Keywords :
Case report, Erasmus syndrome, Interstitial, Salt and pepper rash, Sclerodactyly, Silicosis, Systemic sclerosis
Citation Information :
Dey S, Siddhanta S, Karmakar K, Mukhoti K, Roy D, Sarkar N. Erasmus Syndrome—A Rare Cause of Diffuse Cutaneous Systemic Sclerosis due to Silica Exposure: A Case Report. Bengal Physician Journal 2025; 12 (1):46-48.
Erasmus syndrome is a rare disease characterized by the development of systemic sclerosis (SSc) in the background of silica exposure or silicosis. Here we report a case of a 42-year-old male who presented with skin tightening for 2 years and progressive dyspnea for 1 year. Patient worked as a sandblaster for 6 months. On examination, there was sclerodactyly, fixed flexion deformity of the lower limbs, diffuse skin tightening and salt and pepper rashes on the chest wall and upper back and crepitations on the chest auscultation. Chest imaging and pulmonary function tests were suggestive of interstitial lung disease. The autoimmune profile showed Scl-70 +++. The diagnosis of erasmus syndrome was made based on specific clinical presentations and characteristic chest imaging findings in a male patient with a history of silica exposure.
Barnes H, Goh NSL, Leong TL, et al. Silica-associated lung disease: An old-world exposure in modern industries. Respirology 2019;24(12):1165–1175. DOI: 10.1111/resp.13695.
Greenberg MI, Waksman J, Curtis J. Silicosis: A review. Dis Mon 2007;53(8):394–416. DOI: 10.1016/j.disamonth.2007.09.020.
Pollard KM. Silica, silicosis, and autoimmunity. Front Immunol 2016;7:97. DOI: 10.3389/fimmu.2016.00097.
Costantini L, Gilberti R, Knecht D. The phagocytosis and toxicity of amorphous silica. PLoS One 2011;6(2):e14647. DOI: 10.1371/journal.pone.0014647.
Leung C, Yu I, Chen W. Silicosis. Lancet 2012;379(9830):2008–2018. DOI: 10.1016/S0140-6736(12)60235-9.
Parks CG, Conrad K, Cooper GS. Occupational exposure to crystalline silica and autoimmune disease. Environ Health Perspect 1999;107(Suppl 5):793–802. DOI: 10.1289/ehp.99107s5793.
Miller FW, Alfredsson L, Costenbader KH, et al. Epidemiology of environmental exposures and human autoimmune diseases: Findings from a National Institute of Environmental Health Sciences Expert Panel Workshop. J Autoimmun 2012;39(4):259–271. DOI: 10.1016/j.jaut.2012.05.002.
Qi XM, Luo Y, Song MY, et al. Pneumoconiosis: Current status and future prospects. Chin Med J (Engl) 2021;134(8):898–907. DOI: 10.1097/CM9.0000000000001461.
Go LHT, Cohen RA. Coal workers’ pneumoconiosis and other mining-related lung disease: new manifestations of illness in an age-old occupation. Clin Chest Med 2020;41(4):687–696. DOI: 10.1016/j.ccm.2020.08.002.
Lanzafame M, Vento S. Mini-review: Silico-tuberculosis. J ClinTuberc Other Mycobact Dis 2021;23:100218. DOI: 10.1016/j.jctube.2021.100218.
Ehrlich R, Akugizibwe P, Siegfried N, et al. The association between silica exposure, silicosis, and tuberculosis: A systematic review and meta-analysis. BMC Public Health 2021;21(1):953. DOI: 10.1186/s12889-021-10711-1
Sharma N, Kundu D, Dhaked S, et al. Silicosis and silico tuberculosis in India. Bull World Health Organ 2016;94(10):777–778. DOI: 10.2471/BLT.15.163550.
Erasmus LD. Scleroderma in gold miners on the Witwatersrand with particular reference to pulmonary manifestations. S Afr J Lab Clin Med 1957;3(3):209–231. PMID: 13495613.
Rodnan GP, Benedek TG, Medsger TA, et al. The association of progressive systemic sclerosis (scleroderma) with coal miners’ pneumoconiosis and other forms of silicosis. Ann Intern Med 1967;66(2):323–334. DOI: 10.7326/0003-4819-66-2-323.
Varga J. Systemic sclerosis (scleroderma) and related disorders: Harrison's Principles of Internal Medicine. US: McGraw-Hill Education. 1998. pp. 2546–2560.
Denton C. Overview of the treatment and prognosis of systemic sclerosis (Scleroderma) in adults. UpToDate. 2021. [online]. Available from: https://www.uptodate.com/contents/overview-of-the-treatment-and-prognosis-of-systemic-sclerosis-scleroderma-in-adults. [Last accessed November, 2024].
Rustin MH, Bull HA, Ziegler V, et al. Silica-associated systemic sclerosis is clinically, serologically and immunologically indistinguishable from idiopathic systemic sclerosis. Br J Dermatol 1990;123(6):725–734. DOI: 10.1111/j.1365-2133.1990.tb04189.x.
