Bone
Developmental abnormalities
Fibrodysplasia ossificans progressiva



Topic Completed: 1 June 2012

Revised: 26 December 2018

Copyright: (c) 2003-2018, PathologyOutlines.com, Inc.

PubMed Search: Fibrodysplasia ossificans progressiva[title]


Dariusz Borys, M.D.
Page views in 2018: 456
Page views in 2019 to date: 260
Cite this page: Borys D Fibrodysplasia ossificans progressiva. PathologyOutlines.com website. http://www.pathologyoutlines.com/topic/boneFOP.html. Accessed June 18th, 2019.
Definition / general
  • Rare autosomal dominant disorder with congenital malformation of the great toes and progressive heterotopic ossification in defined anatomic patterns
  • Early preosseous lesions resemble aggressive juvenile fibromatosis

    Pathophysiology:
  • Spontaneous and post-traumatic flareups heralded by intense connective tissue edema with perivascular lymphocytic infiltration into skeletal muscle
  • Angiogenic fibroproliferative lesions that spread along muscle planes and evolve through endochondral ossification to form mature lamellar bone
  • Leads to immobilization of joints making movement impossible, later death due to starvation (ankylosis of jaw) or from restrictive disease of chest wall
  • May be mediated by mast cells (Hum Pathol 2001;32:842)
Treatment
  • None; surgical trauma induces further bone formation
Gross images

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FOP Skeleton

Additional references
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