A small patch of thickened skin might seem like a cosmetic issue or something that will go away on its own — and it could. But if it lasts, it’s worth mentioning to your doctor.
Thick or tight skin, particularly on the fingers or feet, could be a sign of scleroderma (sklair-oh-DUR-muh). Scleroderma translates from Greek as “hard skin.” It often starts with a thickening or tightening of the skin in the fingers or feet. Some people also notice that their fingers get puffy. The skin can be itchy, particularly early in the disease.
What is Scleroderma?
Scleroderma is an autoimmune condition that causes inflammation in the skin and other areas of the body. Then the body starts making excessive collagen — a protein that makes up connective tissue — which builds up. (1)
What are the different types of Scleroderma?
There are two kinds of scleroderma:
- Localized. The skin and soft tissue structures directly underneath it are affected, most often in children or young adults. It often starts as small patches of hard skin (morphea) over the trunk but can start over the arms or legs. There may be one patch, a few patches, or a large number of patches that may conglomerate together and extend over large areas of the skin. Sometimes a single limb — such as an arm or leg — may be affected (linear scleroderma) or hardening of the skin may indent the soft tissue underneath the forehead (en coup de sabre). Internal organ involvement is typically uncommon.
- Systemic. Internal organs are nearly always affected in addition to the skin. This more serious type of scleroderma can be disfiguring if skin thickening extends to the face. It can damage the blood vessels and impact the heart, lungs and kidneys. Based on the extent of skin involvement, it can be limited (skin thickening below knees and elbows) or diffuse (skin thickening extending to upper arms, thighs and trunk). Women between ages 30 and 60 are most commonly affected. Having a family history of autoimmune conditions also is a risk factor.
People are very unlikely to have both types, and localized scleroderma rarely progresses into systemic.
Scleroderma has no cure, but medications can slow progression and reduce its effects.
Ashima Makol, M.D., a rheumatologist at Mayo Clinic in Rochester, Minnesota, helps explain why it’s important to mention a hard patch of skin, puffy fingers or skin tightening over hands — all early signs of scleroderma — to your doctor.
1. Red, white or blue hands need special care.
Hands turning white, blue and red in response to cold temperature or stress is common and can be an early sign of scleroderma, starting months or even years prior to skin thickening. This is the result of blood vessels constricting and reducing blood supply to the fingers. It can cause pain or numbness in the hands. Known as Raynaud’s phenomenon, this can also affect teens and young adults without scleroderma but warrants further medical evaluation for an underlying cause when it develops at a later age.
“The fingers become dead-white-looking at the tips,” explains Makol. “It can be pretty scary for someone to experience. It can cause numbness, tingling, throbbing pain or burning. As you can imagine, if it’s one or two episodes here and there, it’s less bothersome, but it can happen several times a day in some people, which causes a lot of hand discomfort.”
In severe cases, this can lead to difficult-to-heal skin sores or tissue injury with risk of amputation if not addressed promptly. Often people can help mitigate the response by dressing warmly and managing stress, but medications like nifedipine or amlodipine (calcium channel blockers) are often necessary.
2. Daily tasks can be difficult.
Skin tightness can restrict range of motion in fingers and hands permanently, so scleroderma can impact day-to-day living. For example, it can be difficult to hold a pen or use a hairbrush.
“Scleroderma can be a very disfiguring and disabling condition,” Makol says. “It can change the way a person looks and feels. There’s difficulty making a fist, gripping things and performing common activities around the house.”
The condition can also cause joint pain and swelling that make movement difficult. Massage or physical therapy can help. Medications used to suppress the immune system may help loosen the skin and joints.
3. Scleroderma can damage inner organs.
When scleroderma is caught early, doctors have more options for slowing progression of the disease. The scarring from systemic scleroderma can impact the digestive system, lungs, heart and kidneys. This can cause:
- Heartburn
- Difficulty swallowing
- Shortness of breath and cough
- Fluid buildup in the legs, feet or around the heart
- Irregular heart rhythm
- Rapid rise in blood pressure and loss of kidney function
4. Treatment is particularly important early in the disease.
“The first 5 to 7 years are typically the worst phase of the disease,” Makol says. “After that, the skin disease tends to plateau, stabilize or even improve. The risk of major organ involvement goes down drastically, but patients need continued monitoring for heart involvement (pulmonary hypertension) long term as it can be a late complication.”
That means that particularly during those first five years, it’s important to be under the close care of a rheumatologist, preferably at a scleroderma center of expertise. At most scleroderma centers, patients see a multidisciplinary team of specialists in cardiology, pulmonology, gastroenterology and/or vascular medicine along with rheumatology.
Once deadly, scleroderma is now a manageable, treatable disease. There are also two FDA-approved therapies for lung fibrosis in scleroderma, and many ongoing clinical trials to expand the therapeutic possibilities in this complex disease.
5. Scleroderma can impact children’s growth.
Scleroderma in children tends to form in a straight line, for example, up the child’s leg. When this happens, growth can be impacted, so one leg or arm ends up shorter than the other. This form of scleroderma can also change skin pigmentation, but it doesn’t impact major organs.
“Patients can be reassured that it’s not life-threatening by any means,” says Makol.
For all these reasons, tell a health care provider about any persistent puffiness of the fingers, skin thickening, or tightness and new symptoms of Raynaud‘s phenomenon, so you can get any necessary tests for evaluation and early referral to specialists for the most optimal treatment.
