Engelmann's Disease

The condition which is now known as Engelmann's disease, is one of the rarest of the bone dystrophies causing osteosclerosis. Its aetiology is unknown, and the usual investigations into blood chemistry fail to give any indication of the pathological processes that are taking place. A female patient first came under observation in early 1950; she is still alive and is now aged 34. During the course of investigations into the cause of her “pains in the limbs” of which she was complaining, radiographs were taken which revealed changes in her bones characteristic of Engelmann's disease. All the long bones of both the upper and lower extremities were affected. The floor of the anterior fossa of the skull, the frontal bones, and the bones of the pelvis were also affected, but to a lesser degree. The bones of the hands, feet, the whole of the vertebral column, the clavicles and the thoracic cage showed normal bone structure. The changes were most marked in the femora, practically the whole of the bone being involved; only the condyles and a small part of the shaft above them could be regarded as showing normal bone structure. In the cases so far reported in the literature, involvement to this extent in any one of the long bones has not been seen. The changes have been confined, in most cases, to the middle half or have extended up to, but not beyond, the metaphysis of the bone.