DIGITAL ULCERS AND MULTIPLE AMPUTATIONS IN A SYSTEMIC SCLEROSIS PATIENT

We are presenting the case of a 48 years old female diagnosed at the age of 28 with diffuse cutaneous systemic sclerosis. During the course of disease, despite the vasodilatator treatments and immunosuppresion for visceral involvement, the patient developed multiple infected digital ulcers both in the upper limbs and in the lower limbs, which complicated with wet gangrene, requiring transmetatarsal amputations. At that time, a macrovascular disease or a prothrombotic condition were excluded. Patient also developed infected nondigital lower extremity ulcers, which slowly healed after Alprostadil treatment. The evolution of our patient‘s disease demonstrate that the management of scleroderma vasculopathy represent a challange and a multidisciplinary approach is needed.

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Successful treatment of continuous intra-arterial administration of prostaglandin E1, urokinase and heparin for intractable digital ulcers by upper extremity arterial occlusion in diffuse cutaneous systemic sclerosis patient

The Journal of Dermatology ◽

10.1111/1346-8138.13935 ◽

2017 ◽

Vol 44 (10) ◽

pp. e254-e255 ◽

Cited By ~ 3

Author(s):

Shigeki Numata ◽

Yohei Iwata ◽

Masayoshi Kobayashi ◽

Toshimitsu Sato ◽

Kazumitsu Sugiura

Keyword(s):

Systemic Sclerosis ◽

Upper Extremity ◽

Successful Treatment ◽

Prostaglandin E1 ◽

Arterial Occlusion ◽

Digital Ulcers ◽

Diffuse Cutaneous Systemic Sclerosis ◽

Systemic Sclerosis Patient

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High D-dimer plasma concentration in systemic sclerosis patients: Prevalence and association with vascular complications

Journal of Scleroderma and Related Disorders ◽

10.1177/2397198320957558 ◽

2020 ◽

pp. 239719832095755

Author(s):

Sofia Furtado ◽

Bertrand Dunogué ◽

Georges Jourdi ◽

Benjamin Chaigne ◽

Aziza Chibah ◽

...

Keyword(s):

Systemic Sclerosis ◽

Plasma Concentration ◽

Microvascular Complications ◽

Vascular Complications ◽

Macrovascular Complications ◽

Digital Ulcers ◽

Tertiary Referral Center ◽

History Of ◽

Diffuse Cutaneous Systemic Sclerosis ◽

D Dimer

Objective: To determine the frequency of elevated D-dimer plasma concentration (>500 ng/mL) in patients with systemic sclerosis and evaluate its association with systemic sclerosis–specific microvascular and macrovascular complications. Methods: Retrospective observational study of patients with systemic sclerosis followed in a tertiary referral center with at least one measurement of D-dimer between 2010 and 2018. Results: A total of 214 patients were analyzed. Mean age at inclusion was 55.1 ± 14.7 years; 180 (84.1%) were female; 74 (34.6%) had diffuse cutaneous systemic sclerosis. Anti-Scl70 and anti-centromere antibodies were positive in 74 (34.6%) and 75 (35.0%) patients, respectively. D-dimer level was elevated in 93 (43.5%) patients, independently of cutaneous subtype (44.6% in diffuse cutaneous systemic sclerosis vs 42.9% in limited cutaneous systemic sclerosis, p = 0.81). At least one microvascular complication was found in 108 (50.5%) patients: 105 (49.1%) with previous or current digital ulcers, 6 (2.8%) with renal crisis, and 4 (1.9%) with pulmonary arterial hypertension. Microvascular complications were more frequent in patients with elevated D-dimer (57.0% vs 45.5%, p = 0.09), significantly so after exclusion of patients with a history of cancer and/or venous thromboembolism (60.5% vs 44.8%, p = 0.04). Macrovascular complications were detected in 15 (7.0%) patients and were associated with a high D-dimer level (11.8% vs 3.3%, p = 0.03). Over a median follow-up of 2.3 years [1.1–3.3] after D-dimer measurement, new macrovascular complications occurred only in patients with high D-dimer (n = 8). Conclusion: High D-dimer levels are frequently found in systemic sclerosis patients and seem to be associated with the occurrence of macrovascular and microvascular complications after adjustment for confounding factors.

