Localized Scleroderma
The symptoms of localized scleroderma are isolated to the skin and underlying tissues. Two types are recognized: morphea and linear scleroderma.
- Morphea: These are local patches of hardened skin. Red patches of skin develop white centers with purplish borders. Lesions remain active for weeks to several years. Spontaneous softening that leaves a darkened area of skin often occurs. In localized morphea, you will have one or a few patches, whereas in generalized morphea, the patches may grow over large areas of the body. Morphea can occur at any site. Occasionally the lesions can be extensive and gradually evolve into generalized morphea in which the patches extend over most or all of the body surface.
- Linear: A single line or band of thickened and discolored skin develops. The line usually runs down an arm or leg, but sometimes it runs down the forehead. When the band runs down the forehead, it may be called en coup de sabre (“sword stroke” in French).
This disease affects not only the skin and underlying tissues, but also affects the blood vessels and major organs of the body. Two types of systemic disease are recognized: limited and diffuse.
- Limited: In this form, skin thickening is generally limited to the fingers, forearms, legs, face and neck. Raynaud’s phenomenon may be present for years before any other symptoms develop. People with this form are less likely than people with diffuse disease to develop severe organ involvement. CREST syndrome falls within the limited form of the disease and is named after its primary features: calcinosis, Raynaud’s phenomenon, esophageal dysmotility, sclerodactyly and telangiectasia. These symptoms will be discussed below.
- Diffuse: In this form, skin thickening may occur anywhere on the body, including the trunk. Only a short interval of time will elapse between the onset of Raynaud’s phenomenon and significant organ involvement. Damage typically occurs over the first three to five years, after which most patients enter a stable phase that varies in length. During this phase, your skin will stay about the same and the rate of damage to internal organs slows or stops. After the stable phase is over, your skin will start to soften and more serious damage to internal organs is unlikely to occur.
- Raynaud’s phenomenon: Cold temperatures or emotional distress cause the blood vessels feeding the hands and/or feet to constrict. This constriction will cause the hands and feet to feel cold, turn white then turn blue. Once the vessels open back up (after approximately 10 to15 minutes), the hands turn red or mottled. More than 90 percent of people with scleroderma have Raynaud’s phenomenon; it is often the first symptom of the disease. Skin ulcers on the fingertips can result from Raynaud’s phenomenon.
- Skin changes: In the earliest stage of disease, your skin may appear mildly inflamed and swollen. The skin will progressively tighten and flexibility will decrease. As the disease progresses, the skin will become more thickened, sweat and oil glands will stop functioning, and the skin surface will become very dry and itchy. This stage will persist for one to three years, at which time the skin will begin to soften and thin.
- Sclerodactyly: This is a term for the thickening of the skin of the fingers or toes.
- Calcinosis: Calcium deposits can form under the skin or in the muscles. These deposits may cause recurrent inflammation or ulcers on the overlying skin.
- Telangiectasias: Small blood vessels called capillaries near the surface of the skin can widen and be seen through the skin. These are not dangerous, but can be unsightly.
- Arthralgias and myalgias: Pain and stiffness of the joints and muscles are common symptoms early in the course of systemic sclerosis. Later in the disease course, muscle atrophy and weakness are common.
- Dental disease: If the skin around your mouth tightens, you may not be able to open your mouth very wide. You may also have a dry mouth due to secondary Sjögren’s syndrome. [link] Damage to connective tissues in the mouth can lead to loose teeth. These problems can lead to periodontal disease, tooth loss and difficulty chewing, which can compromise your nutritional status.
- Esophageal dysmotility: Smooth muscles in the esophagus lose normal movement, causing swallowing difficulties and chronic heartburn.
- Gastrointestinal dysfunction: Dysmotility along the whole gastrointestinal tract can cause reflux, early satiety, bloating, nausea, vomiting, cramping abdominal pain, diarrhea, weight loss and malnutrition.
- Lung disease: Shortness of breath or difficulty breathing with exertion are the most common initial symptoms when your lungs have become involved with systemic sclerosis. The disease can cause both pulmonary fibrosis (hardening of the lung tissue due to collagen excess) and pulmonary hypertension (high blood pressure in the artery that carries blood from the heart to the lungs). Lung impairment is the leading cause of death in systemic sclerosis. Getting your lung function tested regularly is important for detecting lung disease early.
- Kidney disease: Renal crisis is characterized by accelerated hypertension (high blood pressure) and rapidly progressive kidney failure. About 80 percent of cases of renal crisis occur within the first four or five years of disease, usually in people with diffuse disease.
- Heart disease: Symptoms of heart disease are not seen until late in the course of systemic sclerosis. Symptoms include breathlessness on exertion, palpitations and, less frequently, chest pain.
- Other signs and symptoms:
- Fatigue
- Appetite and weight loss
- Depression
- Sexual dysfunction
- Impotence
- Sjögren’s syndrome
- Carpal tunnel syndrome
- Thyroid disorders
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