Bone & joints
Developmental abnormalities
Fibrodysplasia ossificans progressiva
Author: Dariusz Borys, M.D.
Last author update: 1 June 2012
Last staff update: 8 September 2023
Copyright: 2003-2024, PathologyOutlines.com, Inc.
PubMed Search: Fibrodysplasia ossificans progressiva[title]
Cite this page: Borys D. Fibrodysplasia ossificans progressiva. PathologyOutlines.com website. https://www.pathologyoutlines.com/topic/boneFOP.html. Accessed December 4th, 2024.
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
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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
Additional references
