Read more about this disease, some with Classification – Types – Signs and symptoms – Genetics – Pathophysiology – Diagnosis – Screening – Prevention – Treatment and management – Cures and much more, some including pictures and video when available.
Scleroderma is a chronic autoimmune disease characterized by a hardening[1] or sclerosis[2] in the skin or other organs. The localized type of the disease, known as “morphea”,[3] while disabling, tends not to be fatal. The systemic type or systemic sclerosis, the generalized type of the disease, can be fatal, as a result of heart, kidney, lung or intestinal damage.[4] It is currently not fully understood what exactly causes this disease, although there are various theories.
Scleroderma is characterized by the appearance of circumscribed or diffuse, hard, smooth, ivory-colored areas that are immobile, and which give the appearance of hidebound skin, a disease occuring in both localized and systemic forms[5]:
In the skin, scleroderma causes hardening and scarring. The skin may appear tight, reddish or scaly. Blood vessels may also be more visible. Where large areas are affected, fat and muscle wastage may weaken limbs and affect appearance. Also, patients report substantial, even severe and recurrent itching of large skin areas, the source of much affliction as the condition worsens. There is much variation in severity between patients, with some having scleroderma of only a limited area of the skin (such as the fingers) and little involvement of the underlying tissue; while others have progressive skin involvement.[6]
Diffuse scleroderma can cause musculoskeletal, pulmonary, gastrointestinal, renal and other complications.[4]Patients with larger amounts of cutaneous involvement are more likely to have involvement of the internal tissues and organs. Most patients (over 80%) have vascular symptoms and Raynaud’s phenomenon, which leads to attacks of discoloration of the hands and feet in response to cold. Raynaud’s normally affects the fingers and toes. Systemic scleroderma and Raynaud’s can cause painful ulcers on the fingers or toes which are known as digital ulcers. Calcinosis (deposition of calcium in lumps under the skin) is also common in systemic scleroderma, and is often seen near the elbows, knees or other joints.
The first joint symptoms that patients with scleroderma have are typically non specific joint pains, which can lead to arthritis, or cause discomfort in tendons or muscles.[4] Joint mobility, especially of the small joints of the hand, may be restricted by calcinosis or skin thickening.[7] Patients may develop muscle weakness, or myopathy, either from the disease, or its treatments.[8]
Some impairment in lung function is almost universally seen in patients with diffuse scleroderma on pulmonary function testing;[9] however, it does not necessarily cause symptoms, such as shortness of breath. Some patients can develop pulmonary hypertension, or elevation in the pressures of the pulmonary arteries. This can be progressive, and lead to right sided heart failure. The earliest manifestation of this may be a decreased diffusion capacity on pulmonary function testing.
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