Paget disease of bone: A classic case report
A 58-year-old male patient reported to the dental hospital with the complaint of difficulty in chewing food as his teeth were not in contact during mastication.\nHistory revealed that, there were swellings in the maxillary right and left posterior teeth region, which started 6 years back. Similar swellings were also seen bilaterally in the malar region and in the forehead of the right side. All swellings gradually increased in size.\nOn examination, extra-orally bilateral diffuse swellings were seen in the malar and zygomatic region bilaterally, obliterating the nasolabial sulcus and in the right frontal region [], which were bony hard in consistency on palpation. The swellings were immobile and attached to the underlying bone. Intra-orally, diffuse swelling of the maxillary alveolus was seen bilaterally, extending from 18 to 28 region [] with obliteration of the buccal sulcus. Bicortical expansion was seen in the maxillary premolar-molar region, which were non-tender and hard in consistency, attached to the underlying bone. The occlusion was deranged and on occluding, the teeth were not in contact.\nSince, the swellings were multiple, bilateral, gradually increasing in size, asymptomatic, and bony hard in consistency a provisional diagnosis of disorder of bone was made; radiologic, biochemical and histopathologic investigations were performed.\nPeriapical, occlusal, and panoramic radiographs reveal diffuse radiopacities and hypercementosis of roots of the maxillary teeth []. Postero-anterior and Lateral view of the skull reveal generalized flecks of irregularly shaped radiopacities involving the entire skull, giving a cotton wool appearance [].\nComputed tomography scan reveals osteosclerosis of right maxillary, ethmoid, frontal, and sphenoid sinuses with widening of diploic space with sclerotic and lytic areas involving inner and outer tables of bony calvarium []. There is an expansion, with sclerosis of all walls of right maxillary, right ethmoid, and floor of left maxillary sinuses.\nBiochemical tests revealed normal serum calcium: 9.2 mg/dL and serum phosphorus: 4 mg/dl, but markedly raised serum alkaline phosphatase (SAP): 1251 IU and urinary hydroxy-proline: Creatine ratio: 243.6 mmol.\nAn incisional biopsy of the swelling was carried out in the left maxillary alveolus. Histopathologic examination revealed a cellular proliferating inter-trabecular fibroblastic marrow with many clusters of osteoclasts separating spicules of bone, which appear lamellar in some of the bits with numerous cement lines forming a mosaic pattern. Trabecula of bone with basophilic reversal lines in connective tissue stroma are seen [].\nCorrelating the clinical, radiologic, biochemical, and histopathologic findings a final diagnosis of PDB was established. Patient was referred to the Rheumatologist for treatment.