Scleroderma is a chronic autoimmune disease characterized mainly by hardening of the skin, although it can also affect internal organs. It is broadly separated into two major groups: localized scleroderma and systemic scleroderma.
In localized scleroderma, usually only the skin and/or underlying muscle tissue is affected. Systemic scleroderma, in contrast, also affects internal organs. Patients can have different symptoms depending on the areas of the body affected and the extent.
The hallmarks of localized scleroderma are inflammation and thickening of the skin due to excessive levels of a structural protein called collagen. Localized scleroderma is relatively mild, and commonly affects only a few areas of the body — usually the skin or muscles. 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. Different types of morphea depend on the parts of the body affected and the patterns of abnormal skin.
The most common form of localized scleroderma in adults, plaque morphea is characterized by limited, round, or oval shaped areas of hard and shiny skin. It can affect one or more parts of the body; most commonly, the torso or parts of the limbs near the torso are affected.
People who have morphea plaques affecting two or more areas on the body are said to have generalized morphea. This form is more common in women.
Bullous morphea is characterized by bullae — a type of blister — on morphea plaques.
People with this subtype have usually a single lesion, typically on the upper trunk near the spine. In deep morphea, the surface skin is not overtly affected, but tissue underneath the skin is. Notably, deep morphea does not usually cause overt symptoms or affect internal organs.
People with more than one different subtype of morphea occurring at the same time are said to have mixed morphea.
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. This scleroderma subtype 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 face, but can also affect muscle and bone. In Parry-Romberg syndrome, half of the face is affected, whereas in en coup de sabre, patients show a line of altered skin, usually across the forehead.
Systemic scleroderma is a multi-system form of the disease. It leads to tissue fibrosis (scarring) and damage to blood vessels. 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 arms, hands, fingers, and face. Some patients have problems in the lungs and gastrointestinal tract. Limited scleroderma usually develops slowly over several years.
CREST syndrome is a subtype of limited scleroderma. CREST is an acronym representing five conditions that can occur with limited scleroderma: calcinosis (calcium deposits that form under the skin), Raynaud’s phenomenon, esophageal dysfunction (that can cause difficulty swallowing), sclerodactyly — think skin on the fingers and sometimes toes — and telangiectasias, which refers to dilated blood vessels that appear as red spots on the fingers, face, and hands.
Diffuse systemic scleroderma
In diffuse systemic scleroderma, more areas of the skin become thicken on any area of the body. These patients are at higher risk of developing sclerosis — or fibrous hardening — of the internal organs early in the disease. They also usually experience more rapid worsening of their symptoms.
Last updated: May 18, 2021
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