 Hello everyone welcome back to a new session on dentistry and more today's topic is pages disease or a status deformance or Lyontiasis so it is a bone remodeling disease the normal bone is undergoing resorption and bone new bone formation but if there is a exaggerated or abnormal changes in resorption and osteogenesis that resorption is loss of bone by the osteoclastic activity and osteogenesis is the loss bone is replaced by new bone so if this process is hampered the disease is known as pages disease so let's get into the details of pages disease pages disease is a bone remodeling disease the normal bone formation and bone resorption is disturbed or it is exaggerated so it is a chronic disorder of bone remodeling process because there will be excessive breakdown of bone and abnormal bone formation so normally the bone resorption and bone formation happens but here what happens there is an excessive breakdown of bone and there will be abnormal bone formation so that is a problem with pages disease the routine nature of bone remodeling is disturbed and excessive breakdown of bone and abnormal bone formation happens it is also known as a status deformance because a status we know the inflammation or it is of bones that is bone inflammation which results in deformation or change or appearance the formal change the change the shape of change happens and leontiasis it is when it affects the facial bones it gives an appearance of land face so it is seen in many diseases pages disease vibrates this place here so that's why it is known as a status deformance and leontiasis so in pages disease usually in a healthy bone what happens remodeling which removes the old piece of bone and replaces them with a new a fresh bone so that is a normal routine procedure but what happens in pages disease this process shift out of balance and which results in new bone that is abnormally shaped that is abnormally shaped new bone which could be weak and brittle so that and there will be excessive breakdown of bone at the same place so this process is gone out of balance in pages disease now let's see the clinical features it's seen commonly among males two is to one ratio compared to females and it affects the older skeletal bone which was described first by England surgeon and England James pages that's why it got this name pages disease so basically it has two types one is inflammatory and congenital so etiology also either it could be a inflammatory or a genetical cause so inflammatory could be a viral infection that is paramixovirus infection and a mutation in chromosome number 5 could be the etiology of pages disease while moving on to the pathogenesis as I mentioned it is a bone remodeling disease there is hypervascular or osteolytic phase so what happens there is a initial phase of disorder involves bone resorption by osteoclast so osteoclastic activity is changed and there will be intermediate phase so osteolytic activity and osteoblastic activity is changing and there will be a phase that is known as burnout stage the normal process of osteoblast and osteoclast is completely changed and it gets to a stage known as burnout stage so burnout bone is formed because of the osteolytic and osteoblastic activity so before that we need to see the path of physiology it has three phases that is lytic phase mixed lytic and plastic and sclerotic phase the first lytic phase where the osteoclast action will be pronounced there will be more osteoclastic action the bone resorption is happening in the second phase it is both plastic and lytic phase that is both osteoclast and osteoblast is there and osteoblastic activity that is a bone formation activity is overtaken the bone resorption so there will be more bone formation but this forming bone is not of a good quality it is a weaker than the actual bones and while moving to the third stage that sclerotic phase sclerotic phase where the new bone formation is overtaking the bone resorption so the new bone which is formed which was like structurally disorganized and it is also a weaker bone so eventually what happens the osteoblastic activity will be overtaken will overtake the osteoclastic activity there will be more bone formation more weaker bone formation but eventually the osteoblastic activity also slows down and finally there will be a dormant state it will reach and the bone in that dormant state is known as burned out bone or burned out state so it is a mixed effect of osteolytic and osteoclastic activity so this abnormal matrix persists but cellular activity is nearly absent in the burnout state which is a pathophysiology of pages disease so in symptoms it is usually asymptomatic in earlier stage but may exhibit a variety of non-specific symptoms due to the increased bone remodeling so it is commonly affecting pelvis spine skull and long bones so basically an individual experience two types of problem one is bone pain and another one is fractures so the skeletal deformity includes the both legs and frontal occipital skull enlargement the legs will be both and the enlargement of skull in frontal and occipital region kyphosis that is a spinal problem the patient is like a bent a little bit forward that is kyphosis and there will be warm sensation that is increased blood supply to this abnormal bone and when this bone impinging on the nerves there will be nerve damage when this abnormal bone formation it impinges on the nerve it causes hearing loss or visual abnormalities and there will be cardiac problems that is cardiac failure could be there and shortness of breath and also it is associated with osteoarthritis vertigo tinnitus deafness and malocclusion osteoporosis so all these are the various symptoms of pages disease so when it affects the facial bones it may give a lion phase what's for it's known as lantiasis and in histology it gives a jigsaw puzzle or mosaic pattern that is a unique feature of pages disease there will be both osteoblasts and osteoclasts in the section and investigation we can find out this disease there will be increased alkaline phosphatase increased acid phosphatase and increased hydroproline so while moving on to the x-ray investigation there is a cotton wool appearance of skull that is a unique feature cotton wool appearance and the next thing is there will be widening of diploid space and which results in tamo shanta tamo shanta appearance so tamo shanta is nothing like when this pageate disease happens in skull when it affects skull there will be widening of the diploid space and an overall enlargement of cranium so this combined effect with platybasia leads to appearance of the skull which is falling over the facial bone so that is like tamo shanta hat so tamo shanta hat is like which hat which should fall over the head so it has this particular appearance tamo shanta appearance tamo shanta hat appearance because of this widening of diploid space enlargement of cranium with platybasia and which leads to appearance of skull which is falling over the facial bone so that is tamo shanta appearance and there will be a cantary flame sign which can be seen in longer bones that is blade of grass because of this peculiar appearance of longer bone radiographic feature that is cantal flame or blade of grass cantal flame or blade of grass moving on to differential diagnosis we have osteomalacia fibrous displace here multiple myeloma and osteopetrosis so in treatment modalities we have surgical management for deformity and we need to control the pain relief of pain and suppression of active diseases can be done using this phosphonates vitamin D and calcitonin so that's all about pages disease which is also known as a status deformance or leantiasis so it is improper osteoblastic and osteoclastic atuchi which results in bone deformation and abnormal bone formation of inferior quality and bone resorption so the takeaway points are one is the cantal flame blade of grass appearance in radiography the burnout stage in its pathogenesis the cotton wool appearance of skull in radiography tamo shanta appearance of this diploic space and enlarged skull so tamo shanta hat appearance that is also in radiography mosaic jigsaw pasell appearance in histology so that's basically a mixed osteoclastic osteoblastic effect causing a improper bone formation and resorption so I'll come up with a new new disease and dentistry and more thank you