about Scleroderma

Scleroderma is a chronic autoimmune disease characterized by fibrosis (or hardening), vascular changes, and autoantibodies. Causes of scleroderma is unknown. Scleroderma run in families, but genes are not identified. It affects the small blood vessels known as arterioles, in all organs

There are two major forms of scleroderma:

Limited systemic sclerosis / scleroderma 's Cutaneous manifestations mainly affecting the hands, arms and face. Previously called the CREST syndrome refers to the following complications: calcinosis, Raynaud's phenomenon, esophageal dysfunction, sclerodactyly, and Telangiectasias.

In addition, pulmonary arterial hypertension may occur in up to one third of patients and is the most serious complication to form scleroderma.

Diffuse systemic sclerosis / scleroderma developed rapidly and affects the skin area and one or more internal organs, often kidney, esophagus, heart and lungs. Form of scleroderma can be quite disabling.

There is no treatment for scleroderma itself, but the individual organ system complications are treated.

Other forms of scleroderma including systemic scleroderma sine, which has no skin changes, but has systemic manifestations, and two localized form that affects the skin, but not the internal organs: morphea and linear scleroderma.

The prognosis is generally good for a limited Cutaneous scleroderma patients who had fled lung complications.

Worse prognosis for Diffuse Cutaneous diseases, especially in older age, and for men. Death most often occurs as a complication of lung, liver and kidneys. In Diffuse Cutaneous disease, 5-year survival was 70%, 10-year survival 55%.

First, the arteriole endothelial cells die, along with muscle cells, by the process of apoptosis. They are replaced by collagen and other fibrous materials.

Inflammatory cells, especially CD4 + T helper cells, infiltrate arteriole, and causing further damage. Many signal inflammation and damage to proteins have been identified, and they are potential targets for drugs that could interfere with the process.

Local scleroderma

Local morphea

Morphea-lichen sclerosus et atrophicus overlap

General Morphea

Atrophoderma Pasini and Pieris

Pansclerotic morphea

Morphea profunda

Linear scleroderma

Systemic scleroderma

CREST syndrome

Progressive systemic sclerosis

The disease is found among all races all over the world, but women are four times more likely to develop scleroderma than men. In the United States, about one in 1,000 people affected.

Children who rarely suffer from systemic type, but the general local scleroderma. Most adults are diagnosed after 30 years and before 50 years old.