrosome 1 gene on chromosome 5 ) Viral infection may be necessary to trigger Inflammatory cause Chronic infection ( Osteitis Deformans) Autoimmune disorders

Hormonal Dysfunction

Connective tissue and vascular disorders

Prevalence of Paget's disease increases with age. Most commonly diagnosed after 50 years and rarely in people

younger than 20 years

Systemic findings include: almost all bones can be involved


The major characteristic is enlargement of affected bone. Patient complains of bone pain perceived as a dull constant aching

pain deep below the soft tissues.

The involved bones become warm to touch because of the increased

vascularity.

Non specific headaches, impaired hearing and tinnitus are common

symptoms of Paget's disease.

Bowed legs, Enlarged pelvis

Skull:

skull
and

enlargement
frontally

bitemporally

(frontal bossing);
Dilated scalp veins; headaches,

dizziness, loss of muscle strength in the face (facial droop), or

problems with vision or hearing.

ORAL MANIFESTATIONS include Involvement of jaws


Maxilla exhibits progressive enlargement, the alveolar ridge

becomes widened and palate is flattened.

If teeth are present, they become loose and migrate, producing some

spacing.

When mandible is involved, findings are similar but not usually as

severe as in maxilla.

As disease progress, mouth may remain open, exposing teeth,

because the lips are too small to cover the enlarged jaw.

Edentulous patients complain of inability to wear dentures because

of increasing tightness due to expansion of 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

acetabuli or greenstick fractures.

Mixed phase shows generalized bone enlargement. Sclerotic phase shows increased density, trabeculae and cortical

thickening .

Paget's disease is characterized by enhanced resorption of bone by

giant multinucleated osteoclast with formation of disorganized woven bone by osteoblast.

This process evolves through various phases of activity, followed by

a quiescent stage.

Hence, Paget's disease consist of following 3 phases: Lytic Mixed lytic and blastic

Sclerotic or burned out

Initial osteolytic phase is marked by disordered areas of resorption

by an increased number of overtly large osteoclasts (these abnormal osteoclast consist of as many as 100 nuclei)

The next osteoblastic phase follows with haphazard laying of new

bone matrix and formation of woven bone.

Repeated episodes of bone removal and formation results in

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.

The Paget's bone is coarse with an affinity for calcium and


phosphorus

Marrow spaces are filled with loose highly vascularized connective

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.

Pagetic bone shows no tendency to form haversian systems or to

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

lacks structural integrity.

The proliferation of bone and concomitant hypercementosis

sometimes results in obliteration of PDL.

Histology of Paget's disease shows several bone spicules in highly vascularized connective tissue stroma

Higher magnification : A- osteoclast in howship lacunae B- osteoblast in process of bone formation

MOSAIC PATTERN IN PAGETS DISEASE

Loss

of normal trabeculation and appearance of irregular osteoblastic activity gives rise to typical cotton wool appearance of Paget's bone.

Teeth and adjacent bone also show radiographic changes :

pronounced hypercementosis and loss of well defined lamina Dura.

Root resorption is reported in some cases but it is unusual.

Radiographic image shows typical cotton wool appearance in Paget's disease

Hypercementosis loss of lamina dura

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

Rapid disposition of vascular connective tissue

Increase bone formation

Increase bone size and thickness

Poorly mineralized bones

Compression of nerves

Overgrown bone

Bowing of legs Curve backbone

Bone pain Spinal stenosis Numbness Weakness (Skull) headaches, dizziness, facial droop with vision or hearing impairment

Skull enlargement bitemporally and frontally Enlargement of pelvis

- 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.

Complications of Paget disease depend on the site affected and the

activity of the disease. When Paget disease occurs around a joint, secondary osteoarthritis may ensue. Skull involvement may lead to the following:

Deafness Vertigo Tinnitus

Dental malocclusion Basilar invagination Cranial nerve disorders

Frequently, erythema is present over the affected bone area, which

is due to the increased hypervascularity.

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.

Vertebral involvement of Paget disease may be associated with

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.

Rarely, Pagetic bone may undergo a sarcomatous transformation.

Increase in alkaline phosphatase, which reflects increase in bone

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.

Levels of bone turnover markers (including markers of bone formation

and resorption) are elevated.

Markers of bone turnover that are useful to monitor in persons with

Pagets disease include the following:

Deoxypyridinoline (marker of bone resorption).

N -Telopeptide of type I collagen (marker of bone resorption).

Alpha-alpha type I C -Telopeptide fragments.

The juvenile form of Paget disease differs greatly from the adult

version.

Juvenile Paget disease is characterized by widespread skeletal

involvement and has distinctly different histologic and radiologic features.

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's disease showing immunoreaction for ERs

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.

Incomplete stress fractures frequently occur in Pagets disease.

Mild injuries may cause acute true pathologic fractures which are

more common in women than men.


Degenerative joint disease is associated with Pagets disease. Cardiovascular abnormalities such as increased cardiac output has

been observed.
Left Ventricular hypertrophy is an associated finding.

Florid osseous dysplasia

Familial gigantiform cementoma,


Osteogenesis imperfecta. Sclerosing osteitis,

These all present with similar localized clinico-radiographic

pictures as PDB without the generalized skeletal changes and no


changes in alkaline phosphatase levels.

Florid cemento osseous dysplasia


Seen in middle-aged black women. Shows

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.

COD case do not show an increase in the serum alkaline.


Phosphatase level and in histopathology, there was no jigsaw puzzle

or mosaic pattern of trabeculae seen.

Osteogenesis Imperfecta: Also called brittle bone disease, or "Lobstein syndrome, is a genetic

bone disorder.

Caused due to small or no amount of bone collagen (a protein

found in connective tissue)

Pagetic involvement of the bony labyrinth is often asymmetric, in

contradistinction to the pattern of Osteogenesis Imperfecta .

The patients age (36 years) mitigates against Paget disease, which

affects an older population.

FAMILIAL GIGANTIFORM CEMENTOMA


Multiple, large cemental masses found in the maxilla and mandible.

Inherited as an autosomal dominant trait.


Pagets disease has similar features still the main difference is that it

does not begin at an early age.

Increased bone deposition due to inflammation.

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.

Medication to help regulate bone remodelling

Biphosphonates, Zoledronic acid, Calcitonin

Medication to relieve pain Pain killers & Physiotherapy

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

Contemporary Oral & Maxillofacial Pathology J. Philip

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.

Heimert T. L., Li D. M., Yousem M. D. Osteogenesis

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

Radiology, 59, 277-279


http://drgstoothpix.com/radiographic-interpretation/pulpalapical-

findings/apical-findings-inflammatory/sclerosing-osteitis/

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