Bone
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, PathologyOutlines.com, Inc.

PubMed Search: Fibrodysplasia ossificans progressiva[title]
Cite this page: Fibrodysplasia ossificans progressiva. PathologyOutlines.com website. http://www.pathologyoutlines.com/topic/boneFOP.html. Accessed December 4th, 2016.
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