Osteopetrosis (Albers-Schönberg disease, marble bones, osteosclerosis fragilis generalisata)

Case of osteopetrosis with multiple impacted primary and permanent teeth diagnosed at eight years old

“…Osteopetrosis, an inherited skeletal disorder initially reported by a German radiologist in 1904 [1], is characterized by increased bone density due to a defect in remodeling caused by malfunction of osteoclasts [2]. Based on its inheritance form and clinical severity, osteopetrosis is classified into three distinct types; (1) severe/malignant infantile type with autosomal recessive inheritance, (2) intermediate type with dominant or autosomal recessive inheritance, and (3) mild/late onset/adult type with autosomal dominant inheritance [3,4]. As for the main systemic manifestations, bone osteosclerosis develops, resulting in a short stature and likelihood of bone fractures [5].…”

Socket Sclerosis—An Obstacle for Orthodontic Space Closure?

“…It can occur in different degrees of severity, ranging from the congenital systemic form called osteopetrosis (Albers-Schoenberg disease, marble-bone disease, osteosclerosis fragilis generalisata) to the very benign form of idiopathic sclerosis of the jawbone. [1][2][3][4][5][6] Localized sclerosis of the jawbone presents as a round radiopacity most commonly found in the region of the dental periapex, and it is considered a lesion that is usually formed in direct response to localized chronic inflammation. 7,8 It is called condensing osteitis, focal sclerosing osteomyletis, or focal periapical osteopetrosis.…”