Diagnosis of Scleroderma

Disease Progression in Scleroderma

Localized Scleroderma

Following the initial symptoms of inflammation, loss of hair, and anhidrosis (reduced sweating capacity), over time the skin hardens and may take on an ivory color. The center of the lesion progresses to a yellowish color and begins to pit and, over time, the lesion becomes white in the middle and is surrounded by sclerotic plaque. Muscle and bone beneath the lesions can be affected. The greater the numbers of these lesions, the more severe the case of localized scleroderma. Involvement of organs beyond muscle and bone is rarely seen in localized scleroderma.

Linear scleroderma usually is limited to one side of the body but can spread. When it occurs in young children during an active growth periods, linear scleroderma can result in the affected limb growing slower than the opposite, unaffected limb.

Most reports indicate that while localized scleroderma is progressive for up to two years before it may spontaneously recede, it can continue to be active and spread for several years. If the disease is active at certain ages of growth in children, the lesions can lead to disfigurement and functional damage.

Limited Systemic Scleroderma

CREST symptoms present early on in the development of limited systemic scleroderma. The calcinosis which may form under the skin may appear as white spots and can be very painful. Following the localized inflammation of the skin, the lesion may spread and form a ring-like lilac colored appearance after the inflammation clears. Over time, the skin may harden and take on an ivory color. Usually there is loss of hair and anhidrosis in the lesion. As a general rule, the more extensive the original area of inflammation, the greater the potential effect on muscle and bone in that area. Involvement of organs beyond muscle and bone is rarely seen with limited scleroderma. Studies suggest that while limited scleroderma is progressive for the first 1-2 years, it can continue for several years, though progression is usually slow.

Diffuse Systemic Scleroderma

Following Raynaud's phenomenon and inflammation, the skin becomes thicker, hard, leathery and/or shiny and can become very itchy. Hands and fingers may swell. The duration of this stage varies widely from patient to patient. Eventually the inflammation stops and the skin becomes thinner and may soften and eventually may bind to underlying bones or muscles. Painful ulceration at the joints is common. Organ damage usually occurs during the most active part of the disease process. Organ involvement of the lungs, heart, gastrointestinal tract, or kidney can cause significant illness and must be diagnosed and treated in a timely fashion to prevent further complications.