Types of Scleroderma
There are two main categories of scleroderma: localized and systemic. Each category is made up of several conditions.
- Localized scleroderma: often affects only the skin and not major organs.
- Systemic scleroderma: affects the skin and may affect the tissues under it, including blood vessels and major organs, such as the gastrointestinal tract, heart, lungs or kidneys.
Three Types of Localized Scleroderma: Localized, Generalized and Linear
The skin is typically the only organ impacted in localized scleroderma. However, the tissue injury may extend into the structures underlying the skin, including the subcutaneous tissue, fascia, muscle or bone. Localized scleroderma skin lesions can get better or even go away.
With circumscribed morphea (another name for discolored patches of skin), you may have a single oval patch or you may see a few patches of morphea. The patches vary in size and typically have a red border and a thickened pale-yellow center. These lesions can enlarge when active and then flatten and become asymptomatic with treatment. Deep circumscribed morphea extends into the subcutaneous tissues.
Generalized morphea is seen when there are many patches of morphea (greater than four plaques in many anatomical areas (trunk, arms, head or neck). They are seen as thick, tight patches that can blend into each other. Pansclerotic morphea is a severe form of generalized morphea with involvement of most of the body.
Linear scleroderma is more common in children 10 and younger. These tight, thick bands can appear on the extremities, the back and front of the trunk, the buttocks or the face. They often appear as a single band in one body area and can be seen mixed with patches of morphea. Linear scleroderma can affect the bones and the muscles. When linear scleroderma appears on the arms or legs, it can cause the child’s limb to under develop, causing disability.
Three Types of Systemic Sclerosis (Scleroderma): Limited, Diffuse and Sine
Systemic sclerosis (scleroderma) affects the skin as well as what’s underneath, such as blood vessels, muscles and joints, gastrointestinal (GI) tract, kidneys, lungs and heart.
The skin thickening that accompanies systemic scleroderma can cause tightening so there is a loss of flexibility and ease of movement, especially in the fingers. Facial involvement is common and may be mild or it can reduce facial movements, including decreasing the mouth opening.
Scleroderma may cause chronic joint pain, inflammation and swelling in muscles and joints. Most scleroderma patients also experience Raynaud’s phenomenon, an exaggerated response to ambient temperatures, making one sensitive to cold. Raynaud’s phenomenon causes the skin of the fingers to look white or blue with cold temperature exposure or emotional stress. Skin sores or ulcers can occur due to lack of good blood flow in some cases.
Limited scleroderma is the most common type of scleroderma. The skin hardening and tightening is limited usually just to the fingers and sometimes the hands, forearms or the face. Internal organ damage is less likely in the limited scleroderma type. In general, patients with limited scleroderma have a normal life expectancy. Some have problems with their GI tract, especially heartburn; severe Raynaud’s and musculoskeletal pain; and a small subset can develop pulmonary hypertension that can be life-threatening.
A subtype of limited scleroderma is also known as CREST syndrome. CREST is an acronym for its most prominent clinical features:
- Calcinosis: calcium deposits under the skin and sometimes in tissues.
- Raynaud’s phenomenon: an exaggerated response to ambient temperatures making the skin of the fingers or toes cold, numb or tingling with color changes.
- Esophageal dysmotility: which causes heartburn.
- Sclerodactyly: the skin on the fingers becomes thick.
- Telangiectasias: enlarged blood vessels that appear as red spots on the fingers, face or other parts of the body.
Diffuse scleroderma is a subtype of scleroderma where excess collagen production causes skin thickening over large areas of the body, usually the fingers, hands, arms, anterior trunk, legs and face. There can be significant associated organ damage, including to the gastrointestinal tract, kidneys, lungs and heart. The tightening of the skin is often associated with dryness and itching. Musculoskeletal pain is common. Life-threatening disease occurs when the lung or heart is severely affected. Acute severe systemic high blood pressure can cause kidney damage.
Sine sclerosis is systemic scleroderma that has features of systemic organ disease, including Raynaud’s phenomenon, but not the skin symptoms. The symptoms and complications of either limited or diffuse scleroderma can occur, but there is no skin thickening.