Developmental abnormalities
Fibrodysplasia ossificans progressiva

Author: Dariusz Borys, M.D. (see Authors page)

Revised: 14 September 2016, last major update June 2012

Copyright: (c) 2003-2016,, Inc.

PubMed Search: Fibrodysplasia ossificans progressiva[title]
Cite this page: Fibrodysplasia ossificans progressiva. website. Accessed September 25th, 2018.
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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Missing Image

FOP Skeleton

Additional references