2. DEFINITION
Scleroderma is a group of rare diseases that involve
the hardening and tightening of the skin and
connective tissues.
Scleroderma is a Chronic disease that affects the
skin, connective tissue, and internal organs.
It occurs when the immune system causes the body
to produce too much of the protein collagen which
is an important part of your skin.
As a result, your skin gets thick and tight, and scars
can form on the lungs and kidneys. The blood
3. INCIDENCE
Scleroderma affects women more often than men and
most commonly occurs between the ages of 30 and
50.
While there is no cure for scleroderma, a variety of
treatments can ease symptoms and improve quality of
life.
4. ETIOLOGY
Scleroderma results from an overproduction
and accumulation of collagen in body tissues.
Exact cause of the abnormal collagen
production is not known but the autoimmunity
appears to play a role.
5. RISK FACTORS
Genetics- a small number of scleroderma cases
appear to run in families.
Environmental triggers- exposure to certain
viruses, medications or drugs. Repeated
exposure, such as at work, to certain harmful
substances or chemicals also may increase the
risk of scleroderma.
Autoimmunity
6. TYPES
There are two kinds of scleroderma:
A- Localized scleroderma which mainly
affects your skin.
It happens in one of two forms:
Morphea. This involves hard, oval-shaped
patches on the skin.
Linear. This causes lines or streaks of
thickened skin on mainly arms, legs, or
face.
2-Systemic scleroderma- involve many
body parts or systems. Such as lungs and
7. CLINICAL MANIFESTATIONS
Skin- Hardening and tightening of patches of skin.
Skin can appear shiny because it's so tight, and
movement of the affected area may be restricted.
Fingers or toes- Fingers or toes may turn blue or feel
painful or numb.
Digestive system- If the esophagus is affected,
heartburn or difficulty swallowing. If the intestines,
cramps, bloating, diarrhea or constipation.
Heart, lungs or kidneys.
8. DIAGNOSTIC EVALUATIONS
•Physical Examination
•X-rays and computerized tomography (CT)
scans are used to look for abnormalities.
•Thermography can detect differences in skin
temperature between the lesion and normal
tissue.
•Ultrasound and magnetic resonance imaging
(MRI) can aid soft tissue assessment.
9. MANAGEMENT
Nonsteroidal anti-inflammatory drugs (NSAIDs)
like ibuprofen or aspirin.
Steroids- for Immunosuppression and
inflammation, Steroid creams can be used for
inflammation.
Vasodilators to dilate the blood vessels and to
improve the blood flow.
11. NURSING MANAGEMENT
•Advice the patient to stay active, to protect the
skin, to stop smoking, and to protect the
extremities from cold.
•Helping the patient to cope up and providing
support.
•Advice the patient to Join a support group,
where he can share experiences and feelings
with other people
•Monitor the symptoms