• Sysytemic sclerosis or scleroderma is a very rare disease mostly effects middle aged female.
• It is characterized by hardening of skin leading to tight and shiny appearance.
• It is an autoimmune disease where body’s immune system attacks own cells and leading to excess collagen or fibrous tissue formation.
• In this disease all most all people have skin tightening of finger and whitish or bluish discoloration on cold exposure sometime leading to finger ulceration.
• Two forms of systemic sclerosis exists -
- Limited Systemic sclerosis-Skin tightening limited to forearm, finger leg and face.
- Diffuse systemic sclerosis-Skin tightening extends to arm, chest, abdomen and thigh
• Along with skin tightening there is also involvement of other organs such as joints, lung, oesophagus, kidney and heart

• The exact cause of systemic is not still known
• It is not contagious so it doesn’t transmit from one to another.
• Most people with systemic sclerosis will not have other family members with this condition.
• It is an autoimmune condition where body’s immune system defective or dysregulated because of which it attacks the healthy body
• In systemic sclerosis small blood vessel damage occur leading to finger ulceration and bluish or whitish discoloration on cold exposure (Reynod’s phenomenon)

• This is a very rare disease
• Affects four times more women than men.
• Usually occurs between 25 to 50 yrs of age
• It is extremely rare in children.

• Two forms of systemic sclerosis : limited and diffuse.
- Limited Systemic sclerosis: skin tightening is limited to forearm,leg and the face and neck.
- Diffuse Systemic sclerosis: skin tightening is more widespread and can also affect the trunk, back, thighs and arms.
• Skin- tightening of skin leading to shiny appearance of body
- Face-loss of wrinkles, difficulty in opening of the mouth, cheeks may look hollow.
- Salt and pepper appearance of skin because of loss of skin pigment

• Raynaud’s phenomenon
- Tips of fingers and toes turn white and then blue and/or red in the cold exposure.
- Sometime leading to digital ulceration.
- Almost all systemic scleroderma patients have this raynaud’s phenomenon.
- This usually occur more during winter months

• Heartburn and regurgitation of food-
- Scleroderma affects the esophagus which connects the mouth to stomach leading to difficulty in swallowing of food
- It can have significant heart burn
- Coming out of food material into the mouth if immediately sleep after food intake.
- Some patients have constipation

• Lung –
- breathlessness on exertion, dry cough
- Interstitial lung disease- Similar to skin tightening there is scarring of lung tissue leading to restriction of lung expansion. More seen in patients with diffuse systemic sclerosis
- Pulmonary Hypertension-Narrowing of blood vessels of the lungs leading to increase in pulmonary blood vessels. More seen in limited systemic sclerosis

• Arthritis-
- Inflammation of joint leading to pain and swelling of the joint

• CREAST Syndrome-
- Calcinosis-Calcium deposition in the skin
- Raynaud’s phenomena
- Esophageal dysmotility-Acid reflux and decrease in motility of esophagus
- Sclerodactyl- thickening and tightening of finger
- Telangiectasia-blood vessel dilatation

• Other symptoms-
- Muscle weakness-difficulty in getting up from squatting posture or from sleeping position
- Fatigue-Easy fatigability
- Scleroderma Renal Crisis-Breathlessness, Emergency situation, Severe hypertension, anemia
- Dry eye and dry mouth
- Cardiomyopathy

If you are having the skin tightening of fingers or toes and bluish or whitish discoloration of fingers in cold exposure then consult a rheumatologist.

To diagnose Scleroderma it needs complete history and thorough physical examination.

Sometimes a special blood test and scans are needed

Blood tests:

• Complete blood count-Hemoglobin, WBC, Platelet
• ESR,CRP-Acute phase reactant for inflammation
• Serum creatinine-For kidney function
• CPK,LDH-for muscle involvemet
• Special Blood test-Anti nuclear antibody (ANA)

Urine test-

• To detect proteinuria or hematuria

2D Echo of heart-

• For Pulmonary hypertension

Chest Xray-

• for Interstitial lung disease

HRCT thorax-

• for detection of ILD (Interstitial lung disease)

6 minute walk test-

• for Pulmonary HTN

• Scleroderma has no effect on fertility.
• The chances of getting pregnant for people with scleroderma are the same as with people that don’t have it.
• Scleroderma also has no known effects on the developing fetus or the newborn.