Scleroderma

Scleroderma, or systemic sclerosis, is a chronic autoimmune disease that predominantly affects the skin and connective tissue diseases. It can also involve other organs in the body, including the lungs, heart, and kidneys.

Cause: The exact cause is unknown, but it is believed to be genetic predisposition and environmental factors that trigger the immune system to produce an excess of collagen that can lead to fibrosis.

Types:

Limited cutaneous scleroderma ( formerly known as CREST syndrome)

•   Limited skin involvement- face, hands, and forearms
•   Calcium deposits ( Calcinosis)
•   Raynaud’s phenomenon- fingers turning colors to cold or stress
•   Esophageal dysfunction
•   Telangiectasias ( dilated blood vessels under the skin

Diffuse Cutaneous Scleroderma

•   Widespread skin involvement, including trunk, arms, and legs
•   Rapid progression that can involve the lungs, heart, kidneys and gastrointestinal tract

Symptoms can vary and include:

•   Skin tightening, hardening, and reduced flexibility
•   Ulcers or sores on the fingertips
•   Raynaud’s phenomenon
•   Acid reflux,  difficulty swallowing, bloating, diarrhea, or constipation
•   Weight loss or malnutrition in severe cases
•   Shortness of breath
•   Joint pain, swelling, or muscle weakness
•   Can cause kidney problems

Treatment:  Scleroderma has no cure. Treatment targets symptoms, slowing disease progression and preventing complications.

1. Medications include immunosuppressants, vasodilators to improve blood flow, acid reflux medications, and pain relievers
2. Physical therapy and occupational therapy
3. Skin care, including regular moisturizing
4. Regular monitoring and treatment of organ involvement
5. May involve working with multiple specialists, including rheumatologists, cardiologists, and pulmonologists.