Scleroderma

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This article is about the disease. For the mushroom, see Scleroderma citrinum.

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 eMedicineSubj  = med |
 eMedicineTopic = 2076 |
 DiseasesDB     = 12845 |

}} Scleroderma is a rare, chronic disease characterized by excessive deposits of collagen. Progressive systemic scleroderma or systemic sclerosis, the generalised type of the disease, can be fatal. The localised type of the disease tends not to be fatal. The term 'localised, generalised sclerderma' can be used to describe cases where the disease covers a large area of the body - typically more than 40%.

1 Signs and symptoms
2 Types
3 Therapy
4 Pathophysiology
5 Epidemiology
6 Patients' advocacy
7 External links

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Signs and symptoms

Scleroderma affects the skin, and in more serious cases it can affect the blood vessels and internal organs. The most evident symptom is the hardening of the skin and associated scarring. Typically, the skin appears reddish or scaly in appearance. Blood vessels may also be more visible. Where large areas are affected, fat and muscle wastage will weaken limbs and affect appearance.

The seriousness of the disease varies hugely between cases. The two most important factors to consider are the level of internal involvement (beneath the skin) and the total area covered by the disease. For example, there have been cases where the patient has no more than one or two lesions (affected areas), perhaps covering a few inches. Less serious cases tend not to involve the internal bodily functions.

Cases with larger coverage are far more likely to affect the internal tissues and organs. Where an entire limb is affected, symptoms will almost certainly have serious consequences on the use of that limb. The heart and lungs will be affected when the disease covers this area of the torso. Some patients also experience gastrointestinal problems, including heartburn and acid reflux. Internal scarring may sometimes spread beyond what can be seen by the naked eye.

There is discoloration of the hands and feet in response to cold. Most patients (>80%) have Raynaud's phenomenon, a vascular symptom that can affect the fingers and toes.

Systemic scleroderma and Raynaud's can cause painful ulcers on the fingers or toes which are known as digital ulcers.

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Types

There are three major forms of scleroderma: diffuse, limited (CREST syndrome) and morphea/linear. Diffuse and limited scleroderma are both a systemic disease, whereas the linear/morphea form is localized to the skin. (Some physicians consider CREST and limited scleroderma one and the same, others treat them as two separate forms of scleroderma.)

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Diffuse scleroderma

Diffuse scleroderma is the most severe form - it has a rapid onset, involves more widespread skin hardening, will generally cause much internal organ damage (specifically the lungs and gastrointestinal tract), and is generally more life threatening.

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Limited scleroderma/CREST syndrome

The limited form is much milder: it has a slow onset and progression, skin hardening is usually confined to the hands and face, internal organ involvement is less severe, and a much better prognosis is expected.

The limited form is often referred to as "CREST" syndrome. CREST is an acronym for:

These five are the major symptoms of the CREST syndrome.

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Morphea/linear scleroderma

Morphea/linear scleroderma involves isolated patches of hardened skin - there generally is no internal organ involvement.

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Therapy

There is no cure for scleroderma, though there is treatment for some of the symptoms, including drugs that soften the skin and reduce inflammation. Some patients may benefit from exposure to heat.

A range of NSAIDs (nonsteroidal anti-inflammatory drugs) can be used to ease symptoms, such as Naproxen. If there is oesophageal dysmotility (in CREST or systemic sclerosis), care must be taken with NSAIDs as they are gastric irritants.

Immunosuppressant drugs, such as mycophenolate mofetil (Cellcept®) are sometimes used to slow the progress.

Digital ulcerations can be helped by prostacyclin (iloprost) infusion. Iloprost being a drug which increases blood flow by relaxing the arterial wall.

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Pathophysiology

The cause of the disease is unknown. The overproduction of collagen is thought to result from an autoimmune dysfunction.

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Epidemiology

Scleroderma affects approximately 300,000 people in the United States. It is four times as common in women than in men. Incidence rates are estimated at 2-20 per million per year in the United States.

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Patients' advocacy

The Scleroderma Foundation is a leading organization dedicated to raising awareness of the disease and assisting those who are afflicted. Its national spokesperson is Jason Alexander.

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