PULMONARY PUZZLE
Pulmonary puzzle
| The first 150 words of the full text of this article appear below. |
ANSWER
From the question on page 492.
Macroscopic findings favoured a diagnosis of mesothelioma. Microscopy revealed instead that the pleural thickening comprised fibrosis within which abundant CD68-positive histiocytes and Touton-type giant cells were present, these staining with S-100 but not CD1a. There was also microscopic involvement of the lung along the distribution of the lymphatic vessels and areas of yellow firm thickening in the epicardial fat adjacent to the coronary arteries demonstrating identical pathology. A diagnosis of Erdheim-Chester disease (ECD) was made.
ECD is a non-Langerhans histiocytic disorder primarily affecting bone. Over 50% of cases show involvement of other tissues including the retroperitoneum, retro-orbital tissues, heart, pituitary-hypothalamic axis and lung.1 Diagnosis is usually based on long bone radiographic changes.1–4 Bone scanning reveals symmetrical and abnormally increased distal lower limb long bone uptake. Pulmonary CT findings include distinctive interlobular and visceral pleural thickening associated with patchy reticular and centrilobular opacities and
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Thorax 2008 63: 492.[Extract] [Full Text] [PDF]
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