Feb 24, 2017 sir james paget described paget s disease of bone in 1877. Paget s disease of bone is a common disorder which may affect one or many bones. Paget s disease of bone most commonly occurs in the pelvis, skull, spine and legs. Fortunately, a limited panel of immunohistochemical studies can help establish a correct diagnosis in many cases. Paget s disease paget s disease is a bone disorder characterized by unique histological findings. Pagets disease of bone is a common disorder characterized by increased but disorganized bone remodelling.
Jan 11, 2019 paget s disease of bone interferes with your body s normal recycling process, in which new bone tissue gradually replaces old bone tissue. Department of histopathology, the james cook university hospital, middlesbrough, uk. A common bone disorder pagets disease of bone pdb, also known as ostei7s deformans, is a common disorder characterized by focal areas of increased and disorganized bone remodeling. Pagets disease of bone american college of rheumatology.
Bone scintigraphy is a very sensitive investigation for patients with active pagets disease. A 69yearold man reports increasing pain in the right leg. Mammary and extramammary pagets disease journal of. Dec 21, 2018 paget disease is a localized disorder of bone remodeling that typically begins with excessive bone resorption followed by an increase in bone formation. Pagets disease of bone pdb shows both geographic and familial clustering. This abnormal development may cause bone pain, arthritis, deformities and fractures. Paget s disease of bone is a chronic disease of the skeleton. Pagets disease may affect up to 3% of adults older than 40 years. It is abbreviated pdb there is also a paget s disease of the bone just to make things confusing.
These changes in the bone can lead to bone enlargement and deformity. Foot pathology in patients with pagets disease of bone. A physician needs to evaluate the condition to make sure there are no related problems. Paget s disease of the bone is an uncommon benign disorder of bone with distinctive radiographic findings. Labia majora, scrotum and perineum are most common sites. Paget s disease of bone is a relatively common condition in the uk affecting up to 5% of the population over the age of 55 years with particularly high prevalence in the north west of england.
Pagets disease of bone is the second most frequent metabolic bone disorder after osteoporosis,26 where more than 3% of caucasians older than 55 years are affected. Evidence of the condition is seen on radiography in 2. Over time, the disease can cause affected bones to become fragile and misshapen. Pagets disease of bone is a chronic disease of the skeleton. Affected bones may be misshapen and easily broken fractured. Treatments can include medications, surgery, and lifestyle changes to increase bone health, such as exercise. Paget s disease of bone is a chronic condition that causes abnormal enlargement and weakening of bones. The incidence, pathology, presentation and complication of pagets disease. A bone scan is a test that helps identify which bones have been affected by pagets disease.
Get a printable copy pdf file of the complete article 1. In contrast, metastatic disease usually presents with random spotty tracer uptakes and. Pagets disease of bone causes bones to grow larger and weaker than normal. The new bone is abnormal in shape and structure and is weaker than usual. It affects the way your bone develops and renews itself, causing it to become weaker than normal. Dec, 2016 the incidence, pathology, presentation and complication of paget s disease. Bone consists of osteoblasts, osteoclasts, ostecytes, matrix. Pagets disease is a chronic disorder that can result in enlarged and misshapen bones.
With treatment, many people can manage their symptoms, improve pain, and control the effects of the disease. Dec 20, 2018 however, pagets disease can cause pain in the bones, bone deformity, fractures, and arthritis. There is increased bone turnover in focal areas of the skeleton and one or many bones can be affected there is a lytic phase to the disease process with an increase in bone resorption and abnormal osteoclast activity. Pagets disease of bone is named after sir james paget, who first identified the condition in the 19th century. Pagets disease typically occurs in an older population. Extramammary paget disease of the skin is a malignancyassociated skin disease characterised by an eczematous rash of the anogenital region and vulva, where there is an abundance of apocrine glands. Because this disease causes your body to generate new bone faster than normal, the rapid remodeling produces bone thats softer and weaker than normal bone, which can lead to bone pain, deformities and fractures. Pagets disease of bone symptoms and causes mayo clinic. Pagets disease of bone, diagnosis, histopathology, pagets sarcoma. Pagets disease of bone is a focal disorder characterized by rapid bone remodeling and the formation of structurally abnormal bone. But with pagets disease, this happens too fast and gives your bones an odd shape. Introduction pagets disease of bone represents a common disorder affect ing 34% of the population over the age of 40 years.
Moreover, a complete blood count cbc indicating anemia points to pagets disease. Pagets disease is a condition in which there is increased bone turnover. But with paget s disease, this happens too fast and gives your bones an odd shape. This epidemiologic study of pagets disease of bone used data from 788 cases and 387 spouse controls to investigate the following. Uncomplicated pagets disease is a disorder of aging skeleton, diagnosed in persons usually 55 years or older, showing a slight male predominance, and with attendant morbidity depending on the site and number of lesions. Bone cells increase in number and become larger and more active. When symptoms occur, the most common complaint is bone pain. Unregulated bone turnover causes bone enlargement, deformity, fragility, compression phenomena and, rarely, malignant transformation. Normally it grows on the bone and makes the bone breaks or bones can grow larger than before. The affected bones show signs of dysregulated bone remodeling at the microscopic level, specifically excessive bone breakdown and subsequent disorganized new bone formation. Osteosarcoma in pagets disease of bone hansen 2006.
