What is scleroderma?

Scleroderma is a rare autoimmune disease characterized by skin tightness, thickening and inflammation, but the condition can also affect the lungs, throat, heart, kidneys and intestines, among other areas. Although the condition mainly affects the skin, other types of scleroderma affect other parts of the body.

The disease is caused by the body producing an excess of collagen, which can cause inflammation and the body’s organs to not function normally. However, the way scleroderma affects people is individualized. In people with localized scleroderma, the most common symptom is discolored skin (morphea), or hard skin on the hands or arms (linear scleroderma), with some people developing long lines on the head or neck (coup de sabre). Systemic scleroderma involves hardening of the internal organ systems and can be limited or spread across large areas of the skin (diffuse scleroderma).

To diagnose scleroderma, a primary care physician or rheumatologist will perform a clinical evaluation that involves blood tests, X-rays, a physical examination and thermography. One of the tell-tale signs of scleroderma is Raynaud’s phenomenon, which is characterized by discolored fingers or toes as a result of blood loss to these extremities; it is estimated that 90% of people with scleroderma also display signs of Raynaud’s phenomenon.

Physicians will also look for symptoms, such as heartburn, calcium deposits, shortness of breath, joint problems, high blood pressure due to issues with the kidneys, skin thickening, swelling and tightness, as well as large blood vessels on the hands or face. When scleroderma worsens, patients can expect complications, such as pulmonary fibrosis in the lungs, an increased risk of arrhythmias, dental decay, digestive problems and increased sexual dysfunction.

There is no cure for scleroderma, but certain treatments can manage the symptoms. By managing blood pressure with calcium channel blocks, angiotensin converting enzyme inhibitors or phosphodiesterase type 5 inhibitors, a person with scleroderma can improve their circulation and treat symptoms, such as Raynaud’s phenomenon. Other over-the-counter medications, such as antacids and pain relievers can help reduce symptoms, and immunosuppressive medications may help muscle or joint weakness.

References :

www.hopkinsscleroderma.org/scleroderma

www.mayoclinic.org/diseases-conditions/scleroderma/basics/definition/CON-20021378

www.rheumatology.org/I-Am-A/Patient-Caregiver/Diseases-Conditions/Scleroderma