Digital Ulcers in Systemic Sclerosis – Frequency, Subtype Distribution and Clinical Outcome

Abstract

Digital ulcers (DUs) are frequent and recurrent complication in systemic sclerosis (SSc) and are the main cause of pain, impaired function of the hand and disability in SSc. The current study is a retrospective analysis of 60 SSc patients (47 patients with limited cutaneous SSc, 8 patients with diffuse cutaneous SSc and 5 patients with overlap syndrome, mean age 54.5±14.2 years, 52 women and 8 men). The frequency and evolution of DUs as well as the applied therapeutic strategies were analyzed. During the follow-up for a period between 6 months and 6 years, DUs at the fingers were registered in 35% of patients (21/60), more often in patients with diffuse cutaneous SSc (75%, 6/8) as compared with patients with limited cutaneous SSc (29%, 14/47, p<0.05) and overlap syndrome (20%, 1/5). The most frequently observed DUs were ischemic lesions at the fingerpads (85.7%, 18/21) and ulcerations over bony prominences of the fingers (23%, 5/21), which may be found simultaneously. More rare types of DUs were necrotic lesions (14%, 3/21). Thirty-eight percents (8/21) of the patients with DUs showed signs of inflammation. In one patient (4.76%, 1/21) an osteomyelitis developed and an amputation of a finger's distal phalanx was performed. DUs at the toes were significantly less frequent as compared with DUs at the fingers (10%, 6/60, p<0.05). The period of healing of the DUs is prolonged and in the studied group was 3.39±2.39 months. The treatment regimen in SSc patients with DUs included vasodilators, local antiseptic treatment, antiplatelet drug; anticoagulant in cases with development of necrotic lesions, antibiotics in cases of infection or necrotic lesions, and other symptomatic therapies. In conclusion, DUs are a common complication in SSc and require complex therapeutic measures for achievement of a positive outcome.

Raynaud phenomenon and digital ulcers in systemic sclerosis

“…12 Several studies have reported that fingertip DUs have a higher prevalence than extensor ulcers. [13][14][15] In contrast, Ennis et al, reported that extensor ulcers had a similar prevalence (of 6%) and were as similarly disabling as fingertip DUs. 11 Patients often develop ulcers affecting multiple digits simultaneously, including both fingertip and extensor-aspect DUs.…”

“…11 Patients often develop ulcers affecting multiple digits simultaneously, including both fingertip and extensor-aspect DUs. 15 Despite the availability of a number of advanced therapies to prevent and treat DUs, around one third of patients with SSc may develop recurrent ulceration. 16 Clinical presentation RP is a highly variable symptom complex which results from aberrant digital perfusion.…”

Exploring the patient experience of digital ulcers in systemic sclerosis

“…Ischaemic DUs commonly occur on the tips of the fingers(Figure 1) and over the extensor aspect (in particular, over the small joints) of the hands. Several studies have found that fingertip DUs are more common than extensor-aspect DUs [13,14]. For example, in the retrospective study by Lambova et al [14], which included 60 patients with SSc, over a follow-up period of between 6 months to 6 years, incident DUs were observed in around a third(n=21,35%) of patients, and with a predominance of fingertip(n=18/21) compared to extensor(n=5/21) DUs; however, both types could be found simultaneously.…”

“…Several studies have found that fingertip DUs are more common than extensor-aspect DUs [13,14]. For example, in the retrospective study by Lambova et al [14], which included 60 patients with SSc, over a follow-up period of between 6 months to 6 years, incident DUs were observed in around a third(n=21,35%) of patients, and with a predominance of fingertip(n=18/21) compared to extensor(n=5/21) DUs; however, both types could be found simultaneously. Whereas, in a prospective study, which included 148 patients(15 patients who developed 25 SSc-DUs), the prevalence of both fingertip and extensor DUs was 6% [5].…”

“…Somestudies have reported that SSc-DUs occur more commonly in males [2,3,[19][20][21], whereas, others have not identified gender-related differences [4,22], or have suggested SSc-DU are more common in females [23]. Several studies have reported a higher burden of SSc-DU in diffuse compared to limited cutaneous SSc [14,19,23]. SSc-DUs have been associated with higher skin [1,3,4,20,23,24], including hand and finger score [4] although SSc-DU were unrelated to the extent of skin thickening in the Scleroderma Lung study II [25].…”