Scleroderma is a chronic autoimmune disease characterized by hardening of the skin, although it can also affect internal organs.
An autoimmune disorder is one in which the immune system attacks healthy tissue instead of invaders.
The hallmarks of localized scleroderma are inflammation and thickening of the skin because of excessive levels of collagen protein. This type of scleroderma is relatively mild, and commonly affects only a few areas of the body — usually the skin or muscles. It rarely develops into the systemic form of scleroderma, so internal organs are typically not affected.
Localized scleroderma is sometimes called morphea, which is also the term used to describe the disease’s skin symptoms.
Types of morphea
Patterns of morphea that are considered subtypes of localized scleroderma include plaque morphea, or blistering morphea, generalized morphea, and deep morphea.
Morphea usually involves isolated patches of hardened skin of varying sizes, shapes, and color that can disappear and come back at any time. Different types of morphea can cause different depths of the disease. Some subtypes cause deeper damage to tissue under the skin. These types of localized scleroderma typically do not affect internal organs.
Linear scleroderma is a localized type that can affect the bones and muscles underneath the skin. It appears most commonly on the arms, legs, or forehead, although it can occur in other places as well. It usually shows up in childhood, and can affect the growth of limbs.
There are two types of linear scleroderma: Parry-Romberg syndrome, or progressive hemifacial atrophy, and en coup de sabre, which means “like a sword cut.” Both affect the skin on the head and can also affect the teeth, tongue, eyes, facial nerves, and brain.
Systemic scleroderma is a multi-system form of the disease. It leads to tissue scarring or fibrosis, damage to blood vessels, and abnormal connective tissue growth in many areas, including the muscles and joints. These abnormalities can cause a breakdown in the skin, the tissues under it, muscles, and internal organs such as the heart, lungs, kidneys, and intestinal tract.
Systemic scleroderma is typically classified into two types, based on the extent of skin symptoms. One is limited cutaneous systemic scleroderma, or simply limited scleroderma. The other is diffuse cutaneous systemic scleroderma, also known as just diffuse systemic scleroderma. A rare type of systemic scleroderma, sine sclerosis, damages internal organs but does not thicken the skin.
Limited scleroderma is the more common version of the systemic form. The skin thickening is less widespread and typically confined to areas below the elbows, hands, fingers, and face. In rare cases, the kidneys, lungs, and heart can also be affected.
Limited scleroderma usually develops slowly over several years. It is also known as CREST syndrome. CREST is an acronym for the conditions that can occur along with limited scleroderma: calcinosis, Raynaud’s phenomenon, esophageal dysfunction, sclerodactyly, and telangiectasias.
Diffuse systemic scleroderma
In diffuse systemic scleroderma, more areas of the skin become thick, on any part of the body. Patients have a higher risk of developing sclerosis — or fibrous hardening — of the internal organs early in the disease. They also usually experience rapid worsening of their symptoms.
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