Abstract
Introduction: The Erasmus syndrome is characterized by the association of generalized systemic sclerosis (SSc) and silica exposure with or without silicosis. It was initially described in 1914 by Bramwell and later in 1957 by Erasmus. We present a case who had worked as a stone cutter for over 20 years. Case Report: A-54-year-old male patient visited with chief complaints of tightness and darkness of skin and painful bluish discolouration of fingers. The patient was asymptomatic three months back when he started to develop tightness and darkening of the skin, which started over the dorsum of the right and left hand, fingers and symmetrically progressed to involve the forearms, arms, trunk, legs, and face. Raynaud phenomenon was present. The patient had dyspnea on exertion for the past 8-9 months and decreased mouth opening for two months. Autoimmune workup showed ANA positivity (homogenous and nucleolar pattern) and Scl-70 antibodies. Pulmonary function tests showed mild restrictive lung disease. No additional visceral involvement noted. Skin biopsy showed thickened dermis with broad sclerotic collagen bundles, consistent with cutaneous changes of SSc. The patient was asked to quit his current job, and medication was started. Discussion: The pathogenesis of SSc involves an interplay of three factors- autoantibodies production, small vessel angiopathy and increased extracellular matrix deposition due to fibroblast dysfunction. Occupational exposure to several agents such as crystalline silica, benzene, bleomycin, and epoxy resins can induce SSc. Silica-associated SSc has severe pulmonary involvement, increased prevalence of anti-Scl-70 antibodies and poorer prognosis when compared to idiopathic SSc. We should take occupational history in patients presenting with skin tightening and discolouration and do an autoimmune workup for SSc.
Financial Disclosure:
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