Scleroderma: Not A Single Disease

Scleroderma literally means "hard skin", derived from the Greek words sklerosis which means hardness and derma which means skin. Referred to often as a single disease, scleroderma is actually a symptom of a group of diseases complicated by an abnormal growth of connective tissue which supports the skin and internal organs.

Some types of scleroderma have a limited abnormal process, making the skin hard and tight. Other types are more complicated and affect blood vessels and internal organs such as the:

• heart
• lungs
• kidneys

Scleroderma is considered both a rheumatic disease (conditions characterized by inflammation and/or pain in the muscles, joints, or fibrous tissue) and a connective tissue disease (affects the major substances in the skin, tendons, and bones.)

There are two main classes:

• localized scleroderma (affects only certain parts of the body)
• systemic sclerosis (affects the whole body)

Localized Scleroderma

Localized types of scleroderma affect the skin and related tissues, and sometimes the muscle below. Internal organs are not affected. Localized scleroderma can never progress to the systemic type of the disease. Localized types can improve over time, but the skin changes occuring when the disease is active can be permanent. It can be serious and disabling.

Two types exist: Morphea and Linear.

Morphea

Reddish patches of skin that thicken into firm oval-shaped areas are distinctive of the morphea type of localized scleroderma (eMedicine photo). The center of the patches are ivory, with violet borders that can occur on the:

• chest
• stomach
• back
• face
• arms
• legs

The patches typically sweat little and have little hair growth. Morphea too can be localized (limited to one or several patches, ranging from half-inch to 12 inches in diameter) or generalized (skin patches are hard and dark and spread over larger areas of the body.) Morphea generally fades out in 3 to 5 years but people can have dark skin patches and sometimes, though rare, muscle weakness can remain.

Linear

A distinctive single line or band (eMedicine photo) of thickened and/or abnormally colored skin. The line typically runs down an arm or leg but can run down the forehead.

Systemic Sclerosis

This type of scleroderma not only affects skin but also involves blood vessels and major organs.

CREST

People with systemic sclerosis often have symptoms of all of the following which are known as CREST:

• Calcinosis (formation of calcium deposits in the connective tissue)
• Raynaud's phenomenon (small blood vessels of hands and/or feet contract in response to cold or anxiety. Hands or feet turn white, cold, then blue)
• Esophageal dysfunction (impaired function of esophagus occuring when smooth muscles in the esophagus lose normal movement)
• Sclerodactyly (thick and tight skin on fingers resulting from deposits of excess collagen within skin layers)
• Telangiectasias (small red spots on the hands and face caused by swelling of tiny blood vessels)

Systemic sclerosis is broken into two categories, diffuse and limited.

Limited Scleroderma

Limited scleroderma typically has gradual onset and is restricted to certain areas of the skin such as:

• fingers
• hands
• face
• lower arms
• legs

Raynaud's phenomenon is often experienced for years before skin thickening is evident. Others experience skin problems over much of the body, which shows improvement over time, leaving only the face and hands with tight, thickened skin. Calcinosis and telangiectiasias often follow. Limited scleroderma is sometimes referred to as CREST syndrome because of the predominance of CREST symptoms in these patients.

Diffuse Scleroderma

Diffuse scleroderma typically has sudden onset. Skin thickening is quick and covers much of the body, typically in a symmetric pattern. Major internal organs can be damaged. Symptoms common with diffuse scleroderma can include:

• tiredness
• loss of appetite/weight
• joint swelling
• joint pain

Skin can swell, appear shiny, and feel tight and itchy. The damage of diffuse scleroderma occurs over a period of a few years.

After about 3 to 5 years patients tend to stabilize, a phase when progression seems little and symptoms subside, but gradually skin changes begin again. A phase recognized as softening occurs, during which less collagen is made and the body rids itself of excess collagen. The last areas thickened are reversibly softened. Some patients skin returns to what seems like normal while the skin of others becomes thin and fragile. Diffuse scleroderma patients face the most critical prognosis if they develop complications in the:

• kidneys
• lungs
• heart
• digestive tract

Less than one-third of patients with the diffuse type of scleroderma develop these complications.

Sine Scleroderma

Some recognize sine scleroderma as a third category of systemic sclerosis, different from limited or diffuse systemic sclerosis by not affecting the skin.

Related Resources

_{Source: Scleroderma Handout On Health, NIAMS}