Tophaceous calcium pyrophosphate dihydrate deposition disease of the temporomandibular joint

Tophaceous pseudogout is a rare manifestation of calcium pyrophosphate dihydrate (CPPD) deposition disease that particularly affects the temporomandibular joint (TMJ). We describe a case of tophaceous pseudogout and review the literature. Thirty-four cases of chronic CPPD deposition disease affecting the TMJ are described. Symptoms usually included pain and swelling. Most patients required surgery because of extensive crystal deposits, usually localized to the joint and adjacent structures but occasionally invasive. For many patients, malignancy was the preoperative diagnosis. Although patients with acute pseudogout of the TMJ may have involvement of other joints, tophaceous pseudogout was predominantly isolated to the TMJ.