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 Table of Contents  
CASE REPORT
Year : 2020  |  Volume : 32  |  Issue : 2  |  Page : 199-202

Paget's disease of bone: A rare case report


Department of Oral Medicine and Radiology, Government Dental College, Srinagar, Jammu and Kashmir, India

Date of Submission09-Jul-2019
Date of Decision30-Mar-2020
Date of Acceptance23-May-2020
Date of Web Publication27-Jun-2020

Correspondence Address:
Dr. Tauseefa Jan
Government Dental College, Srinagar - 190 010, Jammu and Kashmir
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/jiaomr.jiaomr_130_19

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   Abstract 


Paget's disease of bone (PDB) is a localized bone remodeling disorder that is chronic, non-inflammatory.. It is also known as “osteodystrophia deformans” as described by Sir James Paget. PDB is the second most common bone disease after osteoporosis. This disease is found to be relatively more common in older people, occurs in approximately 3–4% of the population aged over 50 years with a slight male gender predilection. Jaw involvement is seen with the ratio of maxilla to mandible 2:1.

Keywords: Osteitis deformans, osteoporosis, Paget's disease


How to cite this article:
Tak MM, Chalkoo AH, Jan T. Paget's disease of bone: A rare case report. J Indian Acad Oral Med Radiol 2020;32:199-202

How to cite this URL:
Tak MM, Chalkoo AH, Jan T. Paget's disease of bone: A rare case report. J Indian Acad Oral Med Radiol [serial online] 2020 [cited 2020 Jul 7];32:199-202. Available from: http://www.jiaomr.in/text.asp?2020/32/2/199/288124




   Introduction Top


Paget's disease of bone (PDB) was first described by Sir James Paget in 1877, a British surgeon who named the disease “osteitis deformans,” as he believed the disease was caused by chronic inflammation. Abnormal expansion of bone, with both osteolytic and sclerotic areas are found for increased activity of bone cells. Abnormal multinucleated osteoclasts initiate enhanced resorption of bone followed by disorganized bone formation by osteoblasts.[1] The overlying skin of affected sites of Skelton is erythematous and warm to touch due to increased rate of bone remodeling, high metabolic activity of bone, and the high blood flow to affected sites.[2] After osteoporosis PDB is the second most common metabolic bone disorder.[3] It may be monostotic (17%), but is more frequently polyostotic, with a predilection for the axial skeleton (the spine, pelvis, femur, sacrum and skull, in descending order of frequency) although any bone may be affected.[4]


   Case History Top


A 70-year-old male reported to our department of oral medicine and radiology with a chief complaint of pus discharge from the left posterior region of the upper jaw for 6 months. History revealed a gradual expansion of maxilla with a spacing of teeth in the upper front jaw region.

On extra-oral examination, a tower-shaped skull is appreciated because of a prominent sagittal suture as seen in [Figure 1]. The nasiolabial sulcus is obliterated due to the diffuse expansion of the malar and zygomatic bones[Figure 2]. Intra-oral examination reveals a deranged occlusion, the alveolar mucosa, and attached gingiva is erythematous with dilated vessels. The extraction socket concerning 26 is unhealed and have frank pus discharge. Diffuse expansion of the maxillary alveolus is seen bilaterally, extending from 18 to 28 regions with obliteration of the buccal vestibule. The palatal vault is flat. On palpation, the swelling of the maxillary alveolus is bony hard in consistency and extends from 18 to 28. Teeth present in the maxillary arch are firm with increased interproximal spacing. The mandibular arch did not reveal any significant abnormality [Figure 3].
Figure 1: Prominence of sagittal suture giving tower shaped appearance to skull

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Figure 2: Nasiolabial sulcus is obliterated due to the diffuse expansion of the malar and zygomatic bones

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Figure 3: Intraoral image showing expansion of maxilla and spacing of teeth

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   Investigations Top


Radiological investigations carried out were IOPAR, OPG, skull radiograph, pelvis radiograph, and CT scan.

In Intraoral periapical radiographs, hypercementosis of roots can be appreciated in the periradicular region of 11, 21 [Figure 4].
Figure 4: Intraoral Periapical radiographs of upper central incisors reveals hypercementosis of roots

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OPG shows irregular trabeculae organized into rounded radiopaque patches of abnormal bone, creating cotton -wool appearance in the maxilla. A normal trabecular pattern of bone with normal density is detected in the mandible [Figure 5].
Figure 5: OPG shows irregular trabeculae organized into rounded radiopaque patches of abnormal bone, creating cotton -wool appearance in the maxilla. Normal trabecular pattern of bone with normal density is detected in mandible

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In Posterior - anterior skull view and Lateral view of skull irregularly shaped generalized flecks of radiopacities involving the entire skull are present, giving a cotton wool appearance.

