Spread the word and truly understand how to help people with Scleroderma by knowing the different variations of this disease
The month of June is recognized as Scleroderma Awareness Month and sharing information about Scleroderma with people who don’t know about the condition can save future patients from living a tough life by letting the disease go untreated. Scleroderma is the Greek term for hardened skin and the condition is prevalent throughout the world and women between 30-50 years of age are more prone to developing it than men. Scleroderma affects the connecting tissues that support the framework of the body and the condition is also more prevalent in Caucasians. Scleroderma might only affect some people on the skin but the condition can sometimes turn dangerous by harming the blood vessels, internal organs and the digestive tract and causing the patient a life of discomfort and depression. Scleroderma can be categorized into two kinds- localized scleroderma and systemic sclerosis.
Localized scleroderma, also known as morphea, affects only the skin of a patient by causing the area to become harder than normal. Children commonly experience this condition and this type of plaque usually results in no other problem apart from causing the appearance to change. This will rectify as they grow up. Treatment is recommended only in severe cases and for patients with the linear type of scleroderma. Localized scleroderma doesn’t usually have any complications unlike the systemic sclerosis form of scleroderma which affects internal organs and their functioning. The exact number of cases regarding localized scleroderma have not been determined, especially because a lot of cases go undetected. Researchers are yet to determine the exact cause of this condition, but studies have concluded that cells known as fibroblasts create too much collagen which gets lodged in the skin to form scarring and thickening. The reason for these fibroblasts creating the excess collagen is unknown but researchers say it could be a fault with the immune system because many cases develop after an autoimmune condition is triggered. The condition is recognized by its appearance and is confirmed after a biopsy is conducted. The doctor may sometimes order an ultrasound or MRI scan to check if the scleroderma is damaging the tissues beneath the skin and x-rays are studied to check if it is hindering the development of bones in children.
Plaque morphea- These are oval shaped patches that are mauve in color when they first appear and gradually turn into a brown color. The patches are up to 20 cm in diameter and the areas affected with this type of localized scleroderma is hairless, shiny and smooth. Adults can be affected with this and it causes no other problem apart from looking unattractive to people not used to seeing someone that has the condition.
Linear scleroderma– A child’s arm or leg is usually affected with this affliction by creating lengthy but short of width areas of hardened skin. Severe cases of this condition can affect the tissues under the skin and cause contractures that can lead to deformity in some cases.
Superficial morphea- This mostly affects middle-aged women and causes patches in areas of folded skin in the armpits, groin or under the breasts. The patches are mauve in color and the condition is very similar to plaque morphea.
En coup de sabre- The term for this type comes from its meaning which is “like a sword cut.” In rare cases, this condition can affect the central nervous system but the common kind affects the scalp and temples by forming a sabre cut, with no hair forming over the scar and causing the skull bone to shrink.
Generalized morphea- This is a rare kind and the plaques are found at a minimum of two or more different parts of the body.
Pansclerotic disabling morphea- Kids are prone to this rare kind of scleroderma and it results in hardened skin and underlying muscles and it may affect the development of the bone by causing joint contractures, chronic ulcers and squamous cell carcinoma.
Progressive facial hemiatrophy- This kind of scleroderma is also known as Parry-Romberg syndrome and it causes tissue to disintegrate under the skin on one side of the face.
Localized scleroderma affects only the skin and in the deeper cases, the tissues, bones and muscles right under the affected area hinders the development of the underlying tissue to cause some forms of deformity. However, only a mere one third of folks with deep localized scleroderma face this problem and in some very rare cases of the “en coup de sabre” type, the eye has been affected.
This is a deadlier kind of scleroderma when compared to localized scleroderma because systemic sclerosis is an autoimmune disease that creates autoantibodies that attack connective tissue cells giving it the name connective tissue disease. The connective tissue binds, supports and differentiates the tissues and organs within the body and when a person is affected with systemic sclerosis, the area beneath the hardened skin can affect the internal organs and its functioning. The exact cause of the condition is yet to be determined though researchers suspect that it may be passed down through generations and is triggered by certain infections or drugs. Some statistics on the condition show that about 6,000 cases have been reported in the UK. Middle-aged women are more prone to it than men. The two kinds of systemic sclerosis are Limited cutaneous systemic sclerosis (lcSSc) and Diffuse cutaneous systemic sclerosis (dcSSc).
The symptoms range from Raynaud’s phenomenon (where the skin reacts strongly to warm or cold temperatures), hardened skin patches, swelling of fingers and toes, ulcers forming on the fingertips and itchy skin accompanied with reduced hair growth. These symptoms are often accompanied with fatigue, weight loss, change in appetite, muscle pains, high blood pressure and coughing or breathlessness owing to scarring of the lung tissue. The diagnosis of this condition is difficult because it grows overtime and can take on different forms. Confirmation is usually done by a rheumatologist who orders for a series of tests including examination of the fingernails- where the capillaries display changes linked to systemic sclerosis, biopsy, blood tests and x-rays.
The common complications of the condition are:
Since there is no cure yet for systemic sclerosis, treatment usually begins with keeping the skin well moisturized and treating problems as they arise. Treatment of suppressing the immune system consists of ingesting steroids, methotrexate, cyclophosphamide, azathioprine, mycophenolate mofetil and ciclosporin. Any pulmonary disorders caused by the condition are treated using medication like bosentan, sildenafil, iloprost and epoprostenol. Blood pressure and kidney problems are treated with angiotensin-converting enzyme (ACE) inhibitors. Antibiotics are prescribed for preventing any infections from spreading and thyroid replacement capsules are given for an underactive thyroid gland. Being well informed about the condition is necessary to determine the symptoms and to diagnose it at an early stage so that precautions are taken to defeat it permanently.
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