Dermatology, Rheumatology, Paediatric (Pediatric) Rheumatology


Introduction to Scleroderma

This is a rare autoimmune condition which results in the hardening and thickening of areas of the skin.

Written by Doctify Team 27/04/2020

What is Scleroderma?

This is a rare autoimmune condition which results in the hardening and thickening of areas of the skin. The body’s own immune system attacks the connective tissue which lies under the skin and around internal organs and blood vessels. Localised scleroderma is a mild form of the disease which may resolve itself whereas systemic scleroderma is more severe and can be life threatening. Children more commonly get localised scleroderma and mainly women between 30 and 50 years first present with systemic scleroderma, though men can also be affected.

What are the causes for Scleroderma?

Scleroderma is caused by the body’s own immune system attacking the body’s connective tissue. Why this happens however is unclear.

What are the symptoms of Scleroderma?

Localised scleroderma just affects the skin causing one or more hard patches to develop. The localised scleroderma can either be morphoea or linear. In morphoea, the skin can either appear discoloured and itchy. The patches can be hairless and shiny and may improve without the need for treatment. Linear localised scleroderma is when the skin thickens in lines along the face, scalp, legs and arms. This can also affect the underlying bone and muscle. This can cause permanent growth problems such as shortened limbs.

Systemic sclerosis affects both the skin and internal organs. Like localised, there are two types: limited cutaneous systemic sclerosis and diffuse systemic sclerosis. Limited cutaneous systemic sclerosis is the mild form which often starts as Raynaud’s phenomenon. It is usually limited to the hands, lower arms, feet, lower legs and face as well as effecting the lungs and digestive system. Other symptoms include thickening of the skin over hands, feet, face and skin. It tends to get worse over time but can be controlled with treatment. Diffuse systemic sclerosis is more likely to affect the internal organs and have skin changes across the entire body. Other symptoms include weight loss, fatigue, stiffness and joint pain. The condition gets worse over the first few years and then stabilises. The heart, lungs and kidneys can be affected. This can cause problems such as high blood pressure and shortness of breath.

How is Scleroderma treated?

Treatment aims to relieve symptoms and prevent the condition getting worse and causing complications such as hypertension. Medication is given to help improve circulation, suppress the immune system, relieve joint pain and other complications such as high blood pressure. In severe cases, surgery may be needed to loosen tight muscles and remove hard lumps under the skin.

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