In CT scan of head and neck region expansion of calvarial bones with mixed sclerotic and lytic lesions involving both inner and outer tables are appreciated, giving the cotton wool appearance of bones. Frontal bone appears enlarged with the appearance of skull falling over facial bone –Tam-O'-shanter sign on -sagittal CT section. Similar changes are shown in the maxilla, but mandible displays normal bone density and trabecular pattern. Cervical vertebrae C4-C5 shows lytic and sclerotic areas [Figure 6], [Figure 7], [Figure 8].
Figure 6: Axial view, CT scan of head and neck region shows expansion of calvarial bones with mixed sclerotic and lytic lesions involving inner and outer table giving cotton wool appearance of bones. Frontal bone appears enlarged with appearance of skull falling over facial bone –Tamo' shanter sign. Similar changes are shown in maxilla, mandible displays normal density and pattern

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Figure 7: Coronal view, CT scan of head and neck region shows expansion of calvarial bones with mixed sclerotic and lytic lesions involving inner and outer table giving cotton wool appearance of bones. Frontal bone appears enlarged with appearance of skull falling over facial bone –Tamo' shanter sign. Similar changes are shown in maxilla, mandible displays normal density and pattern

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Figure 8: Sagittal view, CT scan of head and neck region shows expansion of calvarial bones with mixed sclerotic and lytic lesions involving inner and outer table giving cotton wool appearance of bones. Frontal bone appears enlarged with appearance of skull falling over facial bone –Tamo' shanter sign. Similar changes are shown in maxilla, mandible displays normal density and pattern

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The radiograph of the pelvis also shows the cotton wool appearance of bone [Figure 9].
Figure 9: X-ray view of pelvis also shows cotton wool appearance of bone

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Baseline investigations are within normal limits. Biochemical tests revealed normal serum calcium: 9.2 mg/dl and serum phosphorus: 4 mg/dl, Serum alkaline phosphatase (SAP) level is markedly increased: 1153 IU/L

A final diagnosis of Paget's disease of bone was established by correlating the clinical, radiological, and biochemical findings. The patient was referred to the rheumatologist for further treatment and was kept on a periodic followup.


   Discussion Top


PDB is chronic, bone disorder characterized by abnormal bone remodeling process that affects widespread, non-contiguous areas of the skeleton and so the term “osteodystrophia deformans” would be more appropriate as compared to osteitis deformans described by Sir James Paget. After osteoporosis, PDB is the second most common bone disease. A wide geographical variation is seen in the prevalence of Paget disease, as common in Western Europe, America, and Australia, but rare in Asia and Africa.[5] This disease is found to be relatively more common in older people, occurs in approximately 3-4% of the population aged over 50 years with a slight male predilection.PDB is more common in males than female, in a study published in 2002, the incidence was reported as 5.4 cases/10000 people per year in women above 85 years, compared with 7.6 cases/10000 people per year in men of the same age. The age and sex of our case are consistent with previous literature. One of the rare metabolic bone disorder that is inherited as an autosomal recessive trait is juvenile paget's disease or idiopathic hyperphosphatasia. This bone disorder is characterized by increased bone turnover secondary to enhanced osteoclastic activity and unrelated to classic PDB, which is seen in the elderly.[6] The exact etiology of Paget's disease is not clear, certain genetic, and environmental factors are found to cause PDB. SQSTM1 gene mutation is responsible for classical PDB. A wide variety of mutations have now been identified and it has been estimated that 30-40% of patients with a family history of PDB and 10% of patients with 'sporadic' PDB carry a mutation in this gene.[7] From a clinical perspective, more severe and extensive disease and a higher rate of complications are seen in patients with SQSTM1 gene mutations than without SQSTM1 mutations. It has also been noted that risk alleles have additive effects, which means higher the number of risk alleles higher will be the risk of developing PDB and more extensive will be the disease.[8] Among the environmental trigger factors for PDB the most common is a viral infection. The viral hypothesis stemmed from the observation that inclusion bodies present in osteoclasts of PDB resemble the paramyxovirus nucleocapsid.[9] Other environmental triggers include dietary deficiency of calcium during childhood; vitamin D deficiency and childhood rickets; excessive mechanical loading of the skeleton and environmental pollutants.[10] Jaw involvement is seen with the ratio of maxilla to mandible 2:1. Maxilla exhibits progressive enlargement, the alveolar ridge becomes widened and palate is flattened as observed in the present case. Teeth may become mobile and migrate, producing some spacing. Edentulous patients with dentures commonly complain of difficulty in using dentures due to increased tightness because of the expansion of the jaws. When the maxilla increases in size it may encroach to orbit, the nasal cavity, and the maxillary antrum. Various clinical findings which include progressive jaw enlargement, the spacing between teeth, osteomyelitis of jaw bone, hearing loss, and biochemical findings like increased serum alkaline phosphate level, normal serum calcium, and phosphorus levels seen in the present case are consistent with findings reported in the literature from time to time. The chief complaint of the patient was addressed after arriving at a diagnosis,”localized osteitis”, based on the various associated clinical and radiological findings and was subsequently treated with curettage of the extraction socket for 26 along with systemic antibiotic therapy.


