Cellular and morphological aspects of fibrodysplasia ossificans progressiva. Lessons of formation, repair, and bone bioengineering

Abstract

Fibrodysplasia ossificans progressiva (FOP) is a rare congenital disease that causes bone formation within the muscles, tendons, ligaments and connective tissues. There is no cure for this disorder and only treatment of the symptoms is available. The purpose of this study was to review the literature and describe the clinical, cellular and molecular aspects of FOP. The material used for the study was obtained by reviewing scientific articles published in various literature-indexed databases. In view of its rarity and of the lack of insightful information and the unpredictability of its course, FOP is a challenging disorder for professionals who are confronted by it. However, this rare disease raises a great deal of interest because understanding the mechanism of mature bone formation can encourage research lines related to bone regeneration and the prevention of heterotopic ossification.

Keywords: cell differentiation; fibrodysplasia; heterotopic ossification; histomorphology; osteogenesis.

Figure 1.

Images of a patient with FOP. (A) Ossification nodule at the scalp level; (B) photograph of the back, with multiple erythematous papules; (C) biopsy of the skin on the back: aspects of ossification in the dermis around sweat glands. Reprinted with permission from reference 52.

Figure 2.

BMP signaling and negative regulation in bone formation. Reprinted with permission from reference 47.