[Involvement of the facial skull in Erdheim-Chester disease]

We report on a patient suffering from Erdheim-Chester-disease (ECD). ECD represents a very rare entity with lipogranulomatosis of mesenchymal origin. The most common radiological manifestation is the involvement of the long bones of the extremities. Here we find sclerosis of the spongiosa combined with a thinning of cortical structures. This often results in a small crack of hyperlucency between corticals and spongiosa. Our case demonstrates an involvement of the craniofacial part of the skull showing sclerosis of the upper jaw bone. This manifestation has not yet been reported in the literature.