The Scleroderma Hand: Manifestations of Disease and Approach to Management

Abstract

Scleroderma is a rare autoimmune connective tissue disorder that often affects the hands. Manifestations in the hands include calcium deposits within the soft tissues that cause pain and may ulcerate through the skin, digital ischemia resulting in chronic wounds and digital gangrene, and joint contracture. Because of the underlying disease, patients with scleroderma have poorly vascularized tissue and a deficient soft tissue envelope, which make surgery particularly challenging. However, when undertaken with care, surgical intervention is often the best option for addressing the disabling hand conditions that so often accompany this disease.

Revision periarterial sympathectomy for recurrent digital ischaemia: a report with 11 patients

“…Revision periarterial sympathectomy has been discouraged by some authors, warning that it can be associated with higher levels of postoperative complications, such as difficulty with wound healing as a consequence of re-operating in an ischemic surgical site (Balogh et al, 2002;Williams et al, 2018). In our series, we did not encounter any wound healing complications.…”

“…All surgical sites healed uneventfully, and four of five digital ulcers healed after surgery. Potential injury to the neurovascular structures has been another concern, as the initial periarterial sympathectomy may cause scarring around the neurovascular bundles (Williams et al, 2018). This risk can be reduced by using a new incision on the symptomatic digit or by avoiding the prior surgical field and scar burden in the palm.…”

“…Periarterial sympathectomy addresses inappropriate vascular control by removing the sympathetic nerves in the arterial adventia, thereby reducing distal vasoconstrictor tone (Charkoudian, 2003). This procedure has been shown to reduce pain, hasten digital ulcer healing and decrease development of new ulcers through an increased nutritive flow to the digits (Koman et al, 1995;Ruch et al, 2002;Williams et al, 2018). Sustained long-term improvement of symptoms has been demonstrated for up to 10 years postoperatively (Murata et al, 2012).…”

From organic and inorganic phosphates to valvular and vascular calcifications

“… 45 However, in different microenvironments, such protections either disappear or become insufficient to prevent calcium binding to extracellular phosphates, thereby initiating mineralization through precipitation, crystallization and polymerization on soft matrices. Under pathological conditions, soft tissue calcifications can be observed in tumours, 46 skin, connective tissue disorders such as scleroderma, 47 muscles, 48 tendons, 49 abscess, 50 and venous wall (phleboliths). 51 These soft tissue localizations are usually dystrophic, related to tissue injuries, and occur in association with a physiological systemic phosphorous metabolism.…”

Microvascular hand surgery for digital ischemia in scleroderma

“…This is especially the case when the digit is deemed at risk for need for amputation due to inadequate blood flow. [40][41][42] Surgical sympathectomy is aimed at both mechanical denervation to decrease sympathetic tone and removal of the periarterial fibrosis through adventitial stripping, so it addresses both the vasospastic and obstructive fibrotic components of SSc. Current medical therapies tend to target the sympathetic-mediated pathology, so evidence of arterial stenosis or occlusion on vascular imaging, consistent with structural, fibrotic changes, may indicate more advanced disease that is less likely to respond to pharmacologic measures.…”