Scleroderma is an autoimmune disorder that may involve changes in the skin, blood vessels, muscles, and internal organs. There are two main types: localized scleroderma, which affects only the skin; and systemic scleroderma, which affects the blood vessels and internal organs, as well as the skin. These two main types also have sub-types.
Localized scleroderma subtypes include:
- Linear scleroderma
- Morphea
Systemic scleroderma subtypes include:
- Diffuse cutaneous systemic sclerosis
- Limited cutaneous systemic sclerosis (which includes CREST syndrome)
- Limited Systemic Sclerosis
Causes
The underlying cause of scleroderma is currently unknown; however, some scientists suspect it may be related to a buildup of collagen in the skin and other organs due to an abnormal immune system response. Some cases of scleroderma are induced by environmental factors or occur in association with other underlying disorders such as rheumatoid arthritis, lupus or Sjogren syndrome.
Signs and Symptoms
The most common symptoms of localized scleroderma include:
- Cigarette-paper scars
- Hyperpigmentation of the skin
- Hypopigmentation of the skin
- Stiff skin
The most common symptoms of systemic scleroderma include:
- Abnormality of the gastric mucosa
- Arthralgia
- Arthritis
- Atypical scarring of skin
- Autoimmunity
- Chest pain
- Chondrocalcinosis
- Cough
- Edema
- Fatigue
- Gastroparesis
- Hyperkeratosis
- Lack of skin elasticity
- Myalgia
- Nausea and vomiting
- Skeletal muscle atrophy
Management Strategies / Treatments
The following have been approved by the FDA as orphan drugs for systemic scleroderma:
- Nintedanib (Brand name: Ofev)
Clinical Trials
For a full list of clinical trials relating to scleroderma, go here.
Resources
Scleroderma Research Foundation
Steffens Scleroderma Foundation
To learn more about Scleroderma and other Autoimmune/Auto-inflammatory Disorders visit https://checkrare.com/diseases/autoimmune-auto-inflammatory-disorders/