Clinical aspects of systemic sclerosis (scleroderma)

Article Abstract:

Systemic sclerosis, or scleroderma, is chiefly characterized by induration and hardening of the skin. Although it was previously considered to be only a process of fibrosis (growth of fibrous, nonfunctional tissue), active inflammation is now thought to occur in this disease, along with microvascular (small blood vessel) and immunological changes. It is possible that products generated during the immune response affect endothelial cells (the hormonally active cells that line blood vessels) and fibroblasts (which occur in fibrosis and also give rise to connective tissue such as skin and cartilage). Advances in understanding medical aspects of scleroderma are reviewed. Scleroderma may be either localized, limited to skin and adjacent tissues, or systemic, potentially involving the lungs, kidneys, digestive tract, and heart. The systemic disease is subclassified according to whether skin involvement is diffuse, especially affecting the trunk, or limited. Symptoms of scleroderma are discussed. Raynaud's phenomenon (change of finger color to white and then possibly blue and red in response to cold or emotion) occurs in 95 percent of patients with the disease. Patients with the disorder should be tested for blood vessel abnormalities, observed by nailfold capillary microscopy, and for the presence of antinuclear antibodies, or antibodies inappropriately formed against molecules found in the cell nucleus. Skin changes usually occur first with edematous (fluid accumulation) changes, followed by tightening and then atrophy. Gastrointestinal disease usually features dysphagia (difficulty swallowing) and heartburn and is the most common organ system involved, followed by lung disease, which results in dyspnea (difficulty breathing) and hypoxia (low oxygen levels). Kidney dysfunction occurs in 45 percent of patients and is associated with the highest death rate. Heart disease may occur in a variety of forms, all resulting from vascular involvement and fibrosis. Development of the disease is likely related to genetic, connective tissue, vascular, and immunological influences. Special aspects of localized scleroderma, eosinophilia-myalgia syndrome, and childhood scleroderma are discussed. (Consumer Summary produced by Reliance Medical Information, Inc.)

Evaluation

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Raynaud's phenomenon: its relevance to scleroderma

Article Abstract:

Raynaud's syndrome is defined as transient ischemia (low tissue oxygenation due to diminished blood flow) of the fingers occurring in response to cold and emotion, usually shown by whitening, then by cyanosis (bluish discoloration) and reddening of the skin. The whitening is caused by vasospasm (blood vessel constriction), while the blue color is due to removal of oxygen from the immobile pool of blood, and the reddening is caused by hyperemia (increased blood flow) in reaction to the vasospasm and ischemia. This disorder may have a good prognosis when it is unrelated to other conditions (primary Raynaud's disease), but it may be associated with other disorders such as connective tissue diseases (Raynaud's syndrome). A portion of patients with Raynaud's phenomenon progress to systemic sclerosis, or scleroderma; the estimated incidence varies. It is important to identify patients at risk for systemic sclerosis as early as possible. However, the age at onset, skin hardening, scars, or ulceration are strong clues that there is a high risk of developing disease. Capillary (very small blood vessels) abnormalities shown by nailfold capillary microscopy, the presence of particular autoantibodies (antibodies inappropriately made against the body's own molecules), and alterations in blood viscosity, clotting, and release of the protein beta thromboglobulin by platelets may also indicate an increased risk of scleroderma. It is possible that the microvascular changes in Raynaud's phenomenon may underlie the development of fibrosis (growth of fibrous, nonfunctional tissue) in systemic sclerosis. Treatment of mild Raynaud's syndrome includes protection from cold, including use of electrically or chemically heated hand warmers, gloves and socks, and use of comfort shoes to relieve pressure on toes. Severe disease is treated by drugs, including vasodilators such as nifedipine, prostaglandins, and ketanserin, and potentially by drugs that decrease blood viscosity and alter clotting. (Consumer Summary produced by Reliance Medical Information, Inc.)

Development and progression, Raynaud's disease

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Scleroderma 'en coup de sabre'

Article Abstract:

Scleroderma is a skin disease that causes the skin to become tough and leathery. Scleroderma en coup de sabre is a less common form of scleroderma that affects the skin on the face and scalp. It causes the skin to degenerate and hair to fall out. Also, it causes bone and muscles to atrophy. This disease has also been reported to cause seizures. It is not known what causes this disease, but surgery, infection and severe psychological stress have been reported prior to its onset. The case is reported of a four-year-old boy who developed the condition. His symptoms began with pain, redness and inflammation in his left eye. Corticosteroids were prescribed but were not very effective in treating the condition. Over the next two years he had headaches in his forehead, and subsequently experienced a grand mal seizure. Electroencephalography (EEG, a technique used to record electrical impulses in the brain) indicated the presence of inflammation in the brain (encephalitis). The patient was treated with sodium valproate, but continued to have seizures. Further studies revealed the presence of inflammation in blood vessels and the patient was treated with prednisolone. The patient's eye condition improved over the next two months, but the seizures continued. A groove in the skin developed on the top part of the patient's forehead and it extended down to the eyebrow. The patient was diagnosed as having scleroderma en coup de sabre and was treated with penicillamine. During the following six months of treatment the groove in the skin was prevented from increasing in size and the seizures were controlled by epileptic medications. (Consumer Summary produced by Reliance Medical Information, Inc.)

Case studies

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