fibrodysplasia os·sif·i·cans pro·gres·si·va ( -s f -k nz pr gr-s v )n. An inherited generalized disorder of connective tissue in which bone replaces tendons, fasciae, and ligaments. |
fibrodysplasia ossificans progressiva.
fibrodysplasia ossificans progressiva
FOP, Generalized myositis ossificans, myositis ossificans progressiva, stone man An idiopathic or AD condition of irregular penetration and pre-pubertal onset, in which connective/interstitial tissues
undergo extensive fibrosis and heterotopic ossification of ligaments, tendons, muscle, fascia, aponeuroses, skin, first seen in late childhood as firm masses Clinical Microdactyly, focal, transient, ±painful ossifying tumors in neck, back and
extremities, with bony replacement of fasciae, ligaments and fibrotendinous tissue; also baldness, deafness, mental retardation, fever; tragically, Pts may find jobs in circus 'freak' shows; usually glossal, diaphragmatic, laryngeal, and
perineal muscles are spared and Pts die of respiratory infections as intercostal muscles become petrified DiffDx Osseous metaplasia, myositis ossificans, extraskeletal osteosarcoma. Cf Parana hard skin syndrome, Stiff man syndrome.
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