A 21-year-old man with cough and skin thickening
The present case has been reported in the journal Lung India. A 21-year-old male presented with complaints of dry cough and shortness of breath for 5 years. The patient also noticed diffuse depigmentation and hyperpigmentation of the skin for 3 years associated with tightness of skin involving the face, hands, and legs which progressed to involve proximal limbs and trunk for the last 1 year.

There was a history of Raynaud's phenomenon and difficulty in swallowing. He also complained of polyarthralgias involving the small joints of both hands for the last 1 year. There was no history of oral ulcers and proximal muscle weakness. He had been a lifetime never smoker. The patient had worked as stone cutter for the last 7 years and had significant exposure to silica dust. There was no past history of tuberculosis and no family history of autoimmune disease.

On examination, diffuse cutaneous thickening, sclerodactyly, and “salt and pepper” pigmentary changes were present. Chest examination revealed bilateral end-inspiratory fine crepitations. Computed tomography scan of the thorax demonstrated mediastinal lymphadenopathy with eggshell calcification and bilateral upper lobe fibrosis.

Pulmonary function test revealed a restrictive defect with forced vital capacity of 56% predicted. Six-minute walk distance was 430 m, and saturation dropped from 92% to 86% at the end of the test. Antinuclear antibody titers were positive.

In view of significant silica exposure, skin changes suggestive of systemic sclerosis, and radiological findings consistent with silicosis, the final diagnosis was Erasmus syndrome.

Read more about the case here: https://pxmd.co/Kbu2B
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Dr. D●●●●h S●●●●a
Dr. D●●●●h S●●●●a General Medicine
Erasmus syndrome is a rare entity in which systemic sclerosis develops following exposure to silica with or without silicosis
Aug 28, 2018Like