Osteopoikilosis

Abstract

Osteopoikilosis is a rare inherited benign condition, usually a random finding in skeletal X-rays. Clinical manifestations are mild, patients are often asymptomatic or may complain of nonspecific bone pain. We present a case of a patient with a vague and painless thickening in the right inguinal area. MR depict multiple low-signal lesions in T1 and T2 in the pelvic bone, femoral head and necks, with MR characteristic of enostosis. X-ray of the pelvis with the two hip joints also confirmed the findings of Osteopoikilosis. Osteopoikilosis is a rare osteochondroplasias, a sclerosing bone dysplasia, characterized by numerous benign enostoses. The incidence is 1 out of 50 000. Osteopoikilosis is due to a mutation in the LEMD-3 gene. In about 25% of cases the Osteopoikilosis is accompanied with dermatologic abnormalities as closely located, whitish, fibrokolagen infiltrates (syndrome Buschke-Ollendorff). Bone metastases are consider in differential diagnosis. Osteopoikilosis does not require treatment, it is a so-called "Do not touch" skeletal lesions.