Scleroderma is a widespread disease of the connective tissue and occur by changes in the skin, blood vessels, muscles and internal organs.
CREST syndrome, progressive systemic sclerosis, systemic sclerosis, Localized scleroderma
Causes, incidence, and risk factors:
The cause of scleroderma is unknown. People who suffer from this disease have a high concentration of a substance called collagen, in the skin and other organs. This concentration leads to symptoms that associated with the disease.
The disease usually affects people 30-50 years old. The women suffer from scleroderma more often than men. In risk factors included exposure, because of work, to silica dust and polyvinyl chloride.
In skin symptoms include:
• Fingers and toes whiten, bruise or red in heat and cold (Raynaud phenomenon)
• The hair are falling
• The skin hardens
• The skin is abnormally dark or light
• The skin is fattening and shines in the hands and arms
• Showing little white nodules under the skin
• The skin of the face is inflexible and looks like a mask
• Developed ulcers on the fingertips or toes
In bone and muscle symptoms include:
• Joint pain
• Numbness and pain in the legs
• Pain, stiffness, and swelling of fingers and joints
• Wrists pain
In respiratory problems include:
• Dry cough
• 'Whistle' in breathing
In gastrointestinal problems include:
• Bloating after meals
• Difficulty in swallowing
• Oesophageal reflux or heartburn
• Weight Loss
As for the eyes, additional symptoms associated with this disease are:
• Burning, itching and secresions
The skin on the test shows inflexibility, fattening and hardening. In diagnostic tests include:
• Antinuclear antibodies
• Chest X-ray
• Computed tomography of lung
• Blood sedimentation rate
• A blood test for rheumatoid factor
• Study of pulmonary function
• Skin Biopsy
Medications for treatment of scleroderma include:
• Immunosuppressive (methotrexate, cyclophosphamide)
• Non-steroidal anti-inflammatory drugs
Other treatments for specific symptoms may include:
• Antacids for heartburn
• antihypertensive drugs (particularly ACE inhibitors) for high blood pressure or kidney problems
• Bronchodilators for facilitating breathing
• Medicines to treat Raynaud phenomenon
The treatment usually consists with a combination of physical therapy and skin and joints precautionary measures and joints (eg, somebody who suffers from the Raynaud phenomenon need to avoid the cold).
In most patients, the disease gradually worsens. People who infected only in the skin have better progress. Death can occur if infected the gastrointestinal, heart, kidneys or lungs.
One type of scleroderma, called localized scleroderma, linked only to skin problems of the hands and face. Deteriorates very slowly and usually does not affect any other part of the body.
Systemic scleroderma can infect many organs in the body. In some people, it has a slow development and will not affect any vital organ. In others, however, will infect organs such as the lungs, kidneys, intestines, gallbladder and heart.
In some cases, symptoms and problems develop quickly during the early years and continuously deteriorate. In other cases, deterioration is much slower. The complications in the lungs is the most common cause of death in patients with scleroderma.
• Heart failure (syncope)
• Kidney failure
• Incomplete (intestinal) absorption
• Pulmonary fibrosis (the most common cause of death in patients with scleroderma)
• Pulmonary hypertension
When to contact your doctor:
• Have symptoms of scleroderma
• You suffer from scleroderma and symptoms become worse or develop new symptoms
There is no known way to prevent. Minimize the exposure to silica dust and polyvinyl chloride.