Lymphangiomatosis with dental involvement.

Abstract

Lymphangiomatosis characterized by lymphangiomatous proliferation is a rare disease of unknown etiology, which seems to be more aggressive than Gorham-Stout Syndrome, also known as "the vanished bone disease." A woman presented with a lytic lesion in the tibia and multiple lytic lesions in teeth dentin. Upon autopsy, a single lytic bone lesion and fulminant lymphangiomatous proliferation were found in the pleura, pericardium, and peritoneum. The histological findings from a tooth lytic lesion included fibroblastic proliferation interspersed with vascular spaces embedded with fragments of lamellar bone and dentin containing osteoclast like multinucleated giant-cell lined resorption bays. The histological findings support the notion that hard tissue resorption is conducted by lymphangiomatosis and osteoclastic activity.