The patient
This 65-year-old warehouseman presented with an ulcer on his knuckle that had been present for weeks. His history included osteoarthritis of his knees and hips, for which he had taken NSAIDs. But these were stopped a year ago when he had an endoscopic diagnosis of GORD. His dysphagia was still troublesome. The only other recent entry in his notes was from six months ago, when he said his hands went blue and painful when cold or wet – in the absence of any other symptoms at the time, I'd made a diagnosis of Raynaud's disease and encouraged him to stop smoking.
First instinct
At first glance, this looked like a traumatic ulcer – perhaps a burn, possibly from the cigarettes he continued to smoke. There were no similar ulcers elsewhere, and he seemed otherwise well, although he did mention his cold and painful hands again. On examination, they certainly were cool and the skin seemed odd – coarse to the touch, while looking shiny.
Differential diagnosis
- Trauma
- Worsening Raynaud's with a probable underlying disease
- Malignancy
- Keratoacanthoma
- Orf.
It did occur to me that his Raynaud's could have been implicated – severe Raynaud's can certainly cause trophic changes, in which case you'd suspect some underlying connective tissue disease. But while I've seen this pathology result in digital ischaemia, I've never known it to cause an ulcer of this sort.
Malignancy is always a possibility with any non-healing skin ulcer in the elderly – but the appearance and situation certainly weren't typical. A keratoacanthoma was worth considering, though – the rapid onset, sun-exposed site and keratin-filled core were all compatible with this diagnosis.
Orf was a long shot – I've seen a couple of cases previously and they looked similar. But it seemed unlikely that he would have had significant contact with sheep.
The hidden clue
The penny really should have dropped six months ago when he first presented with Raynaud's disease. After all, primary Raynaud's usually occurs in the teens or early 20s – a later presentation makes an underlying cause very likely. This only occurred to me as I pondered this odd, slow-to-heal ulcer and the strange appearance and feel of his skin. And it started to make sense – it looked like he had scleroderma.
Getting on the right track
I referred him on, and the dermatologist confirmed the diagnosis. The letter highlighted telangiectasia on his hands too, though no obvious calcinosis – and noted that his oesophageal problems might have been part of the same underlying problem. The dermatologist felt this pointed to the CREST form of scleroderma.
Dr Keith Hopcroft is GP in Laindon, Essex
