Ulcers In Systemic Sclerosis Research Articles

Successful treatment with bosentan for refractory digital ulcers in a patient with systemic lupus erythematosus

We report a 32-year-old woman with a 12-year history of systemic lupus erythematosus. Physical examination revealed indurated plaques with small ulcers on her extremities and trunk, which were histologically diagnosed as lupus erythematosus profundus. On her arms and knees, multiple small calcified nodules were noted in the dermis and subcutis. An elevated level of anti-phosphatidylserine/prothrombin antibodies was noted. She had been suffering from digital ulcers on the left fourth finger. Despite conservative therapies, new ulcers appeared on other fingers. After the administration of bosentan, no new lesion has developed. Bosentan, recognized as a choice of treatments for digital ulcers in systemic sclerosis, is worth trying in systemic lupus erythematosus patients with refractory digital ulcers.

Topical opioids for painful ulcers in systemic sclerosis

Painful cutaneous ulcers are frequent in systemic sclerosis (SSc). They are due to vasoconstriction and fibrosis, and are often located in digits and toes though they also occur at other...

Statins Alleviate Vascular Disease, Stymie Ulcers in Systemic Sclerosis

Statins Alleviate Vascular Disease, Stymie Ulcers in Systemic Sclerosis

Successful treatment of patients with severe secondary Raynaud's phenomenon with the endothelin receptor antagonist bosentan

Secondary Raynaud's phenomenon (RP) in patients with systemic sclerosis (SSc) and other collagen vascular diseases is a serious manifestation of microvascular damage that may precede the onset of visceral and/or cutaneous sclerosis for years. Recent studies have demonstrated that the endothelin receptor antagonist bosentan prevents the development of new digital ulcers in SSc. We investigated the potential benefits of bosentan in patients with secondary RP associated with pre-scleroderma and with SSc, independent of digital ulcers. Three patients with secondary RP received bosentan 62.5 mg twice daily for 4 weeks followed by 125 mg twice daily for 12 weeks during the winter season. Pain (visual analogue scale), Raynaud's disease activity (Scleroderma Health Assessment Questionnaire), number and severity of daily Raynaud's attacks (diary) and peripheral thermoregulation (thermography) were assessed during treatment periods. Pain, Raynaud's disease activity, number and severity of Raynaud's attacks significantly decreased during treatment periods. Thermography after 16-week treatment demonstrated improved peripheral thermoregulation. Although this is a small observational study, treatment with bosentan appears to measurably reduce the daily impact of Raynaud's disease and improve peripheral thermoregulation in patients with secondary RP, independent of digital ulcers.

Topical lidocaine helps reduce pain of digital ulcers in systemic sclerosis (scleroderma)

Topical lidocaine helps reduce pain of digital ulcers in systemic sclerosis (scleroderma)

Digital ulcers in systemic sclerosis: Prevention by treatment with bosentan, an oral endothelin receptor antagonist

Recurrent digital ulcers are a manifestation of vascular disease in patients with systemic sclerosis (SSc; scleroderma) and lead to pain, impaired function, and tissue loss. We investigated whether treatment with the endothelin receptor antagonist, bosentan, decreased the development of new digital ulcers in patients with SSc. This was a randomized, prospective, placebo-controlled, double-blind study of 122 patients at 17 centers in Europe and North America, evaluating the effect of treatment on prevention of digital ulcers. The primary outcome variable was the number of new digital ulcers developing during the 16-week study period. Secondary assessments included healing of existing digital ulcers and evaluation of hand function using the Scleroderma Health Assessment Questionnaire. Patients receiving bosentan had a 48% reduction in the mean number of new ulcers during the treatment period (1.4 versus 2.7 new ulcers; P = 0.0083). Patients who had digital ulcers at the time of entry in the study were at higher risk for the development of new ulcers; in this subgroup the mean number of new ulcers was reduced from 3.6 to 1.8 (P = 0.0075). In patients receiving bosentan, a statistically significant improvement in hand function was observed. There was no difference between treatment groups in the healing of existing ulcers. Serum transaminase levels were elevated to >3-fold the upper limit of normal in bosentan-treated patients; this elevation is comparable with that observed in previous studies of this agent. Other side effects were similar in the 2 treatment groups. Endothelins may play an important role in the pathogenesis of vascular disease in patients with SSc. Treatment with the endothelin receptor antagonist bosentan may be effective in preventing new digital ulcers and improving hand function in patients with SSc.

The effect of percutaneous dimethyl sulfoxide on cutaneous manifestations of systemic sclerosis.

DMSO exerts a palliative, therapeutic effect on healing of cutaneous ulcers in systemic sclerosis. The therapeutic response was variable and, therefore, the concentration of DMSO, as well as frequency and duration of treatments, should be individualized to obtain maximum healing effect with a minimum of adverse reactions. There was no evidence of ocular toxicity or other serious toxicity manifestations in this group of patients treated with topical DMSO for one year or longer. Delayed improvement was observed in the untreated extremity in the majority of patients studied. In no instance did improvement in the untreated extremities exceed improvement in the treated, bilateral counterpart. It is believed this resulted from a systemic, carry-over effect of DMSO rather than spontaneous improvement in the disease course. DMSO is a worthwhile, supplemental, therapeutic agent providing the limitations of therapy are understood.