[Erasmus' syndrome with pseudo-tumour masses].

Rev Mal Respir

Service de pneumologie, pavillon 2, hôpital A.-Mami, 2080 Ariana, Tunisie.

Published: September 2011

Introduction: Erasmus' syndrome involves the association of systemic scleroderma (SS) and exposure to silica. Silicosis may precede the SS but the latter may be the presentation, in which case a history of exposure to silica should be sought as part of the diagnosis.

Case Report: A 46-year-old man with history of pulmonary tuberculosis presented with dyspnoea and dysphagea. Clinical examination revealed thickening of the facial skin with a pointed nose, erythema and telangiectasia, Raynaud's syndrome and sclerodactyly. A thoracic CT scan revealed bilateral, fibrotic, pseudo-tumoural masses. Antinuclear antibodies, anti-topoisomerase 1 and antihistone were positive.

Conclusion: The clinical presentation of Erasmus' syndrome associating systemic scleroderma and pulmonary pseudo-tumours may pose a problem of differential diagnosis from lung cancer. This condition requires regular clinical and radiological monitoring, particularly as both scleroderma and silicosis increase the risk of lung cancer.

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http://dx.doi.org/10.1016/j.rmr.2011.06.003DOI Listing

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