Skin Fibrosis in Scleroderma: Complications and Treatments

Often skin fibrosis first shows up in the fingers. It may be preceded by itchy skin. In the face, tightening may occur that results in a small mouth opening and a “pinched” nose.

Sclerodactyly is the scarring and tightening of skin on the fingers. It’s a hallmark sign of scleroderma, and no other disease causes it. If a person has sclerodactyly, doctors know scleroderma is present. It is typically symmetrical.

Over time, this can cause tasks that require hand dexterity to become difficult—things like writing with a pencil or opening a water bottle. The fingers will lose their natural cylindrical shape and will taper at both ends. The tightened skin and underlying connective tissue tighten around the joints so that the fingers are pulled into a permanently bent position.

The skin and connective tissue around the mouth thickens and tightens, losing normal elasticity. The mouth opening shrinks and can lose its normal folds and wrinkles.

To assess skin fibrosis, doctors will determine the modified Rodnan skin score (mRSS) which can have a value of 0 to 51. Each body area evaluated gets a score of 0 to 3, and then all scores are added up to determine the total score. Doctors use this score to track scleroderma over time. If the score rises, it’s a sign the disease is progressing.

One biomarker, called RNA polymerase III (RNA poly III), can offer clues. People who test positive for it often develop severe skin symptoms early on. Starting treatment quickly is important. This can help prevent the fingers from becoming permanently bent (called joint contractures).

People with RNA poly III don’t always have Raynaud’s phenomenon, a common scleroderma symptom where fingers turn white or blue in the cold. Instead, they may notice puffy fingers as an early sign.

There are therapies that cause immune suppression and targeted therapies that can be used to slow down disease progression. The European Alliance of Associations for Rheumatology (EULAR), a leading rheumatology society, has made recommendations on therapies that may be of use to treat skin fibrosis.

It’s important to note that these recommendations are made conditionally, which means that not every treatment option is appropriate for every SSc patient. For skin fibrosis, EULAR recommends methotrexate, mycophenolate mofetil, and/or rituximab.²

There are different ways to manage skin fibrosis without the use of therapies, aimed at improving quality of live. Hand stretching exercises, which can be performed at home and manual lymph drainage have been shown to improve hand function. There are also mouth stretching exercises that can improve the reduction in mouth opening.

Yes. Individuals with sine scleroderma have no evidence of fibrosis in the skin, but they will exhibit the characteristic fibrotic features of scleroderma.