Scleroderma

Introduction:

 

Scleroderma is a rare, potentially devastating autoimmune disease that affects the connective tissue of the human body, causing skin tightening and organ fibrosis.

 

Symptoms:

 

Hallmarks of the disease include:

 

• Skin thickening, especially on hands and feet, but may also occur across the nose, around the mouth and on the forearms and chest

• Bluish discoloration of fingers and toes (Raynaud’s phenomenon)

• Heartburn and food pipe/ gullet problems.

• Telangiectasia: Patchy flat reddish spots on torso, neck, tongue and other areas that blanch (turn white) with pressure

• Calcinosis: Hard stony deposits around joints especially elbows and pressure spots like the buttock, which can be quite uncomfortable

 

The severest complication from the disease involves the lungs (interstitial lung disease or pulmonary hypertension) and can lead to death.

 

Raynaud’s phenomenon:

 

The bluish discoloration of digits in Scleroderma patients can be treated with a combination of preventative measures and medical treatment. First, keep hands and feet warm. Scleroderma patients should practice cold avoidance by wearing gloves and stockings. Medications can help further and prevent ulcers and gangrene of the digits.

 

Role of Steroids in Scleroderma:

 

Unlike other rheumatologic diseases, scleroderma can potentially worsen with steroids (especially if a particular blood antibody test is positive). Steroids can precipitate sudden kidney damage leading to a renal emergency and can increase the speed with which lung and skin damage might occur. Scleroderma patients need to be cautioned about this.

 

Importance of Treatment:

 

It is crucial to catch Scleroderma early and initiate treatment, especially if lung disease has begun. Effective therapy to halt Scleroderma is available, even if options are limited when compared to other rheumatologic diseases. Promising research with options such as stem cell therapy and newer drugs is ongoing.