The challenge of scleroderma ulcers

Ulcers are common in systemic sclerosis, but the etiologies are diverse and often multifactorial. It is important to discern the primary factors contributing to ulcer formation in individual patients to devise therapeutic regimens effectively. Clinical symptoms such as the absence or presence of RP are useful to identify vasospasm as a causative factor. A biopsy may be helpful to distinguish vasculitic from thromboembolic ulcers if the clinical picture is not clear. Once the main etiologies are identified, appropriate therapies can be selected. Patients with systemic sclerosis who have never had an ulcer should be educated to avoid RP triggers and stop smoking. RP should be treated. Statins with or without acetylsalicylic acid should be started. Systemic sclerosis patients with healed ulcers should receive the same therapy as above with the addition of maintenance therapy with a dual vasodilatory-endothelial protective agent such as a PDE5I or bosentan that are both well tolerated and safe to continue on an indefinite basis with appropriate monitoring. Patients with active ulcers should be treated with standard care, including pain control, wound care with hydrocolloid occlusion dressings, and antibiotics as needed for superinfection. Treatment to reduce ischemia and improve healing should be tailored to the patient's ulcer etiology. Despite interesting recent developments in treatment, systemic sclerosis ulcers will continue to be a therapeutic challenge until the pathogenesis of the disease itself is better elucidated and directed, and individualized therapies can be developed. A systematic approach that attacks the ulcerpromoting process at its roots - most importantly targeting vasoconstriction, endothelial dysfunction, and vessel damage and integrity - is necessary to treat these challenging ulcers properly.