Scleroderma refers to a range of disorders in which the skin and connective tissues tighten and harden. It is a long-term, progressive disease. This means it gradually gets worse.
It is considered a rheumatic disease and a connective tissue disorder. It is also thought to be an autoimmune condition, in which the body’s own immune system attacks the body’s tissues.
This results in an overproduction of collagen, the protein that forms the basis of connective tissue. The result is a thickening, or fibrosis, and scarring of tissue.
Scleroderma is not contagious. It may run in families, but it often occurs in patients without any family history of the disease. It ranges from very mild to potentially fatal. Up to 1 in 3 people with the condition develop severe symptoms.
It is thought to affect between 75,000 and 100,000 people in the United States, mostly women aged from 30 to 50 years.
Scleroderma means “hard skin.”
Early symptoms of scleroderma include changes in the fingers and hands, for example, stiffness, tightness, and puffiness because of sensitivity to cold or emotional stress.
There may be swelling in the hands and feet, especially in the morning.
Overall symptoms of scleroderma include:
calcium deposits in connective tissues
a narrowing of the blood vessels to the hands and feet, known as Raynaud’s disease
problems of the esophagus, which links the throat and stomach
tight, thickened skin on the fingers
red spots on the face and hands
However, symptoms will vary according to the type and how it affects the person, and whether it affects one part of the body or a whole body system.
The two main types of scleroderma are localized and systemic.
Localized scleroderma mainly affects the skin, but it may have an impact on the muscles and bones.
Systemic scleroderma affects the whole body, including the blood and internal organs, and especially the kidneys, esophagus, heart, and lungs.
Localized scleroderma is the mildest form of scleroderma. It does not affect the internal organs. There are two main types: morphea and linear scleroderma.
Morphea: Symptoms include oval-shaped patches of lighter or darker skin, which may be itchy, hairless, and shiny. The shapes have a purple border, and they are white in the middle.
Linear scleroderma: There may be bands or streaks of hardened skin on the limbs, and rarely the head and face. It can affect bones and muscles.
Systemic scleroderma affects the circulation of the blood and the internal organs.
There are two main types:
limited cutaneous systemic sclerosis syndrome, or CREST
diffuse systemic sclerosis
Limited cutaneous systemic sclerosis
Limited cutaneous systemic sclerosis is the least severe type of systemic scleroderma. It affects the skin on the hands, feet, face, and lower arms and legs. There may be problems with the blood vessels, lungs, and the digestive system.
It is sometimes known as CREST syndrome, because the symptoms form the acronym, CREST:
C: Calcinosis, or calcium deposits in tissues and under the skin
R: Raynaud’s disease
E: Esophageal problems, including GERD
S: Sclerodactyly, or thick skin on the fingers
T: Telangiectasias, or enlarged blood vessels, manifesting as red spots
The first sign is often Raynaud’s disease, in which blood vessels narrow in the hands and feet, leading to circulatory problems in the extremities. Patients may experience numbness, pain, and color changes in response to stress or cold.
The skin on the hands, feet, and face may start to thicken.
The impact on the digestive system can lead to difficulty swallowing and gastroesophageal reflux disease (GERD), or acid reflux.
The intestinal muscles may be unable to move food through the intestines properly, and the body may not absorb nutrients.
Some of these symptoms can have other underlying causes. Not everyone who has Raynaud’s disease or GERD will have scleroderma. Most do not.
Sclerodactyly: What it is, causes, and treatment
Find out more about sclerodactyly, which affects the fingers
Diffuse systemic sclerosis
In diffuse systemic sclerosis, the skin thickening usually affects the area from the hands to above the wrists. It also affects the internal organs.
People with systemic types of scleroderma may experience weakness, fatigue, difficulty breathing and swallowing, and weight loss.