Myositis Ossifikans, Progresiva, Munchmeyer Hastalığı

Fibrodisplasia (myositis) ossificans progressiva nadir görülen herediter bir bağ dokusu hastalığıdır. Hastalık iskelet kaslarının bağ dokusu içerisinde başlangıçta ağrılı olan, progresif ektopik ossifikasyonlar ve simetrik iskelet malformasyonları ile karakterizedir. Hastalıkta ilk belirtiler çocukluk yaş gurubunda başlar. Nadir, otozomal dominant geçişli konjenital bir hastalıktır. Halen etkin bir tedavisi yoktur. Bu yazımızda büyük eklemlerinde hareket kısıtlılığı ile kliniğimize başvuran boyun, sırt, omuzlar, kalçalar ve dizlerde kas içerisinde ektopik ossifikasyonları ve başparmaklarında kısalığı bulunan ekstensif tutulumlu 23 yaşındaki bir erkek hastayı literatür bilgileri ışığında sunduk.

Anahtar Kelimeler:

Myositis ossifikans, Erişkin, Tanı

Myositis Ossificans Progressiva, Munchmeyer' Disease

Fibrodysplasia ossificans progressiva is a rare hereditary connective tissue disorder. Patients with fibrodysplasia ossificans progressiva develop progressive ossification of muscle and connective tissue associated with pain and disability. Onset is typically in childhood, and congenital feet anomalies are the early signs of this condition. Pain and stiffness of the spine or an inflammatory mass are common presenting features of fibrodysplasia ossificans progressiva. Studies on twins and families suggest that fibrodysplasia ossificans progressiva is a genetically inherited autosomal dominant trait with complete penetrance but variable expressivity. Unfortunately, effective therapy is unavailable. We present a 23 years old man with fibrodysplasia ossificans progressiva with a review of the literature on the clinical, radiographic, and genetic manifestations of this disorder.

Keywords:

Myositis ossificans, Adult, Diagnosis,

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