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Systemic sclerosis presenting as digital ulceration in an elderly female
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  1. Martin E Winstanley1,
  2. Amy C Carmichael2 and
  3. Ella Harrison-Hansley3
  1. 1Nevill Hall Hospital, Abergavenny, UK
  2. 2Hanham Health, Bristol, UK
  3. 3Prince Charles Hospital, Merthyr Tyddfil, UK
  1. Correspondence to Dr Martin E Winstanley; martin.winstanley{at}uhbw.nhs.uk

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Description

An 80-year-old female presented to the emergency department with a 3-week history of worsening digital discolouration. She explained that her right little finger was affected first, turning blue and then blistering at the tip (figure 1). Her left index finger tip subsequently turned black, and the changes were spreading proximally at the time of presentation (figure 2). For several years, she had noted digital changes consistent with Raynaud’s phenomenon, but her symptoms were manageable with lifestyle modification and she had not previously sought medical attention. On examination, her left index finger was dusky and cool to touch, and there was evidence of sclerodactyly and finger pad telangiectasia. She was admitted for inpatient investigation of her digital ulcers. Serology revealed anti-centromere antibodies, and echocardiography revealed pulmonary hypertension. These findings supported a diagnosis of limited systemic sclerosis. In general, patients with late-onset systemic sclerosis (over 75 years) suffer more frequently with the limited subtype, more frequently with pulmonary hypertension and less frequently with digital ulcers.1

Figure 1

Right hand palmar view.

Figure 2

Left hand dorsal view.

The digital ulcers seen in this case were ischaemic complications of systemic sclerosis. Microvascular complications of systemic sclerosis range from mild symptoms of Raynaud’s phenomenon, which are experienced by virtually all patients, to digital ulcers, irreversible tissue injury and gangrene.2 The National Institute for Health and Care Excellence advises immediate admission for severe digital ischaemia in the context of Raynaud’s phenomenon.3 In elderly patients, microvascular complications should raise suspicion of macrovascular involvement.4 Overall, ischaemic complications are the primary cause of disease-related morbidity in systemic sclerosis.2

Learning points

  • Raynaud’s phenomenon with digital ulceration suggests an underlying cause and warrants inpatient investigation.

  • Systemic sclerosis is associated with a variety of ischaemic complications, including Raynaud’s phenomenon, digital ulcers and macrovascular disease.

Ethics statements

Patient consent for publication

Acknowledgments

The authors are grateful to the patient for her permission to publish and learn from these images.

Unfortunately she has passed away and this piece is dedicated to her.

References

Footnotes

  • Contributors MEW examined and treated the patient. MEW and ACC wrote the manuscript. EHH photographed the patient and supported the publication process.

  • Funding The authors have not declared a specific grant for this research from any funding agency in the public, commercial or not-for-profit sectors.

  • Competing interests None declared.

  • Provenance and peer review Not commissioned; externally peer reviewed.

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