Erasmus syndrome: A rare case report

Abstract

Erasmus syndrome is a rare clinical entity in which systemic sclerosis develops following exposure to silica with or without development of silicosis. There are very few cases reported in literature from India. Here we report a case of Erasmus syndrome in a 52 years male who is a stone crusher by occupation for last 22 years presented with dry cough, low grade irregular fever, progressive shortness of breath, slowly progressive sclerodactyly and features suggestive of Raynaud’s phenomenon. Radiological evaluations revealed multiple subpleural and centrilobular nodules in bilateral upper, lower lobes and right middle lobe, conglomerated nodular opacities (progressive massive fibrosis- PMF). There was pleural thickening and calcification, bronchial dilatation and fibrosis, calcified right hilar and mediastinal lymphadenopathy. Serological markers like Anti Scl-70 antibody came out to be positive (3+) Based on clinical features diagnosis of systemic sclerosis was made. Based on exposure history and radiology silicosis was diagnosed. Association of silicosis and systemic sclerosis establishes the diagnosis of Erasmus syndrome. Treatment was started with Prednisone, cyclophosphamide and Nifedipine. Strict avoidance to cold and regular follow-up was advised.