Polyostotic fibrous dysplasia

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Polyostotic fibrous dysplasia
Classification and external resources
ICD-10 Q78.1
ICD-9 756.54
DiseasesDB 7880
MeSH D005359

Polyostotic fibrous dysplasia (also known as "Albright's disease"[1]:578) is a form of fibrous dysplasia affecting more than one bone.[2]

McCune-Albright syndrome includes polyostotic fibrous dysplasia as part of its presentation.Polyostotic fibrous dysplasia (PFD) is a sporadic disorder which affects multiple sites in the skeleton. The bone at these sites is rapidly resorbed and replaced by abnormal fibrous tissue or mechanically abnormal bone. PFD may occur alone or as part of the McCune-Albright Syndrome (MAS), a syndrome originally defined by the triad of PFD, cafe-au-lait pigmentation of the skin, and precocious puberty. The bony lesions are frequently disfiguring and painful, and depending on the location of the lesion, can cause significant morbidity. Lesions in weight-bearing bones can lead to disabling fractures, while lesions in the skull can lead to compression of vital structures such as cranial nerves.[3]

One treatment that has been used is bisphosphonates.[4]

See also[edit]

References[edit]

  1. ^ James, William; Berger, Timothy; Elston, Dirk (2005). Andrews' Diseases of the Skin: Clinical Dermatology. (10th ed.). Saunders. ISBN 0-7216-2921-0.
  2. ^ "Fibrous Dysplasia: Overview - eMedicine Radiology". Retrieved 2009-02-23. 
  3. ^ Lee, Peter A. (5 December 1986). "McCune-Albright Syndrome<subtitle>Long-term Follow-up</subtitle>". JAMA: The Journal of the American Medical Association 256 (21): 2980. doi:10.1001/jama.1986.03380210076028. 
  4. ^ Khadilkar VV, Khadilkar AV, Maskati GB (September 2003). "Oral bisphosphonates in polyostotic fibrous dysplasia". Indian Pediatr 40 (9): 894–6. PMID 14530553.