When Silica Returns-Erasmus Syndrome, a Rare Entity

Systemic sclerosis (SS) is an autoimmune disease with vascular changes and diffuse tissue fibrosis. Silica exposure is one of the strongest recognized risk factors for the development of systemic sclerosis (relative risk 3.2). The co-occurrence of systemic sclerosis in a patient with silica exposure is termed Erasmus Syndrome. Few case reports are available in the literature.

We report here a case of Erasmus syndrome in a 54-year-old stone worker. The patient presented with diffuse skin thickening, hyperpigmentation, Raynaud's phenomenon, and arthralgia along with radiological features of silicosis. Serological markers of systemic sclerosis were strongly positive, and skin biopsy confirmed scleroderma. Hence, a diagnosis of Erasmus syndrome was made. The clinical, serological, and histopathological features of SA-SS in the patient were indistinguishable from those of idiopathic SS; however, a history of silica dust exposure and evidence of silicosis supported the diagnosis of SA-SS.

Careful screening should be done in patients with silicosis along with systemic manifestations to rule out any associated connective tissue disorder. A detailed exposure history should be carefully obtained for all patients with systemic sclerosis.