Erasmus Syndrome, An Autoimmunity Paradox: A Case Report and Literature Review

Erasmus syndrome (ES) is a rare condition characterized by the link between crystalline silica exposure, with or without silicosis, and systemic sclerosis (SSc). Although first noted over a century ago, its underlying mechanisms remain unclear. However, it is indistinguishable from idiopathic SSc in the general population. Its clinical presentation is heterogeneous, depending on the affected systems, with notable features, including skin fibrosis, microstomia, telangiectasia, Raynaud's phenomenon, arthralgia, and interstitial lung disease. Currently, there is no unified consensus on its treatment; however, organ-specific therapy is a reasonable approach. We report the case of a 43-year-old miner diagnosed with diffuse cutaneous SSc, where ES was diagnosed after an exhaustive history was taken, occupational exposure was characterized, differential diagnoses were excluded, and radiological and histopathological evidence of pulmonary silicosis was presented.