ERDHEIM-CHESTER-DISEASE WITH PROMINENT PULMONARY INVOLVEMENT ASSOCIATED WITH EOSINOPHILIC GRANULOMA OF MANDIBULAR BONE

We report a patient with eosinophilic granuloma localized to the left mandible who was subsequently shown to have Erdheim-Chester disease in volving the lower extremities, omentum and lung, The diagnosis of eosi nophilic granuloma was based on the presence of typical CD1a(+) Langer hans' cell granulomas in a biopsy of mandible, The diagnosis of Erdhei m-Chester disease was established on the basis of the pattern of radio isotopic uptake by long bones, seen on a technetium bone scan, and the presence of characteristic histopathological features in biopsies of lung and peritoneum. The pathological findings in lung were compatible with the abnormalities observed by tomodensitometry, but strikingly d ifferent from those seen in Langerhans' cell granulomatosis, The diffe rences in the histological features of pulmonary involvement seen in t he two diseases, and the possible relationship between Langerhans' cel l granulomatosis and Erdheim-Chester disease, are discussed.