Pagets disease is first seen by the dental surgeon. May 15, 2002 paget s disease of bone also known as osteitis deformans is a nonmalignant disease involving accelerated bone resorption followed by deposition of dense, chaotic, and ineffectively mineralized. Paget s disease of bone is a focal disorder of bone remodeling accompanied initially by an increase in bone resorption, followed by a disorganized and excessive formation of bone, leading to pain. Pagets disease is the result of local increase in bone turnover, leading to pain and deformity.
Pagets disease of bone in pagets disease, the process of renewal and repair is disrupted. The diag nosis is usually made on the basis of clinical and radiographic findings, and therefore pathologists infrequently deal. Some patients are asymptomatic but others present with bone pain or other complications such as fracture and deformity. The classic form of paget disease of bone typically appears in middle age or later. Pagets disease of bone is a chronic longlasting disorder that causes bones to grow larger and become weaker than normal. The first indication of paget s disease of bone is often abnormalities found on xrays done for other reasons.
Pagets disease of bone causes, symptoms, treatments. It is characterised by abnormal resorption and apposition of osseous tissue in one or more bones of the skeleton. Pagets disease of bone osteitis deformans hosain bouallouch shen zhou university 10 maart 2006 slideshare uses cookies to improve functionality and performance, and to provide you with relevant advertising. Xray images of your bones can show areas of bone reabsorption, enlargement of the bone and deformities that are characteristic of paget s disease of bone, such as bowing of your long bones. Physical examination reveals warmth and anterolateral bowing of the right shin.
Pagets disease pagets disease is a bone disorder characterized by unique histological findings. Studies estimate that about 1% of population in the united states have pagets disease. Pagets disease as stated is a chronic pathological condition of the bones in which there is a disorder of the normal process of bone remodeling. Coexistence of pagets disease with prostate carcinoma. Both conditions have similar clinical features, which mimic inflammatory and infective diseases. Histological diagnostic confusion can arise between pagets disease and other neoplastic conditions affecting the skin, with the most common differential diagnoses being malignant. Paget disease of bone knowledge for medical students and. In pagets disease, more bone breaks down than usual and more new bone forms than usual. Paget disease of bone is a disorder that causes bones to grow larger and weaker than normal. Pagets disease of bone pathology mini tutorial youtube. Pagets disease is an unusual disorder, for although we now have safe and highly effective treatment, there are many aspects of its pathogenesis and natural history that we do not yet understand. The differential diagnosis of anal paget s disease is pagetoid spread by a primary rectal adenocarcinoma, malignant melanoma, and acinbowen s disease table 287. The renewal and repair of bone becomes uncontrolled and increases by up to 40 times. Bone is a living, active tissue thats constantly being renewed and repaired.
Physical examination reveals warmth and anterolateral bowing of the. Paget disease of bone pdb, also known historically as osteitis deformans, is a focal disorder of bone metabolism that occurs in the aging skeleton. Pagets disease of bone pictures, causes, signs and. The procedure is also a useful way to determine the extent and activity of the disease. Pagets disease of bone is a chronic, slowly progressive skeletal condition of abnormally rapid bone destruction osteolytic and reformation osteoblastic. Pagets disease of bone with particular reference to. This osteoclastic overactivity followed by compensatory osteoblastic activity leads to a structurally disorganized mosaic of bone woven bone, which is mechanically weaker, larger, less comp. It is often asymptomatic and usually progresses slowly. The incidence of osteogenic sarcoma in southwest england and its relationship to pagets disease of bone. Paget s disease of bone may present with bone pain but is often asymptomatic. The new bone growth tends to be softer and more fragile than normal bone, and can develop in a haphazard pattern.
Some of the bones that are commonly affected by pagets disease are spine, femur, pelvis, skull, clavicle, and humerus. The affected bones show signs of dysregulated bone remodeling at the microscopic level, specifically excessive bone breakdown and subsequent disorganized new bone. Paget disease of bone pdb, or ost eitis defo rmans is a slowly progressive monostotic or polyostotic skeletal disease. Pagets disease of bone pdb is a condition of unknown etiology characterized by excessive and abnormal bone remodeling. The new bone may occur in one or more regions of the body and is structurally abnormal, dense and fragile. Pagets disease nord national organization for rare. Pagets disease pd is a focal disorder of bone remodelling that occurs commonly in older people. The most frequently affected bones are the pelvis, femur, spine, and skull. Pagets disease can be confirmed with a blood test since a person with pagets disease usually has high levels of serum alkaline phosphatase in blood. Your body constantly builds new bone to replace the old. Oct 07, 2008 pagets disease of bone osteitis deformans hosain bouallouch shen zhou university 10 maart 2006 slideshare uses cookies to improve functionality and performance, and to provide you with relevant advertising. Pagets disease of bone osteitis deformans springerlink. Apr 24, 2020 paget s disease is an unusual disorder, for although we now have safe and highly effective treatment, there are many aspects of its pathogenesis and natural history that we do not yet understand.
