paget 's disease ( pd ) of the bone was first described , in 1877 , by sir james paget under the term  osteitis deformans  . it is characterized by rapid bone resorption and deposition , resulting in formation of numerous reversal lines , which give rise to a mosaic pattern in the lamellar bone with profuse local vascularity and fibrous tissue in the marrow . in the initial phase of pd however , both may occur simultaneously , resulting in osteoporosis and sclerotic bone . within the sclerotic bone there is reduced vascularity ( localized vascularity ) and the ability for normal healing is compromised , hence it can lead to osteonecrosis . therapeutic agents for pd include bisphosphonates like alendronate , risedronate , and zoledronic acid . a 70-year - old male patient presented with a 3-month history of perforation in the left maxillary region with drainage of oral fluids from the nasal cavity . extra - oral examination showed localized hyperthermia with enlarged cranium , zygoma , and incompetent lips [ figure 1 ] . intraoral examination showed enlarged maxilla , fistulous tract connecting posterior maxilla and maxillary sinus , and ill - fitting temporary partial denture in relation to left maxillary molars [ figure 2 ] . 70-year - old male with perforation in the left maxillary region diagnosed with paget 's disease . extra - oral clinical photograph shows enlarged cranium , zygoma , and incompetent lips ( arrows ) . 70-year - old male with perforation in the left maxillary region diagnosed with paget 's disease . pd of the bone is diagnosed primarily by radiographic examination . early in the course of the disease subsequently , areas of sclerosis develop leading to the characteristic appearance of mixed lytic and sclerotic areas , thickened trabeculae , bone expansion , cortical thickening , and deformity . generalized mixed radiolucent and radiopaque areas were seen in the maxilla and mandible with characteristic cotton  wool appearance in the left maxilla [ figure 3 ] . paranasal sinus view revealed generalized increase in the fuzziness of all the facial bones with mixed radiolucencies in the frontal bones and marked haziness in the maxillary sinuses . lateral skull view showed generalized mixed radiolucent and radiopaque areas suggestive of pagetic calvaria [ figure 4 ] . 70-year - old male with perforation in the left maxillary region diagnosed with paget 's disease . panoramic radiograph shows generalized mixed radiolucent and radiopaque areas in the maxilla and mandible ( arrows ) with characteristic cotton wool appearance in the left maxilla . 70-year - old male with perforation in the left maxillary region diagnosed with paget 's disease . lateral skull view shows generalized mixed radiolucent and radiopaque areas ( arrows ) suggestive of pagetic calvaria . histopathology of the lesion showed increased osteoblasatic activity and hematoxyphilic reversal lines giving an appearance of the characteristic pagetoid bone . marrow spaces were filled with fibrous connective tissue , confirming osteitis deformans [ figure 5 ] . biochemical analysis showed abnormal increase ( 740 u / i ) in serum alkaline phosphatase enzyme level ( normal range is 40 - 125 u / l ) and urinary hydroxylproline ( 88 mg / day / m ) . 70-year - old male with perforation in the left maxillary region diagnosed with paget 's disease . hematoxylin and eosin stained biopsy tissue ( 100 ) shows immature bone either with osteoid and osteoblastic rimming , reversal lines , and enough fibrovascular stroma ( arrow ) . on correlating the clinical , radiographic , histopathological , and biochemical findings , it was finally diagnosed as a case of pd involving the maxilla and skull . an obturater was placed in the left maxillary ridge to prevent draining of oral fluids into the nasal cavity [ figure 6 ] . 70-year - old male with perforation in the left maxillary region diagnosed with paget 's disease . intraoral clinical photograph shows an obturator ( arrow ) placed in the left maxillary molar region . pd is a relatively common disease in western countries ; it is very rarely encountered in the indian population . it occurs mostly in the fifth to sixth decade of life , with a high preponderance in the male population . the etiology of pd is still not totally clear , but genetic and environmental factors may play a role . pd may affect the jaw bones , cementum of the teeth and pulp tissue , which lack cellular structure with manifestation of interglobular dentine . in our case altered size and shape of the maxilla , osteonecrosis that resulted in a fistulous tract , radiographically hypercementosis , displacement of the teeth , and cotton  wool appearance in both the jaws were evident . non - healing extraction sites and exposed alveolar bone following the removal of maxillary teeth in a patient with advanced pd has been reported . a case of chronic osteomyelitis involving the maxilla following dental extractions in a pagetoid patient a total of 63 cases of bisphosphonate - induced osteonecrosis of the jaws were reported ; majority of the cases affected the maxilla . in the clinical scenario , the question posed is whether osteonecrosis in postoperative sites occurs as a result of pd , bisphosphonate therapy , or a combination of the two . in both , but , in our case there was no evidence of any non - healing socket and the patient had not used any medication to treat pd . the patient was referred to a physician for treatment of pd , who prescribed alendronate 40 mg / day for 6 months . drug induced osteonecrosis in paget 's disease is common . in the present case , osteonecrosis secondary to paget 's disease ( non - drug induced ) seen in a 70-year - old male patient shows characteristic radiographic and pathologic features of paget 's disease .