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Successful Treatment with Bosentan of Lower Extremity Ulcers in a Scleroderma Patient

Case Reports in Medicine ◽

10.1155/2013/690591 ◽

2013 ◽

Vol 2013 ◽

pp. 1-3 ◽

Cited By ~ 1

Author(s):

Alix Naert ◽

Petra De Haes

Keyword(s):

Molecular Weight ◽

Systemic Sclerosis ◽

Lower Extremity ◽

Low Molecular Weight Heparin ◽

Local Treatment ◽

Low Molecular Weight ◽

Digital Ulcers ◽

Endothelin Receptor ◽

Vasodilator Therapy ◽

Scleroderma Patient

Digital ulcers are a well-known problem in patients with systemic sclerosis. Lower extremity ulcers are less prevalent but are also a challenging and underestimated complication of the disease causing important pain and morbidity. Bosentan, an oral dual endothelin receptor antagonist, has been shown to be effective in preventing digital ulcers in patients with systemic sclerosis. A few recent observations showed the efficacy of bosentan for accelerating the healing of nondigital ulcers in scleroderma patients. This report deals with a 48-year-old patient with systemic sclerosis who developed painful ulcers on the left ankle and hallux. The ulcers were refractory to a combination of vasodilator therapy with a calcium antagonist and several courses of intravenous prostanoids, low molecular weight heparin, aspirin, simvastatin, and intensive local treatment. Bosentan treatment showed spectacular healing of the ulcers already after 4 months of therapy. This case supports the previous few observations of accelerating wound healing of lower extremity ulcers in systemic sclerosis patients with bosentan treatment.

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Paradoxical Reaction of Raynaud Phenomenon following the Repeated Administration of Lloprost in a Patient with Diffuse Cutaneous Systemic Sclerosis

Annals of Pharmacotherapy ◽

10.1345/aph.1r093 ◽

2012 ◽

Vol 46 (10) ◽

pp. 1439-1439 ◽

Cited By ~ 6

Author(s):

Rebeca Iglesias Barreira ◽

Belén Bardán García ◽

Mónica Granero López ◽

Iria Rodríguez Legazpi ◽

Hortensia Álvarez Díaz ◽

...

Keyword(s):

Systemic Sclerosis ◽

Repeated Administration ◽

Vascular Involvement ◽

Digital Ulcers ◽

Probability Scale ◽

Paradoxical Reaction ◽

Raynaud Phenomenon ◽

Naranjo Probability Scale ◽

Case Summary ◽

Diffuse Cutaneous Systemic Sclerosis

Objective TO report a paradoxical reaction of Raynaud phenomenon following the repeated administration of iloprost in a patient with diffuse cutaneous systemic sclerosis with vascular involvement. Case Summary In January 2006, a 40-year-old male was diagnosed with diffuse cutaneous systemic sclerosis with pulmonary, esophageal, cutaneous, and vascular involvement (Raynaud phenomenon, with digital ulcers on his hands). In December 2008, treatment with iloprost was started due to worsening disease. Nine cycles of iloprost were administered at a rate of 0.5–1 ng/kg/min (6 hours per day, for 5 days every 6–8 weeks); the patient tolerated this treatment well. However, on the fourth day of cycles 10 and 11, the patient developed paradoxical Raynaud phenomenon in the hand with perfusion when the infusion was increased to 1 ng/kg/min, requiring treatment to be stopped. Treatment was continued during cycles 12 and 13 at 0.5 ng/kg/min; the patient tolerated the treatment well, although paradoxical Raynaud phenomenon occurred when the rate of infusion was increased. Discussion Raynaud phenomenon is extremely common in patients with scleroderma, and often is severe. Iloprost has vasodilating, antiplatelet, cytoprotective, and immunomodulating properties, and has been found to be an efficacious alternative to nifedipine for the treatment of Raynaud phenomenon in patients with scleroderma. The Naranjo probability scale indicated that iloprost was the probable cause of the paradoxical Raynaud phenomenon in this patient. Conclusions This case demonstrates a probable relationship between the rate of infusion of iloprost and the paradoxical reaction of Raynaud phenomenon.

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