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This book describes Scleroderma, Diagnosis and Treatment and Related Diseases
Scleroderma is an autoimmune disease typically featured by the poor regulation of the immune system, vasculopathy and fibrosis.
It is a connective tissue disorder featured primarily by the thickening and hardening of the skin produced by accumulation of collagen, and by injuries to the smallest arteries.
There are 2 main types of scleroderma: localized and systemic.
In localized scleroderma, the disease involves mainly the skin and may have an effect on the muscles and bones.
In systemic scleroderma, there is a participation of the internal organs, such as the digestive tract, heart, lungs, and kidneys, among others.
The severity and prognosis of scleroderma are inconsistent.
There are 2 subtypes of Scleroderma:
1. Limited cutaneous Systemic Sclerosis (LcSSc)
In LcSSc the skin thickening and hardening happens in the fingers, hands, face, forearms distal to elbow, and lower legs distal to the knees.
LcSSc is linked with anti-centomere antibodies, pulmonary arterial hypertension (PAH) with or without interstitial lung disease (ILD).
CREST syndrome (Calcinosis, Raynaud's phenomenon, Esophageal dysfunction, Sclerodactyly, Telangiectasias) is linked with LcSSc.
2. Diffuse cutaneous Systemic Sclerosis (DcSSc)
In DcSSc the skin thickening and hardening happens proximal to the elbows and knees and may affect the trunk.
DcSSc normally have a faster start and progression and is at danger of progressing to the visceral organs, such as the kidneys, heart, lungs and gastrointestinal tract.
Normal involvements are:
a. Interstitial lung disease with or without PAH, cardiomyopathy,
b. Diffuse gastrointestinal disease and
c. Renal crisis.
The precise cause of scleroderma is not fully known.
There is some presence that genetic and environmental factors have a part in the genesis of scleroderma.
Silica and certain organic solvents are documented as danger factors.
The result is a trigger of the immune system, causing blood vessel injury and damage to tissues that cause scar tissue formation and the collection of excess collagen.
Genetic factors have at least a limited part.
Scleroderma produces thick, hard patches of skin.
Localized scleroderma is restricted to the skin and underlying tissue
Systemic Sclerosis you are so scary
Your disease attacked my whole body
Most of all my skin is tight and thickened
My fingers are all puffy and swollen.
My skin is so itchy and sometimes painful
My fingers becomes blue when it becomes cool
A condition called Raynauld's phenomenon
There is distension always in my abdomen
Sometimes I have heartburn and problem swallowing
I feel as though my intestinal tract is not moving
Sometime my body is unable to absorb any nutrition
And I end up with either diarrhea or constipation
I tend to get overgrowth of bacteria in my GIT
I even soil my underwear before I could pass it
My heart enlarges and I develop pulmonary arterial hypertension
Is that why I am so breathless or is it due to lung infection
Doctor please be kind, keep me warm
And give medicine to keep me strong
Do not let my kidney fail or falter
Let me spend some time with my son and daughter
-An original poem by Kenneth Kee
1. Physical signs of skin thickening, puffy fingers
2. Auto antibodies-antinuclear antibody test is positive in majority of cases (90%)
3. Nail capillaroscopy is a method to study micro circulation of nail bed
There is no cure for Scleroderma
Topical corticosteroids are the primary treatment.
Stem cell transplantation and treatments directing on cytokine signaling, such as interleukin-6 (IL-6), have produced promising results
TABLE OF CONTENT
Chapter 1 Scleroderma
Chapter 2 Causes
Chapter 3 Symptoms
Chapter 4 Diagnosis
Chapter 5 Treatment
Chapter 6 Prognosis
Chapter 7 Raynaud’s Syndrome
Chapter 8 Psoriasis