It took two seasoned rheumatologists in the UK to spot the disorder behind this salt-and-pepper skin.

A 68-year-old man presented to their clinic with a five-year history of skin changes on his back and chest, according to the case report in the New England Journal of Medicine.

A smoker, the man had been hospitalised for dyspnoea three months earlier, where a chest CT had shown fibrotic and emphysematous changes in the lower lobes of the lungs and a transthoracic echocardiography had demonstrated elevated pulmonary artery systolic pressure.

The rheumatologists noted “clubbing of the fingers, crackles in both lung bases, salt-and-pepper skin changes on the chest and back, characterised by hypopigmentation and hyperpigmentation in areas of sclerotic skin and diffuse skin thickening that extended down each arm to a point proximal to the elbows”.