Although pulmonary involvement in Niemann-Pick disease is common, the appearance of interstitial lung disease in adulthood is rare. The differential diagnosis of adult-type Niemann-Pick disease includes pulmonary histiocytic disorders. We reported a 21-year-old man with a family history of Niemann-Pick disease type B and hepatosplenomegaly and a miliary appearance on chest X-ray suspicious of tuberculosis. The clinical, laboratory and radiologic data of our case were discussed.
Key words: Niemann-Pick, histiocytic disorders, lung, lipoid pneumonia
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