A case of phosphoglyceride crystal deposition disease in the maxilla

Phosphoglyceride crystal deposition disease (PCDD) is a rare disorder in which phosphoglyceride crystals accumulate in soft tissues and bones. It tends to occur years after surgery, trauma, or repeated injections.

An 81-year-old woman was referred to our department because of swelling of the left maxillary gingiva. The left maxillary second molar had been extracted more than 10 years earlier. Surgical biopsy was performed, and histopathological findings indicated a foreign body granuloma. The patient underwent tumorectomy, during which we found a yellowish tumor. The pathologic findings were the characteristic crystal deposition, fibril-like crystals, and giant cells around the crystals. Gold hydroxamic acid staining revealed positivity for the crystals. The final pathological diagnosis was PCDD. The patient had no further symptoms and no disease recurrence.

It is relatively easy to diagnose PCDD from the characteristic histopathological findings; however, it may be overlooked by pathologists who are unaware of the disease. T2-weighted magnetic resonance imaging of PCDD in the jawbone has depicted low intensity, a finding that differs from those of ordinary cancers and odontogenic tumors. The oral cavity often undergoes surgical procedures, and PCDD may form, and grow.