Erasmus Syndrome: Co-existence of silicosis and progressive systemic sclerosis in a 55 year old male

Abstract

The Erasmus syndrome describes the association of generalised progressive scleroderma following exposure to silica with or without silicosis. We report a case of Erasmus Syndrome in a 55-year old labourer presenting with shortness of breath, arthralgia, Raynaud’s phenomenon, skin tightening and microstomia along with pulmonary arterial hypertension. Among investigations, serological markers were strongly positive, high-resolution computed tomography chest showed evidence of interstitial lung disease (ILD) with mediastinal lymphadenopathy and histopathology of skin biopsy were suggestive of systemic sclerosis. Correlating the occupational history, clinical features, haematological investigations and histological findings, a diagnosis of Erasmus syndrome was safely made.