Digital ulcers in patients with systemic sclerosis.
Digital ulcers (DU), defined as necrotic lesions located at distal digits or overlying bony prominences, occur in up to 50% of patients with limited or diffuse systemic sclerosis (SSc). These lesions are extremely painful and lead to substantial functional disability. The pathogenesis of DU differs depending on their location. DU located at distal aspects of digits are thought to be related to tissue ischemia from several processes, including vasospasm secondary to Raynaud's phenomenon, intimal fibro-proliferation, and thrombosis of digital arteries. DU located over bony prominences, such as the phalangeal joints and elbows, are thought to be due to repetitive microtrauma and difficulty healing due to atrophic, avascular tissue overlying the joints. Management of DU include non-pharmacologic and pharmacologic modalities. This review summarizes the current available and investigational therapies for the treatment and prevention of DU in patients with SSc.
Chung
Lorinda
L
Stanford University School of Medicine, Department of Immunology and Rheumatology, 1000 Welch Road, Suite #203, Stanford, CA 94305, USA. shauwei@stanford.edu
Fiorentino
David
D
D000925:Anticoagulants / Q000627:therapeutic use; D011464:Epoprostenol / Q000031:analogs & derivatives / Q000627:therapeutic use; D005385:Fingers / Q000473:pathology*; D006801:Humans; D010879:Piperazines / Q000627:therapeutic use; D010975:Platelet Aggregation Inhibitors / Q000627:therapeutic use; D011687:Purines; D012595:Scleroderma, Systemic / Q000150:complications*; D017367:Selective Serotonin Reuptake Inhibitors / Q000627:therapeutic use; D000068677:Sildenafil Citrate; D012883:Skin Ulcer / Q000209:etiology / Q000517:prevention & control* / Q000628:therapy; D013450:Sulfones; D035703:Therapies, Investigational; D014665:Vasodilator Agents / Q000627:therapeutic use
D000925:Anticoagulants; D011464:Epoprostenol; D010879:Piperazines; D010975:Platelet Aggregation Inhibitors; D011687:Purines; D017367:Selective Serotonin Reuptake Inhibitors; D000068677:Sildenafil Citrate; D013450:Sulfones; D014665:Vasodilator Agents; C427248:treprostinil