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HEALTH: Why developing dry, hard skin is harmful

An auto-immune disease, scleroderma mostly affects women. Awareness is important as it can be fatal and can’t be cured
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Discolouration of skin is a feature of scleroderma.
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Shefali Sharma

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In her twenties, Monica would experience bluish discolouration of her fingertips during winter. She didn’t give it much thought, attributing it to cold weather. However, the problem worsened after a couple of years and her hands started becoming cold and discoloured during summer as well. Gradually, she also noticed that the skin on her face and hands was thickening. She consulted her family doctor, but there was no improvement. Finally, she sought treatment at the PGI, Chandigarh. By that time, the skin on her hands, arms, legs and face had already thickened. She would also feel breathless while walking. Monica was diagnosed with a rare disease called systemic sclerosis or scleroderma. She had to quit her job, as these issues were impacting her normal life.

Systemic sclerosis (SSc), or scleroderma, is an autoimmune multi-organ disease characterised by fibrosis (thickening and scarring of connective tissue) of skin and internal organs, including blood vessels, muscles and joints, gastrointestinal (GI) tract, kidneys, lungs and heart and immune dysfunction. There is dearth of data because of the rarity of the disease, but available studies put the number of cases to be 120 per 10 lakh Indian population. It also affects women more than men.

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  • Eat small, frequent meals. Have early & light dinner.
  • If gastro esophageal reflux persists, then elevation of bed on the head-side is helpful.

Skin symptoms

  • Use creams, vaseline, coconut oil to lubricate the skin.

    • Blood pressure should be kept under control.
    • Corticosteroids should not be given unnecessarily.
    • Renal function tests should be routinely performed.

    Musculoskeletal Involvement

    • Patients suffer from aches and joint pains. There’s difficulty in opening and use of hands. Immuno-suppresants are useful. (For further information, visit www.sclerodermaindia.com)

    The disease or its subtypes are often characterised by the extent of skin involvement. When skin thickens on the trunk, face and other parts of the body, it’s called diffuse cutaneous scleroderma.

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    In limited cutaneous scleroderma, skin hardening and thickening is confined usually to the fingers and sometimes, the hands, forearms or the face. This tightening causes loss of flexibility and ease of movement, especially in the fingers and face, reducing facial movements, including decreasing the mouth opening.

    Many studies have also found association between systemic sclerosis and cancer, suggesting common genetic and environmental risk factors involved in the development of both diseases. Immunosuppressive drugs widely used in systemic sclerosis may increase the risk of cancer occurrence.

    Factors

    The auto-immune disease could result due to genetic factors and/or environmental factors like exposure to silica or heavy metals such as mercury; organic chemicals like vinyl chloride, benzene, toluene; and certain drugs such as bleomycin, pentazocin, taxol or appetite suppressants like L-tryptophan, mazindol, fenfluramine, etc.

    Symptoms, associated risks

    Most patients may also experience Raynaud’s phenomenon, an exaggerated response to temperature or emotional stress. Exposure to cold causes bluish discolouration of the fingers. Other symptoms include chronic joint pain, morning stiffness, inflammation and swelling in muscles and joints. Skin sores can occur due to lack of proper blood flow. Skin loses hair, oil and sweat glands and is dry, itchy and coarse.

    In severe cases, the skin thins and ulcerates easily, predisposing to infection. When the disease affects the face, it can cause pursed lips, puckered mouth, pinched nose and mask-like face.

    There could be difficulty in swallowing, intermittent bloating, abdominal cramps, chronic diarrhoea, severe constipation, etc.

    Shortness of breath and non-productive cough are typical features of pulmonary fibrosis. Cardiac disease is a major clinical cause of death in systemic sclerosis, affecting 10 per cent of the total cases.

    Scleroderma renal crisis (SRC) occurs in 10 per cent patients with diffuse scleroderma. Patients treated with high-dose glucocorticoids are at the greatest risk. It is characterised by a sudden onset of malignant hypertension, renal failure and proteinuria (elevated protein levels in urine).

    In Monica’s case, after six months of treatment, she stabilised and responded remarkably. She now leads a normal life.

    — The writer is Professor, Department of Internal Medicine, PGI, Chandigarh

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