Scleroderma Associated Digital Ulcer

Digital ulcers are painful, open wounds that affect approximately half of all people with scleroderma at some point during their disease, significantly impacting hand function and quality of life.

Table of contents

• What Are Digital Ulcers?
• How Common Are Digital Ulcers?
• Why Digital Ulcers Develop in Scleroderma
• Location and Characteristics
• Impact on Patients
• Prevention Measures
• Treatment Options

What Are Digital Ulcers?

Digital ulcers are open sores that develop on the skin, most commonly on the fingers and toes of people with scleroderma. These wounds are defined as areas with a clearly visible depth that show a loss of skin covering, involving both the outer layer of skin (the epidermis, which is the protective surface layer) and the deeper layer beneath it (the dermis, which contains blood vessels and nerves). The ulcer may appear as a raw, exposed area or may be covered by dead tissue or a scab.^[1][2]

These wounds are distinct from simple cuts or scrapes. They have well-defined borders and represent a significant breakdown of the skin that can be slow to heal.^[2]

How Common Are Digital Ulcers?

Digital ulcers are a frequent complication in people with scleroderma. Research shows that approximately 30% of scleroderma patients experience digital ulcers each year, while about 50% will develop at least one ulcer at some point during the course of their disease.^[2][6][7]

Among a study of 1,085 scleroderma patients followed over an average of five years, nearly half (48.6%) experienced at least one digital ulcer. Those who developed ulcers were more likely to have certain characteristics, including diffuse disease subtype, younger age at disease onset, and the presence of specific antibodies.^[7]

Why Digital Ulcers Develop in Scleroderma

Digital ulcers in scleroderma result from problems with blood vessels that supply the fingers and toes. The condition causes both structural damage and functional problems in these small blood vessels.^[2]

The structural damage involves two main problems. First, the inner lining of blood vessels becomes thickened and filled with scar tissue, which significantly narrows the opening through which blood can flow. Second, this damage to the vessel walls causes an imbalance in the chemicals that control blood vessel width. The body produces more substances that make vessels narrow and fewer substances that help them widen. Together, these changes create conditions where blood flow is severely reduced.^[2]

The functional problem involves repeated episodes of vasospasm, which means the blood vessels suddenly tighten and narrow. This repeated narrowing leads to progressive tissue damage from lack of oxygen and creates harmful oxygen-free radicals that further damage the tissue. This creates a cycle that continues to worsen over time.^[2]

Nearly all people with scleroderma experience Raynaud’s phenomenon, a condition where fingers or toes change color in response to cold or stress. In scleroderma, Raynaud’s phenomenon is closely linked to digital ulcers. Unlike primary Raynaud’s, where blood vessels narrow temporarily and then return to normal size, in scleroderma the blood vessels become progressively smaller and may even disappear entirely over time. This leads to a reduced supply of oxygen and nutrients to the skin, resulting in dry, cracked skin and ultimately the formation of ulcers.^[4][6]

Location and Characteristics

Digital ulcers most commonly appear at the fingertips, where reduced blood flow causes tissue damage. These are considered ischemic lesions, meaning they result from inadequate blood supply.^[2]

Ulcers can also develop over bony bumps, such as the knuckles (proximal phalanges) or elbows. These ulcers typically form at pressure points where tight, stretched skin repeatedly experiences minor injuries. They may also extend under the fingernails or occur on the legs or other parts of the body following minor trauma such as a bruise.^[2][6]

Impact on Patients

Digital ulcers cause severe pain and can make it extremely difficult to use the hands or affected body parts. These sores represent a significant burden for patients beyond just the physical wound.^[2][6]

People with digital ulcers experience reduced quality of life due to extreme pain, difficulty with hand function, and loss of independence in daily activities. The ulcers can affect employment and work productivity. Patients require close contact with the healthcare system for careful wound care, adequate pain relief, and monitoring for complications such as infections of the skin or underlying bone, which may require immediate treatment.^[7][9]

The healthcare costs associated with digital ulcers are substantial. In one study, patients with a history of digital ulcers used significantly more healthcare resources annually than those without ulcers, including more hospital admissions, emergency department visits, and outpatient services. The excess annual healthcare cost per patient with digital ulcers was approximately AUD$12,474, driven primarily by hospital admission and emergency department costs.^[7]

In severe cases, digital ulcers can lead to serious complications including tissue death (necrosis) or, rarely, loss of part of the finger (self-amputation or surgical amputation).^[4][6]

Prevention Measures

Several measures can help prevent digital ulcers or reduce their severity. These focus on avoiding triggers that reduce blood flow and protecting the skin from injury.^[4][6]

Important preventive steps include:

• Avoiding cold environments and keeping as warm as possible
• Wearing layers of clothing to maintain body temperature
• Wearing hats in cold weather
• Choosing shoes and socks that provide adequate room for circulation
• Avoiding stress, which can trigger Raynaud’s attacks
• Not smoking cigarettes, as tobacco constricts blood vessels
• Avoiding caffeine, which also constricts vessels
• Using protective bandages on vulnerable areas

Early and intensive treatment of Raynaud’s phenomenon in scleroderma patients is one of the most effective preventive measures to avoid the development of digital ulcers and tissue loss.^[3][4]

Treatment Options

Basic Care and Medications

Basic wound care and over-the-counter medications may help with healing and pain management. For more serious cases, several types of medications may be prescribed to improve blood flow to the affected areas.^[6]

The first-line medication treatment for Raynaud’s phenomenon and digital ulcers typically involves calcium channel blockers (such as Nifedipine or Amlodipine). These medications work by relaxing blood vessels, allowing better blood flow to the extremities. The dose is gradually increased as tolerated until symptoms improve.^[4][6]

Other medications that have shown promising results include:

• Endothelin receptor blockers (such as Bosentan), which block substances that cause blood vessels to narrow
• Prostacyclin analogues (such as Iloprost), which help dilate blood vessels
• Phosphodiesterase 5 inhibitors, which improve blood flow

These medications have shown effectiveness both as standalone treatments and in combination for treating existing ulcers and preventing new ones.^[9][12]

Currently, there are no US Food and Drug Administration (FDA)-approved medications specifically for the treatment of digital ulcers in the United States, though clinical trials have supported the use of various treatment approaches.^[2]

Advanced Treatments

For severe or difficult-to-heal ulcers, more advanced treatments may be necessary. These can include treatment of infections with antibiotics (oral or intravenous), medications to improve blood flow, and in some cases, surgical procedures.^[6]

Some less common treatment approaches that have shown success include hyperbaric wound chambers, which dramatically increase oxygen delivery to tissues, and digital sympathectomy, a surgical procedure that removes scar tissue from arteries and veins in the wrist to provide better blood flow to the hand.^[17]

Other options that may be useful in recalcitrant cases include autologous fat grafting and botulinum toxin injections, though these are not readily available everywhere.^[9]

If tissue is too damaged to heal, surgery or amputation may be necessary.^[6]

Self-Care Strategies

Some patients have found certain self-care approaches helpful in managing ulcers. One approach involves keeping the ulcer as dry as possible, as moisture can increase pain and slow healing. Protecting ulcers from water during showering or hand washing by covering them with gauze or plastic bags may help.^[17]

Some people have reported success using medical-grade honey (such as organic mānuka honey) on ulcers, taking advantage of its antibacterial properties. Applied in a small amount on a bandage, honey may help create a protective layer and promote wound closure.^[17]

Keeping hands warm and avoiding Raynaud’s attacks as much as possible is essential, as these attacks can cause existing ulcers to become more painful.^[17]