Causes and risk factors
Scleroderma is an autoimmune disorder. In this, the body attacks its own cells thereby giving rise to symptoms. The cause of autoimmunity is not known.
Clinical presentation
Skin changes: Straight lines or oval patches of skin with thickening, tightening and hardening. Glossy or shiny appearance of the skin. Restriction of movement of the affected area.
Raynaud’s phenomenon: In Raynaud’s phenomenon, exposure to cold environment results in color changes in the fingers due to temporary reduction of blood flow. This can lead to can lead to swelling and numbness and discoloration of the fingers and toes. People with other diseases can also have Raynaud’s and some people with Raynaud’s do not have any other disease.
Telangiectasias: Enlarged red blood vessels on the hands, face and around nail beds
Digestive problems such as dysphagia, bloating, heartburn and constipation may be present. Other symptoms due to heart, lungs or kidney problems may be seen.
The complications of scleroderma include:
- Lung – pulmonary fibrosis, pulmonary hypertension
- Skin and circulatory disturbances – ulcers or gangrene at fingertips which may need to be amputated
- Heart – arrhythmias, pericarditis, congestive heart failure
- Renal failure, myositis and sexual problems in women
Investigations
Scleroderma must be differentiated from conditions like graft versus host disease, eosinophilic fasciitis, mixed connective tissue disease and nephrogenic systemic fibrosis
In addition, localized scleroderma can resemble several local pathologies affecting that particular body structure.
After a thorough history taking and physical examination, certain tests may be advised. Blood tests are done to check for antibodies. Biopsy of the affected portion of the skin is carried out. Other investigations include ECG, pulmonary function tests and CT scan of the lungs.
Treatment
The primary aims of treatment for scleroderma are relief in the symptoms and prevention of complications.
Physical therapy helps to keep your joints supple
Medications: Since scleroderma can affect any part of the body and produce varying symptoms at each location, a large number of drugs are useful in treatment as and when required. These include antibiotics, antacids, H2 blockers, proton pump inhibitors, painkillers, antihypertensive drugs, histamines and antidepressants.
Surgery may be necessary in case of complications e.g. lung transplant, amputations etc.
Patients with diffuse sclerosis have a very poor prognosis. The prognosis of localized sclerosis is fairly good with a 10 year survival rate of 60-70%.
There is no specific prevention against this condition. To prevent complications, the patient:
- Must not smoke
- Must not expose himself to cold weather conditions
- Must treat any wound or ulcer promptly
