Scleroderma skin ulcers are challenging to manage and can be approached with a variety of therapies and treatments.
Scleroderma skin ulcers are challenging to manage and can be approached with a variety of therapies and treatments, according to a Professor of Dermatology and Medicine in the Department of Medicine at the University of Alberta in Edmonton, speaking at the annual meeting of the Canadian Association of Wound Care (CAWC).
Managing numerous systemic diseases that produce wounds and ulcers such as scleroderma, pyoderma gangrenosum, necrobiosis lipoidica and vasculitis may require the use of systemic medications, says Alain Brassard, M.D., FRCPC. Dressings are not sufficient to treat wounds and ulcers associated with numerous systemic diseases, says Dr. Brassard.
In the case of scleroderma of the skin, approximately 35% of patients will eventually get cutaneous ulcers, according to Dr. Brassard, whose work has been done in collaboration with Michelle Ramien, M.D., FRCPC, a dermatologist in Ottawa.
"Scleroderma is a disease that is systemic and needs to be treated systemically to heal the ulcers," says Dr. Brassard, in an interview with Dermatology Times. "The ulcers are related to endothelial cells that are driving the scleroderma. Raynaud's phenomenon is usually present in many patients with scleroderma. That phenomenon may create digital ulcers that are horrible in terms of the impact on quality of life of patients."
Digital ulcers are typically caused by minor trauma in a context of progressive ischemia which creates skin changes, explains Dr. Brassard.
Pharmacologic approaches include vasodilators, which target Raynaud's phenomenon, and agents aimed at endothelial cell protection such as Phosphodiesterase-5 inhibitors (sildenafil and tadalafil). Secondary Raynaud's phenomenon linked with scleroderma can be more severe than primary Raynaud's phenomenon.
Calcium-channel blockers have been shown to offer a significant benefit in managing Raynaud's phenomenon because of the improvement in the frequency and severity of ischemic attacks.1
Another study has found that infusions of iloprost was a preferred therapy for acute necrosis. Scleroderma ulcers may herald pulmonary hypertension. Bosentan monohydrate, an endothelial cell protector, avoids vasoconstriction and has demonstrated that it is effective in preventing the formation of new digital ulcers as well as reduce the development of pulmonary arterial hypertension.2
Statins are agents that are immunomodulatory, vasodilatory, and antifibrotic.3 The evidence, however, for their use in managing scleroderma and systemic sclerosis is weak, as they have not been studied in large trials but could be considered and should be studied further.4
Another emerging medical therapy in the treatment of digital ulcers in patients with systemic sclerosis is botulinum toxin type A, which decreases swelling, decreases pain, and enhances perfusion of fingers by opening up the vasculature and permitting better oxygenation.
"There has been incredible change in the oxygenation of the fingers," says Dr. Brassard, commenting on the effect of botulinum toxin to manage digital ulcers. "You can have rapid decrease in pain with the use of botulinum toxin."
Clinicians should be cautious about injecting botulinum toxin so as not to cause excessive relaxation of the hand muscles. Injections should be initially limited to the fingers, with successive injections to the hand if necessary at subsequent visits.
Injection of botulinum toxin is a less invasive alternative to surgical sympathectomy, producing a chemical sympathectomy that will have a duration of several months.
Non-pharmacological management avenues include avoidance of cold, stress, and trauma, as well as smoking cessation. On the horizon lie future treatments for sclerodermal skin ulcers, such as stem cell therapy.
Skin ulcers associated with scleroderma can be very painful for patients, making supportive therapies integral to the management of sclerodermal ulcers. Some of the supportive therapy options can include topical opioids like morphine gel, systemic opioids, and benzodiazepines, according to Dr. Brassard.
Dr. Brassard and Dr. Ramien had no relevant disclosures.
Thompson AE, Shea B, Welch V, Fenlon D, Pope JE. Calcium-channel blockers for Raynaud's phenomenon in systemic sclerosis. Arthritis Rheum. 2001;44(8):1841-7.
Korn JH, Mayes M, Matucci cerinic M, et al. Digital ulcers in systemic sclerosis: prevention by treatment with bosentan, an oral endothelin receptor antagonist. Arthritis Rheum. 2004;50(12):3985-93.
Derk CT, Jimenez SA. Statins and the vasculopathy of systemic sclerosis: potential therapeutic agents?. Autoimmun Rev. 2006;5(1):25-32.
Kuwana M. Potential benefit of statins for vascular disease in systemic sclerosis. Curr Opin Rheumatol. 2006;18(6):594-600.