Bone & joints

Developmental abnormalities

Fibrodysplasia ossificans progressiva

Last author update: 1 June 2012
Last staff update: 10 January 2022

Copyright: 2003-2023,, Inc.

PubMed Search: Fibrodysplasia ossificans progressiva[title]

Dariusz Borys, M.D.
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Cite this page: Borys D. Fibrodysplasia ossificans progressiva. website. Accessed June 1st, 2023.
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

  • 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)
  • None; surgical trauma induces further bone formation
Gross images

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FOP Skeleton

Additional references
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