Paget's disease


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Paget's disease

 [paj´ets]
any of three diseases named after Sir James Paget (1814–1889): Paget's disease of bone, Paget's disease of the breast, and extramammary Paget's disease.

Paget's disease of bone is a localized bone disorder that is also called osteitis deformans. It is relatively common, particularly in the United States, United Kingdom, Australia, France, and Germany, occurring in 3 per cent of the population over the age of 40 and 10 per cent of those over the age of 70. It occurs more often in males than in females. Once thought to be a rare form of localized bone disease, the disorder is now diagnosed more frequently because of newer diagnostic techniques, such as bone scanning, and routine testing of plasma alkaline phosphatase.

Symptoms of Paget's disease of bone depend on the site of the bone lesions and their severity. In 20 per cent of diagnosed cases there are no symptoms at all, and in others the symptoms are very mild. Lesions in the long bones seem to cause the most difficulty. The disease disturbs the growth of new bone tissue with the result that the bones often thicken, become soft, and coarsen in texture. In an advanced case, the weakened bone may be fractured by even a light blow, or, as in the case of the vertebrae, may collapse.

Lesions in the long bones can cause pain, deformity from bowing, and disability due to the arthritic changes that are a complication of the disease. When the disease process affects the skull the patient may complain of headaches, intermittent ringing in the ears and dizziness, and hearing loss. Severe involvement of the occipital region can cause pressure on the pons and cerebellum and compression of the spinal cord. These pathologic changes produce loss of coordination, muscle weakness, diplopia, ataxia, and other signs of neurologic dysfunction.

Diagnosis of this disease is verified by radiologic studies of the affected bones and by laboratory tests. The serum alkaline phosphatase and urinary hydroxyproline levels are elevated. Drugs of choice for its treatment are calcitonin, mithramycin, and etidronate disodium.

Paget's disease of the breast is an erythematous scaling lesion of the breast, involving the nipple and areola unilaterally, and associated with an underlying malignancy. It usually appears around the age of 55 in women, but can also affect males. (See Atlas 2, Part I.)

Extramammary Paget's disease is characterized by similar lesions occurring in middle-aged women and men, but the lesions are located in the anogenital area. The skin disorder is not always associated with malignancy, as it is when affecting the breast, but in almost half the cases there is an underlying carcinoma.

Paget's disease

(păj′ĭts) or

Paget disease

(-ĭt)
n.
1. A disease, occurring chiefly in old age, in which the bones become enlarged and weakened, often resulting in fracture or deformation. Also called Paget's disease of bone.
2. A rare form of breast cancer involving the areola and nipple and usually associated with underlying ductal carcinoma. Also called Paget's disease of the breast, Paget's disease of the nipple.

Paget's disease

A bone disease affecting up to 3% of the elderly population and involving mainly the skull, the collar bones (clavicles) the spine, the pelvis and the leg bones. The affected areas become warm with softening and increased bone growth with distortion. Skull thickening and enlargement may cause headache and compression of the CRANIAL NERVES. Legs may become bowed, spinal changes may cause compression of the cord and paralysis and fractures may occur spontaneously. Paget's disease is due to an increased number, size and absorptive activity of OSTEOCLASTS. Genetic and paramyxovirus infective factors have been implicated. It is treated with pain-relieving drugs and with bisphosphonate drugs especially zoledronic acid and risedronate. (Sir James Paget, 1814–99, English surgeon).

Paget's disease

Chromic disorder of unknown cause, usually affecting middle aged and elderly people, characterized by enlarged and deformed bones. Excessive breakdown and formation of bone tissue occurs with Paget's disease and can cause bone to weaken, resulting in bone pain, arthritis, deformities, and fractures.
Mentioned in: Bone X Rays, Fractures
References in periodicals archive ?
However, rarely, cases are reported from Eastern Asia including countries such as Taiwan, Japan, and Korea.[sup][4],[5] Pelvis and lumbar spine are the most commonly affected sites in both polyostotic and monostotic variety.[sup][6] Monostotic variety can be asymptomatic for a long duration before the first symptom breaks out.[sup][7] Symptoms of monostotic vertebral Paget's disease may be due to the fragile bone itself or occur as a result of compression to the surrounding structures caused by the expanding bone.[sup][7] In case of vertebral Paget's, neurologic dysfunction is the most debilitating presentation.
Lorette, "Associated Paget's disease of the vulva and of the nipple in the same patient: pathology with ultrastructural study," Annales de Dermatologie et de Venereologie, vol.
(9,10) This series represents one of the longest follow-up periods in a relatively large group of patients with Paget's disease who underwent cementless THA.
Bisphosphonate therapy is now the most commonly used treatment for Paget's disease, often normalizing biochemical markers of the bone turnover for prolonged periods.
Now, the Paget's Association - the only charity in the UK dedicated to Paget's disease - has named the Newcastle Bone Clinic as a UK Centre of Excellence.
Anjioid streaks and disciform macular detachment in Paget's Diseases. Am J Ophthalmol.
extramammary Paget's disease: mimicry in epidermotropic carcinoma.
RHEUMATOLOGISTS at Middlesbrough's James Cook University Hospital are urging people to be more aware of Paget's disease - a common bone disorder which affects hundreds of people without many of them knowing about it.
Dela Cruz, who was widowed last year and has three kids to support, was diagnosed with Paget's disease of the breast, which is a rare type of cancer involving the skin.
Neurotologists, otolaryngologists, and skull base surgeons from the US outline a practical approach to the clinical management of skull base tumors and lesions, beginning with surgical approaches described from the neurotologist's and neurosurgeon's perspectives, then minimally invasive and endoscopic skull base approaches; specific tumors and approaches, including acoustic neuroma, meningioma, neurofibromatosis, petrous apex lesions, and petroclival tumors; temporal bone malignancies; unusual temporal bone lesions, such as Paget's disease, fibrous dysplasia, and osteopetrosis; stereotactic radiotherapy for various types of skull base pathology; and the importance, benefit, and interpretation of intraoperative monitoring.
Immunohistochemistry helps to distinguish the entities: the tumor cells of Bowen disease are p63 positive, those of Paget's disease react for p-CEA, Ber-EP4, CK7, and CAM5.2, while melanoma cells are positive for S-100 and Melan-A [20].