Scleroderma & C.R.E.S.T

Scleroderma is an autoimmune disease that can cause thickening, hardening, or tightening of the skin, blood vessels and internal organs. Scleroderma is chronic, which means it can last a long time. This is one disease in which the patients never gain weight, due to the effect it has on tightening their skin they all look skinny. They also have very hard hands, with skin around the fingers tight. They have difficulty swallowing as the esophagus is tight, so is the stomach wall tight and they cannot tolerate large meals. There are two types of scleroderma localized and systemic.

A) Systemic Scleroderma (SS) also called systemic sclerosis, the immune system causes inflammation in the small blood vessels and the collagen-producing cells located in the skin and throughout the body. SS causes the small blood vessels in the fingers to be inflamed; this causes injuries on the hands and fingers to heal slowly. In severe cases, ulcers form on the hands and fingers. People with Systemic Scleroderma are usually cold-sensitive. The inflamed small blood vessels and the reduced blood supply cause cold temperature sensitivity. Systemic Scleroderma patients also have problems with their heart, lungs and gastrointestinal tract. These problems occur as tissue builds up in the skin and organs due to inflammation.

B) Localized Scleroderma called Morphea affects the collagen-producing cells in just some areas of the body, and usually does not affect the internal organs and blood vessels. Localized Scleroderma can be seen as patches of thick skin or as a line of thick skin. The line may extend down a leg or arm.

C) A sub type of scleroderma is called CREST which has a distinct set of characteristics that give the syndrome its acronymic name. These characteristics include:

• Calcinosis: Tiny calcium deposits form under your skin, on elbows, knees and fingers; and can occur almost anywhere, in the body.
• Raynaud's phenomenon: The hands and forearms become cold and blue due to inflammation in the blood vessels the upper extremity.
• Esophageal dysfunction: Inflammation in the stomach and esophagus can cause swallowing problems and retention of fluids in the stomach.
• Sclerodactyly: Thick hard patches of skin start to calcify. This bone-like skin can even be seen on X-ray.
• Telangiectasia: Small, spider-like blood vessels start to form on lips and fingers.