Paget s disease of the breast, also paget disease of the breast and paget s disease of the nipple, is a lesion of the breast. The pain experienced in the bone from pagets disease will be from the bone that gets affected. The excessive breakdown and formation of bone tissue causes affected bone to weaken resulting in bone pain, misshapen bones, fractures, and arthritis in the joints near the affected bones. Although many patients are asymptomatic, a variety of symptoms and complications may occur. Paget disease of bone is the most common metabolic bone disease after osteoporosis, affecting 24% of adults over 55 years of age. Primary cutaneous forms exist associated with underlying adnexal carcinoma, in addition to forms associated with underlying anal, rectal or. This article outlines the clinical features, diagnosis and management options for paget disease of bone. Histological diagnostic confusion can arise between paget s disease and other neoplastic conditions affecting the skin, with the most common differential diagnoses being malignant melanoma and atypical squamous. The epidemiology of pd appears to be changing rapidly, with several groups in different parts of the world reporting a marked reduction in the prevalence and incidence of pd, as well as in the severity of.
Due to underlying carcinoma in 10 20% of cases of vulvar disease. It is unrelated to the paget s disease of the breast and extramammary paget s disease. The materials fromwhichthese observations have been. Pagets disease of bone also known as osteitis deformans is a nonmalignant disease involving accel erated bone resorption followed by deposition of dense, chaotic, and ineffectively. It is unrelated to the pagets disease of the breast and extramammary pagets disease. Thus the specimens which the pathologist receives for histological examination maybe teeth or fragments of bone fromthe jaws, andit is the purpose of this paper to describe the appearances in these tissues when affected by pagets disease. A small bone marrow sample is collected after local anesthesia, to be tested in a medical lab. In a bone scan, a safe amount of a radioactive substance is injected into the persons vein. It usually occurs in one or a few bones and does not spread from one bone to another. Mammary and extramammary paget s disease are uncommon intraepithelial adenocarcinomas. Its aetiology is only partly understood, but includes both genetic and environmental factors. In normal bone, a process called remodeling takes place every day. Pagets disease of the bone is an uncommon benign disorder of bone with distinctive radiographic findings. The abnormal bone is weak, leading to fracture, and can also cause pressure symptoms because of.
Pagets disease of bone overview nih osteoporosis and. It may be localized to one or several skeletal segments. It is a common disease in older people, occurring in about 3 percent to 4 percent of the population over age 50. Fortunately, effective pharmacologic therapy, primarily with potent bisphosphonates, is now available to treat patients with complications or symptoms. Pagets disease of bone uconn musculoskeletal institute. Paget s disease of bone commonly known as paget s disease or, historically, osteitis deformans is a condition involving cellular remodeling and deformity of one or more bones. Pagets disease of bone australia pdf ppt case reports. Of greater rarity, however, is the occurrence of this pathological entity in areas other than the breast. In this disease, an initial rise in osteoclast activity is followed by a stage in which osteoclasts and osteoblasts are both active, and finally by a stage in which osteoblasts dominate. Pagets disease of bone is the second commonest metabolic bone condition after osteoporosis.
But it can be treated with medicine, surgery, and healthy lifestyle habits. Treatment typically with bisphosphonates is indicated in patients with pain that is localized to an affected site but. Any part of the skeleton can be affected, but the most common sites include the skull, spine, pelvis, thigh bone, shin and the bone of the upper arm. Pagets disease of bone may present with bone pain but is often asymptomatic. Pagets disease may cause pain or lead to other medical problems. In pagets disease, the tracer uptake on scintigraphy is often intense and welldistributed in the affected skeleton with preserved anatomic configuration. It is particularly common in the uk, north america and australasia, where its prevalence increases with age. Mammary and extramammary pagets disease are uncommon intraepithelial adenocarcinomas. Abstract paget s disease of bone osteitis deformans is a relatively common disorder of uncertain aetiology. Emerging strategies and therapies for treatment of pagets disease of bone.
May originate from intraepidermal portion of sweat glands or primitive basal cells with ability to differentiation towards glandular elements. In pagets disease, rapid bone repair leads to bones that are too soft or enlarged. Most people who have paget s disease of bone have no symptoms. It is characterized by increased bone turnover, which causes normal lamellar bone to be replaced by weak woven bone. Pagets disease of bone diagnosis and treatment mayo. What is paget s, where does it occur, what causes it and how is it. Pagets disease of bone nih osteoporosis and related. Diagnosis and treatment of pagets disease of bone american. Pagets disease of bone is an uncommon, chronic condition where bone rebuilds at a faster than normal rate. Paget s disease of bone pdb is a condition of unknown etiology characterized by excessive and abnormal bone remodeling. The incidence, pathology, presentation and complication of paget s disease.