In traditional Chinese medical studies, this disease belongs to the categories of pi bi (skin pain due to external factors such as wind , cold and moist ) and ji bi (muscular pain due to external factors such as wind , cold and moist ), the causative factors are mainly due to weakness of Yang Qi in the organism, insufficiency of blood causing decreased immunity, affection of skin due to wind and cold externally with its accumulation at the skin’s turgor and blockade of its elimination causing imbalance of the fluid metabolism, injury of Qi and blood, insufficient of muscular nourishment, thrombose and blockade of mai luo (in TCM, it is not only vessels but also pathway for Qi and blood) with appearance of hardened skin like leather, skin atrophy, blockade of sweat pores resulting in sweating disturbances, loss of hair and etc.. If the pi bi is not treated for a long time, it will later affect the internal organs.
Scleroderma, nowadays, is also called systemic sclerosis. It is characterized clinically by local (limited) or diffuse thickening of the skin caused by accumulation of connective tissue and by involvement of visceral organs, including the gastrointestinal tract, lungs, heart, and kidneys. This disease has a worldwide distribution and affects all races. The incidence increases with age, peaking in the third to fifth decade (20-50 years-old); the onset of disease is unusual in childhood and young men). Women overall are affected approximately three to four times as often as men and even more often during the late childbearing years.
1.. Diffuse scleroderma and extremities scleroderma
The extremities scleroderma has the features of the CREST syndrome, the scleroderma process begins distally towards proximal part, rarely involves the trunk and internal organs, slow course of disease with good prognosis; while the diffuse scleroderma begins from proximal part of body towards distal part, has less CREST syndrome, often involves the internal organs, course of disease is fast with bad prognosis.
1) CREST syndrome: it is a signs of most patients; when finger (toe also) feels cold or one has emotional disturbances, it will become pale (white) to green purplish to red; it will become better after warming.
2) Skin: pathological changes can be divided into 3 stages- edematous, sclerotic (hardened) and atrophic.
a) Symptoms and body signs:
1. Limited scleroderma
1) Sclerotic plaque scleroderma: often happens at waist and back followed by limbs and face; circular, oval or irregular shape edematous plaques, begin with pale red or purplish-red which gradually expand and hardened after few weeks or months; after few years, its colour will turn to pale yellow or beige colour, no sweats, hair loss locally and became white or light brown coloured atrophic scar (skin may soften and return to normal thickness or become thin and atrophic).
2) Band scleroderma: often happens to children and youths, more in female than male, pathological changes happen between ribs and one side of the body (band forms), it can be single or multiple bands, its pathogenesis is same as above.
3) Linear scleroderma: often happens at neck, chest, shoulder and back regions, about a size of a green bean, compactly arranged in a line form and its pathogenesis is same as above.
1) Localized scleroderma which includes sclerotic plaque scleroderma, band scleroderma and linear scleroderma.
2) Systemic sclerosis which includes thickening of proximal and distal extremities (extremities scleroderma), diffused scleroderma and the CREST syndrome (standing for calcinosis, Raynaud s phenomenon, esophageal dysmotility, sclerodactyly, and telangiectasia).
The localized scleroderma can be diagnosed according to typical skin changes.
The systemic sclerosis is diagnosed due to the year 1998 standards of ARA (American Rheumatic Association):
A main standard: sclerotic process of skin of joints around fingers and palm, may involve whole limb, facial region, whole body and trunk.
B substandard: 1)finger scleroderma: skin changes as described as above but limited to the fingers only; 2)volar pads of the fingertips develop pitting scars and lose soft tissue;3)fibrinous process of base of both lungs (pulmonary fibrosis).
One can be diagnosed with one main standard or two substandard, others that help in diagnosis: CREST syndrome, polyathritis (inflammation of many joints) or athralgia (pain in joint), esophageal dysmotility, edematous and fibrosis of skin, positive results of ANA (antinuclear antibodies) immune test such as antitopoisomerase 1 (Scl-70) and ACA (anticentromere).
CREST syndrome, 3 from 5 symptoms or more than 3 symptoms with positive results of ANA can also be used to diagnose.
Cause of disease
The etiology and pathogenesis of this disease are not well-known yet, it may be associated with factors as below:
1. Hereditary: in some patients, certain anti HLA-2 (HLA-DQ) is higher than normal people.
2. Chemical substances and drugs: organic solvent, epoxy resin, etc can induce sclerotic process of skin and fibrosis of internal organs.
3. Abnormality of immune system: fluid and cell immune system is abnormal, antinuclear antibody Scl-70 is found in patient s serum. This explained that the disease is closely connected to the body immune system.
4. Disorganization of cell regeneration: the characteristic of this disease is excessive of fibroblast production causing fibrosis; contain of fibroblast in the skin is increased significantly.
5. Effect of cell s gene: some cell’s genes are related to this disease, e.g. maturate growth gene, skin regenerative gene, platelet regenerative gene, etc.
6. Vascular abnormalities: most of the patients show CREST syndrome, pathophysiological studies shows that increased thickness of endothelium of small arteries and capillaries with narrowing or blockade of vessels.
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