Symptoms of Scleroderma

Scleroderma (sklere-o-DER-muh) is a rare, progressive disease that leads to hardening and tightening of the skin and connective tissues — the fibers that provide the framework and support for your body. Scleroderma usually starts with a few dry patches of skin on the hands or face that begin getting thicker and harder. These patches then spread to other areas of the skin. In fact, scleroderma literally means "hard skin."

In some cases, scleroderma also affects the blood vessels and internal organs. Scleroderma is one of a group of arthritic conditions called connective tissue disorders. In these disorders, a person's antibodies are directed against his or her own tissues.

Researchers haven't established a definitive cause for scleroderma. It's more common in women than in men and more common in adults than in children. Scleroderma can run in families, but in most cases it occurs without any known family tendency for the disease. Scleroderma isn't considered contagious or cancerous, but this chronic condition can greatly affect self-esteem and the ability to accomplish everyday tasks.
Signs and symptoms

In addition to thickening and hardening of your skin, scleroderma can cause your skin to lose its elasticity and become shiny as it stretches across underlying bone. Other signs and symptoms may include:

Numbness, pain or color changes in your fingers, toes, cheeks, nose and ears, often brought on by cold or emotional distress (Raynaud's phenomenon)
Stiffness or pain in your joints and curling of your fingers
Digestive problems ranging from poor absorption of nutrients to delayed movement of food due to impaired muscular activity in your intestine
Sores over joints, such as your elbows and knuckles
Puffy hands and feet, particularly in the morning
Causes
Collagen is a fibrous type of protein that makes up your body's connective tissues, including your skin. Scleroderma results from an overproduction and accumulation of collagen in body tissues.

Although doctors aren't sure what prompts this abnormal collagen production, the body's immune system appears to play a role. For unknown reasons, the immune system turns against the body, producing inflammation and the overproduction of collagen. In addition to its effects on your skin, some types of scleroderma affect tiny blood vessels and can affect almost every organ.

Types of scleroderma
Doctors classify scleroderma into different subsets:

Localized scleroderma
This type of scleroderma is limited to your skin and the deep tissues below your skin. It includes the following subclassifications:

Morphea. In this form, oval-shaped thick patches appear on your skin — white in the middle, with a purple border. These patches are most likely to occur on your torso, but they can also appear on your arms, legs or forehead.
Linear scleroderma. This form results in bands or streaks of hardened skin on one or both of your arms or legs, or on your forehead.
Systemic scleroderma
This type of scleroderma affects not only your skin but also your blood vessels and major organs. It's also called systemic sclerosis and includes the following subclassifications:

Diffuse cutaneous systemic sclerosis. This type affects the skin of your fingers, hands, arms, legs, face, neck and trunk. It can also affect internal organs, such as your lungs, heart, kidneys and gastrointestinal tract — including your esophagus. It can hinder the functions of your digestive system, create respiratory problems and cause kidney failure. When left untreated, systemic scleroderma may be fatal within several years of onset.
Limited cutaneous systemic sclerosis. This type involves the skin of your fingers, lower arms and legs, face, and neck. A variation is called CREST syndrome.
Sine scleroderma. Some doctors may describe one variation of systemic scleroderma as sine scleroderma, which can be similar to either limited or diffuse scleroderma, the difference being that this form doesn't affect your skin.
Overlap syndrome
This is diffuse or limited systemic sclerosis with features of one or more of the other connective tissue diseases.

Undifferentiated connective tissue disease
This has features of systemic sclerosis, but there are no clinical or specific laboratory findings to make a definite diagnosis.