   Conclusion Top


For the proper and early diagnosis with effective management of Paget's disease, it is a dental practitioner needs to know the features and dental manifestations of this disease. It is also important to create a treatment plan, in which both the health and the aesthetic concern of the patient are addressed. Paget's disease is a chronic, non-inflammatory, endocrine bone disease which is usually asymptomatic in the beginning and can be diagnosed early as it presents with classic radiologic features and an early diagnosis can help in decreasing the patient morbidity because of the complications like hearing loss, pathologic fractures, arthritis, neurologic complications, heart failures, and osteosarcoma in rare cases. Certain recent advances in the treatment protocols such as introducing bisphosphonate compounds which improve bone quality as they help in the production of normal and near-normal bone. These patients need multidisciplinary care and a periodic follow-up as the complications of the disease are diverse.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
   References Top

1.
LeBoff MS. Metabolic bone disease. In: Kelly WN, Ruddy SR, Harris ED, Sledge C, editors. Textbook of Rheumatology.5th ed, vol 2. Philadelphia: W.B. Saunders; 1997.p. 1574-80.  Back to cited text no. 1
    
2.
Al Nofal AA, Altayar O, Ben Khadra K. Bone markers in Paget's disease of the bone: A systemic review and meta-analysis. Osteoporosis Int 2015;26:1875-91.  Back to cited text no. 2
    
3.
Van Staa TP, Selby P, Leufkens HG. Incidence and natural history of Paget's disease of bone in England and Wales. J Bone Mine Res 2002;17:465-71.  Back to cited text no. 3
    
4.
Meunier PJ, Salson C, Mathieu L, Chapuy MC, Delmas P, Alexandre C, et al. Skeletal distribution and biochemical parameters of Paget's disease. Clin Orthop 1987;217:37-44.  Back to cited text no. 4
    
5.
Bastin S, Bird H, Gamble G, Cundy T. Paget's disease of bone: Becoming a rarity. Rheumatology (Oxford) 2009;48:1232-5.  Back to cited text no. 5
    
6.
Bae KB, Kwon JH, Kim YH, Jung TY, Cho JH. Juvenile Paget's disease with paranasal sinus aplasia. Clin Exp Otorhinolaryngol2008;1:224-6.  Back to cited text no. 6
    
7.
Ralston SH, Layfield R. Pathogenesis of Paget's disease of bone. Calcif Tissue Int 2012;91:97-113.  Back to cited text no. 7
    
8.
Visconti MR, Langston AL, Alonso N, Selby PL, Fraser WD, Mutations of SQSTM1 are associated with severity and clinical outcome in Paget's disease of bone. J Bone Miner Res 2010;25:2368-73.  Back to cited text no. 8
    
9.
Ralston SH, Langston AL, Reid IR. Pathogenesis and management of Paget's disease of bone. Lancet 2008;372:155-63.  Back to cited text no. 9
    
10.
Siris ES. Epidemiological aspects of Paget's disease: Family history and relationship to other medical conditions. Semin Arthritis Rheum 1994;23:222-5.  Back to cited text no. 10
    


    Figures

  [Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5], [Figure 6], [Figure 7], [Figure 8], [Figure 9]



 

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