Scleroderma: Overview
What is scleroderma?
When a person has scleroderma, the body makes too much collagen. This excess collagen, the substance that holds our body together, causes hardening and tightening.
Most people have hardening and tightening on their skin. Scleroderma means “hard skin.” But this disease can affect more than the skin. Joints, muscles, and even internal organs like the kidneys and lungs can harden and tighten.
Many people who have scleroderma lead normal—or almost normal—lives.
Where you have the hardening and tightening varies with the type of scleroderma you have. When scleroderma affects the skin and sometimes the underlying tissue, a person has localized scleroderma.
Types of scleroderma
There are many types of localized scleroderma, including:
Morphea on skin
Morphea (more-fee-uh): People see 1 or a few patches of thickened skin, which are usually red or purple. The patches can itch but are usually painless. Sometimes the excess collagen develops deep in the skin. In rare cases, morphea affects muscle.
Patches of morphea on patient's back
Gerneralized morphea: Patches of morphea can develop on different areas of the body. The patches can grow together. Some people see a band of thickening skin on an arm or leg. This type can go deep into the tissue beneath the skin.
Linear morphea on lower leg and foot
Linear scleroderma: Often beginning in childhood or the teenage years, this type causes a line of thickening skin, usually on an arm or leg. The hardening can extend beyond the skin, going deep into to the muscle and sometimes the bone.
En coup de sabre on forehead
En coup de sabre: A line of thickened skin forms on the scalp, face, or both, and the tissue beneath disappears. A person with a mild case develops a bit of tissue loss. En coup de sabre can also be disfiguring.
A dermatologist can use a filler, such as hyaluronic acid, to restore the face.
When scleroderma affects an internal organ, the person has a type of scleroderma called systemic sclerosis. There are 2 types of systemic sclerosis:
Sores and calcium deposits on fingers
Limited cutaneous scleroderma: This often develops slowly. Hard, thick, and tight skin usually forms below the elbows and knees and sometimes on the face and neck.
Many people develop calcium deposits beneath their skin and sores, as show here.
The hardening can also occur in the digestive tract and internal organs like the lungs. The health problems caused by this type are less serious than those caused by diffuse cutaneous scleroderma.
Woman is unable to straighten her fingers
Diffuse cutaneous scleroderma: In just a few weeks or months, hard, thickened skin can develop on many areas of the body. The excess collagen also develops on one or more internal organ.
The hardening can cause a person to lose the ability to move freely. The patient shown in this picture lost the ability to straighten her fingers.
Under the care of a team of doctors who specialize in different areas of the body, it may be possible to manage this type.
There is no cure for scleroderma, but treatment can help reduce the effects of this disease.
Because scleroderma can affect the body in so many ways, many people who have scleroderma receive treatment from a team of medical specialists. A dermatologist may be a part of this team and can treat problems with the skin.
Images
Images 1 - 4: J Am Acad Dermatol 2011;64(2):217-28.
Image 5: J Am Acad Dermatol 2012;67(4):769-84.
Image 6: J Am Acad Dermatol 2011;65(1):1-12
Image 7: J Am Acad Dermatol 2000;43(4)670-74.
References
Falanga V and Killoran CE. “Morphea.” In: Wolff K, Goldsmith LA, et al. Fitzpatrick’s Dermatology in General Medicine (seventh edition). McGraw Hill Medical, New York, 2008:543-6.
Fett N and Werth VP. “Update on morphea: Part I. Epidemiology, clinical presentation, and pathogenesis.” J Am Acad Dermatol 2011;64(2):217-28.
Fett N and Werth VP. “Update on morphea: Part II. Outcome measures and treatment. J Am Acad Dermatol 2011;64:231-42.
Rőcken M and Ghoreschi K. “Morphea and lichen sclerosus.” In: Bolognia JL, et al. Dermatology. (second edition). Mosby Elsevier, Spain, 2008:1469-76.
Walls A, Goldberg D, et al. “Correction of morphea en coup de sabre with hyaluronic acid filler.” Presented as a poster at the 2012 Annual Meeting of the American Academy of Dermatology and published in the J Am Acad Dermatol 2012:66(4) AB209. No commercial support identified.
Zwischenberger BA and Jacobe HT. “A systematic review of morphea treatments and therapeutic algorithm.” J Am Acad Dermatol 2011;65(5):925-41.