J Rheum Dis.  2016 Jun;23(3):193-197. 10.4078/jrd.2016.23.3.193.

Successful Treatment of Newly Developed, Intractable Digital Ulcers and Gangrene with Bosentan in Systemic Sclerosis

• Tag HS
• Jun SM
• Lee SG
• Park EK
• Koo DW
• Kim GT

• KMID: 2326736
• DOI: http://doi.org/10.4078/jrd.2016.23.3.193

Abstract

In systemic sclerosis, digital ulcers and gangrene are somewhat common clinical characteristics of obliterative vasculopathy. These manifestations increase morbidities, such as pain, infections, and acroosteolysis. However, patient responses to the appropriate treatments are often inadequate. We treated a patient with systemic sclerosis who had a refractory digital ulcer and gangrene with bosentan, an endothelin receptor antagonist, and observed improvement. Here we systematically review this case.

Keyword

Bosentan; Systemic scleroderma; Gangrene

MeSH Terms

Acro-Osteolysis
Gangrene*
Humans
Receptors, Endothelin
Scleroderma, Systemic*
Ulcer*
Receptors, Endothelin

Figure

• 

  Figure 1. Clinical course of digital ulcer. (A) An ulcer with pain had developed on the tip of her right middle finger by her first visit.(B) Six weeks after the first visit, despite using beraprost, the ulcer had worsened. Her treatment was switched to intravenous alprostadil. (C) Ten weeks after the first visit, the fingertip ulcer had deteriorated to gangrene (red arrow), despite the use of alprostadil for four weeks. Moreover, a new ulcer on the lateral side of the finger was noted (arrowhead). At this time, alprostadil was switched to bosentan. (D) Her digital ulcers and gangrene had improved after 10 weeks of bosentan treatment.

• 

  Figure 2. Three phase bone scan of both hands. (A, B) Blood pool activity and delayed 3-phase bone scan images at the time when the initial digital ulcer was noted. Localized increased blood pool activity was observed in the distal portion of the right third digit (A, dotted arrow). Minimally increased uptake was visible at the distal phalanx of the right third digit on the delayed image (B, arrow), which might have been caused by secondary hyperemia due to the soft tissue ulcer. (C, D) At the time of the 2-month follow-up 3-phase bone scan, the patient's symptoms were worsening. The localized blood pool activity of the right third digit was more intense (C, dotted arrow). At this time, prominent uptake was noted in the distal phalanx of the right third digit on the delayed image (D, arrow). This finding suggested the possibility of osteomyelitis of the distal phalanx of the right third digit.

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