[Head and neck lesions caused by systemic progressive ossifying fibrodysplasia (Munchmeyer's disease]

Fibrodysplasia ossificans progressiva is a rare hereditary connective tissue disorder due to an unknown mutation conditioning the ossification of striated muscle and soft tissues frequently related to joints and stimulated by traumatisms or aggressive iatrogenic manouvres. It produces stiffness, pain, disability, swelling and fibro-osseus masses disseminated everywhere, with a very spectacular, invalidant and irreversible clinical picture. Affection of head and neck is unusual, excepting lesions in sternocleidomastoid or masticatiry muscles. We show a systemic case of fibrodysplasia osificans progressiva with enhanced clinical and radiological findings in cervical rhachis, pharynx and larynx, oral cavity, facial bones and ear. Association of biphophonates and steroids for a long period of time seems up to now stopping the evolution but not its regression.