A Case of Skin Thickening with Occupational Lung Disease

Sir,

A 35-year-old male patient, stone crusher for 10 years, presented with history of cough, generalized body pain, and progressive respiratory distress for 1 year. There was history of intermittent nondocumented low-grade fever and generalized hyperpigmentation was present. On examination, there was skin thickening with salt and pepper pigmentation [Figure 1a, 1b and 1c]. On probing, he also had history suggestive of Raynaud's phenomenon and gastroesophageal reflux disease. Hence, a provisional diagnosis of scleroderma was made which was confirmed by rheumatologist. Examination revealed bilateral Velcro crackles and spirometry was suggestive of severe restriction. He underwent a CT Chest [Figure 2a and 2b] followed by trans-bronchial lung biopsy (TBLB) [Figure 2c, 2d]. The patient was strongly positive for scl-70 antibody.

Figure 1.

(a) Clinical photograph of face showing binding down of skin with pinched nose and thin, pursed lips. (b) Clinical photograph of hands showing sclerodactyly (digital skin tightening), puffiness, and distal resorption of digits causing pseudo clubbing. Third digit of right hand also shows a digital tip ulcer healing with a scar. (c) Clinical photograph of right foot showing binding down of skin and early salt and pepper pigmentation

Figure 2.

(a) CT chest lung window showing bilateral perilymphatic nodules, few of them showing coalescence with interlobular septal thickening and subpleural cysts in bilateral lungs.(b) CT chest mediastinal window showing calcified right paratracheal (4R station) lymph node and subpleural areas of fibrosis in the right lung. (c) Hematoxylin and Eosin stain of the transbronchial lung biopsy showing Pink strands of fibrosis in 400 × magnification. (d) Polarized light microscopy showing crystal in 400 × magnification

Thus, the final diagnosis of Erasmus Syndrome was made. There was history of silica exposure for 10 years associated with scleroderma confirming Erasmus syndrome. The TBLB showed features of fibrosis with crystalline changes with birefringence suggestive of silicosis.

A recent review article and a meta-analysis document occupational exposure of silica as a risk factor for Systemic sclerosis.[1,2] Erasmus syndrome is systemic sclerosis with silica exposure with or without silicosis.[3] Originally described in gold miners of South Africa,[4] it was first reported from India in 1997.[5] Since then, there are few case reports from India, one having coexisting pulmonary tuberculosis.[6,7,8]

The mechanism is unclear; adjuvant effect of silica particles on antibody production and depressed cellular immunity have been postulated to cause autoimmune reaction. Silica increases lymphokine production by pulmonary macrophages causing collagen production and chronic inflammation. The risk is more in males and miners.[9] Rest of the clinical features are similar to idiopathic systemic sclerosis, and the diagnosis is based on exposure history.[10]

Scant literature suggests treating systemic sclerosis and avoiding further silica exposure. Few cases report a favorable response to steroids, immunosuppressant, and calcium channel blockers.[6] Our patient showed response to oral steroids in terms of respiratory failure and cough.

KEY POINT-Erasmus syndrome is a rare clinical entity presenting like idiopathic systemic sclerosis. Hence, a high index of suspicion should be kept and detailed exposure history should be sought.

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Conflicts of interest

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