Scleroderma
Scleroderma Face
Scleroderma Face
Scleroderma Hand
Scleroderma Hand
Scleroderma Ulcers
Scleroderma Ulcers

Scleroderma is an autoimmune disease with tight, thick, firm, hard, hidebound skin along with involvement of various internal organs such as lungs, gastrointestinal tract, heart and kidneys. It is a rare disease which affects 8-15 adults per million in UK and 276 adults per million in USA. The disease can affect children too. The exact incidence in India is not known and it seems that milder cases remain undiagnosed. Females are affected more often (3:1) than males.
The exact cause of this disease is not known although some agents such as organic solvents, hair dyes, metal dust, pesticides, drugs (bleomycin) and some viruses appear to be related to development of skin thickening. Damage to small blood vessels is the primary event associated with their obliteration. Immune activation in the form of inflammation and appearance of certain auto antibodies is another event although auto antibodies are not directly involved in the disease process. Fibrosis (increase of scar tissue) of skin and internal organs is the final event giving rise to various problems in these patients.

Two main types of scleroderma are localised and systemic. Localised scleroderma (morphea, en coup de sabre, etc) is usually restricted to skin and is just a cosmetic problem. Deeper and extensive involvement in localised scleroderma can interfere with joint movements and daily activities. Localised scleroderma does not involve internal organs and is never life threatening.Two main types of scleroderma are localised and systemic. Localised scleroderma (morphea, en coup de sabre, etc) is usually restricted to skin and is just a cosmetic problem. Deeper and extensive involvement in localised scleroderma can interfere with joint movements and daily activities. Localised scleroderma does not involve internal organs and is never life threatening.

Systemic scleroderma is a multisystem disease in which skin involvement can be limited (face, neck and beyond elbows/knees) or diffuse (generalised). It usually starts as fever, fatigue, malaise and other generalised complaints as well as puffy fingers and hands, Raynaud’s phenomena (pallor of fingertips followed sequentially by blue and pink discoloration on cold exposure) and heartburn. The skin becomes thick and tight with increased or decreased skin pigmentation and decreased secretion of sweat and oil (dry skin). The tightness of skin increases slowly and leads to peculiar facial appearance and slender stiff fingers. Inability to open mouth (‘mauskopf’ face), extend neck and move fingers are frequent problems due to tightness of skin in these areas. This process stops after 1-3 years and skin becomes thinner with regrowth of hair on forearms and decrease in tightness. Painful pitting ulcers at tips of fingers and toes can complicate the situation as they heal very slowly, can get infected, involve bone and may require amputation.
Organ involvement in systemic scleroderma indicates severe disease, poorer prognosis and need for aggressive rheumatological therapy. Clinical features of common organ involvements are listed below:Organ involvement in systemic scleroderma indicates severe disease, poorer prognosis and need for aggressive rheumatological therapy.

Clinical features of common organ involvements are listed below:

  1. Gastrointestinal - Difficulty in swallowing, heartburn, regurgitation, chronic throat irritation, shrinking of gums, loss of teeth enamel, bloating, early satiety, nausea, vomiting, change of bowel habits, constipation, pain in abdomen.
  2. Lungs - Dry cough, breathlessness on exertion, swelling of feet, blood in sputum.
  3. Heart - Chest pain, fainting, dizziness, palpitations, increasing breathlessness.
  4. Kidneys - Sudden rise in blood pressure (headache, fits, breathlessness, blurring of vision), low urine output.

Scleroderma has no effect on fertility or pregnancy although pregnancy can increase risk of complications in these patients.

Laboratory Tests:
The diagnosis of scleroderma is based on clinical examination and there is no laboratory test for establishing the diagnosis. Blood tests for auto-antibodies helps in making the diagnosis but this information may not be conclusive. Many other tests are essential to diagnose and monitor organ involvement. These include tests for kidney function (urine and blood), chest X-ray, Pulmonary function tests with DLCO, High resolution CT-scan of chest, Bronchoscopy and examination of aspirated fluid, Gastroscopy, Oesophageal manometry, Xylose breath test, Tread-mill stress test, Echocardiography, MRI and perfusion studies of heart, Holter monitoring for rhythm of heart beats and right heart catheterization. Some of these tests may be required to be repeated during follow up to assess response to therapy.
Treatment:
There is no effective treatment that can arrest or reverse the disease. Early recognition of organ involvement is necessary to avoid life-threatening complications. Various drugs effectively control organ involvement in this disease and are, therefore, used as needed. Immunosuppressants like methotrexate and cyclophosphamide are beneficial for skin and lung involvement and are shown to improve survival. Glucocorticoids (steroids) are of no use and may precipitate life-threatening kidney disease. Good drugs are now available for control of gastrointestinal and cardiac complications. Significant research is ongoing to develop new therapies. Haemopoietic Stem Cell Transplant has shown encouraging results in properly selected cases.

Patients with Raynaud’s phenomena and finger tip ulcers must stop smoking, protect from cold exposure (wear gloves and socks) and keep them warm. Protection of skin from trauma is essential. Skin must be adequately hydrated (use moisturizers), washed carefully and properly disinfected. The ulcers should be kept open as blocking of gas exchange causes ‘green house effect’ and delays healing. Patients with Raynaud’s phenomena and finger tip ulcers must stop smoking, protect from cold exposure (wear gloves and socks) and keep them warm. Protection of skin from trauma is essential. Skin must be adequately hydrated (use moisturizers), washed carefully and properly disinfected. The ulcers should be kept open as blocking of gas exchange causes ‘green house effect’ and delays healing.

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