Acral ulcer

Introduction Acromegaly is an important complication of systemic scleroderma. Persistent and recurrent ulcers can cause severe pain, infection, gangrene, dysfunction and declining quality of life, causing great pain to patients. Because of this, the research on acral ulcers in scleroderma has become a hot issue in recent years.

Cause

(1) Causes of the disease

It is not clear, and it is summarized in the following aspects:

1. Genetic factors

According to some patients with a clear family history, the incidence of HLA-B8 is increased in critically ill patients and there are chromosomal abnormalities in the relatives of the patients. It is believed that the characteristics of the genetic type may be on the dominant allele of the X chromosome.

2, infection factors

Many patients often have acute infections before onset, including angina, tonsillitis, pneumonia, scarlet fever, measles, and sinusitis. Paramyxovirus-like inclusion bodies have been found in the patient's striated muscle and kidney.

3. Connective tissue metabolism abnormalities

The patient showed extensive connective tissue lesions, and the collagen content in the skin increased significantly. There were more soluble collagen and unstable intermolecular side chains in the virus active skin lesions. The fibroblast culture of the patient showed a marked increase in the activity of collagen synthesis.

4, abnormal blood vessels

Patients with Raynaud's phenomenon, not only limited to the extremities, but also in the visceral blood vessels; histopathology shows that the skin lesions and internal organs may have small blood vessels (arterial) contracture and intimal hyperplasia, so some people think that this disease is a primary blood vessel Disease, but because vascular disease is not seen in all patients, it is also considered that vascular disease is not the only cause of the disease.

5, immune abnormalities

This is the most important view in recent years. A variety of autoantibodies (such as anti-nuclear antibodies, anti-DNA antibodies, anti-ssRNA antibodies, antibodies against scleroderma skin extract) can be detected in patients; the number of B cells in patients increases, and humoral immunity is significantly enhanced in the system. The positive rate of circulating immune complexes in patients with type is up to 50%. Most patients have hypergammaglobulinemia; some cases are often complicated by lupus erythematosus, dermatomyositis, rheumatoid arthritis, Sjogren's syndrome or Hashimoto's thyroiditis. At present, most believe that this disease may be an autoimmune disease caused by a persistent chronic infection based on a certain genetic background.

(two) pathogenesis

Pathogenesis

(1) Theory of vascular injury: The Raynaud's phenomenon is often the early manifestation of SSc, which indicates that early lesions are obvious vascular damage, not only at the end of the finger (toe), but also in the internal organs. Recently, it has been suggested that SSc is the result of repeated damage of vascular endothelial cells. Endothelial cell damage causes changes in capillary permeability, and damage to the arterioles leads to platelet aggregation and endothelial cell proliferation. Endothelial cells are damaging to diversity, with swelling and hyperplasia, followed by thrombosis leading to narrowing of the lumen and tissue ischemia. These vascular lesions are found in many systemic blood vessels such as skin, skeletal muscle, digestive tract, lung, heart, kidney and brain. Although there are significant vascular lesions in the early stage of the disease, immunoglobulin, complement and immune complexes are rarely found in the blood vessel wall, so it is also indicated that endothelial damage is the basis of this disease.

(2) Immunology: The disease often coexists with autoimmune diseases such as systemic lupus erythematosus, dermatomyositis, Sjogren's syndrome, rheumatoid arthritis, etc., and idiopathic thrombocytopenia often occurs in the course of the disease. Purpura, autoimmune anemia, etc. There are a variety of autoantibodies in the serum, and there are often multi-cell strains of hypergammaglobulinemia, immune complexes, etc., suggesting autoimmune diseases.

These autoantibodies include: Scl-70, also known as anti-local isomerase I antibody, anti-centromere antibody, anti-nucleolytic antibody (including different nucleolar components), anti-PM/SSc antibody, etc., the role of these autoantibodies The mechanism is not very clear, but its corresponding target antigen is an important component of nuclear metabolism, so it is suggested that the disease is caused by molecular simulation. T lymphocytes also have significant abnormalities in the circulation and tissues around the SSc. In the peripheral blood, T cells decrease, and the ratio of T helper lymphocytes to inhibitory T cells increases; the lymphocytes of the skin dermis are mainly T helper lymphocytes, and the locally isolated lymphocytes can stimulate the activation of skin fibroblasts. Cytokines. In short, there are obvious humoral immune abnormalities and cellular immune abnormalities in this disease.

(3) Abnormal fiber proliferation: The skin and internal organs of this disease are extensively fibrotic because the newly synthesized collagen replaces most or all of the subcutaneous tissue, thus tightening and hardening the skin. Certain subtypes of fibroblasts isolated from the skin of SSc patients can synthesize excess collagen (mainly type I and III), glycoprotein, etc. in culture. Experiments have also shown that local collagen breakdown is reduced. Current studies have shown that TGF-B (growth-transforming factor) not only directly stimulates but also secretes PDGF- (platelet-derived growth factor) to indirectly stimulate the growth of fibroblasts.

2. Pathology

The main pathological changes of SSc are connective tissue inflammatory cell infiltration, intimal hyperplasia, vascular occlusion, fibrous tissue hyperplasia and sclerotic atrophy. In the early stage of skin lesions (inflammation), interstitial edema of the dermis, separation of collagen fibers, infiltration of lymphocytes around small blood vessels, edema of blood vessel walls, and breakage of elastic fibers. Thereafter, the infiltration of inflammatory cells around the blood vessels subsided, the collagen swollen, and the acidic mucopolysaccharides and collagen around the small blood vessel fibers increased. In the late stage (hardening period), collagen fibers are homogenized, collagen fibers bundles parallel to the epidermis are increased, collagen fibers are proliferated, and spread to the deep. The small vessel wall is thickened, the lumen becomes smaller, and even occluded. Continued changes in the late stage, leading to atrophy of the epidermis and appendages, calcium deposition, fascial muscle sclerosis and atrophy.

Visceral lesions are basically consistent with skin lesions. Multiple systemic sclerosis: smooth muscle (including esophageal muscle fiber bundle) showed uniform sclerosis and atrophy, extensive atrophy and fibrosis of intestinal wall muscle and myocardium; fibrin-like protein degeneration, inflammatory infiltration and collagen in endocardium and pericardium Hyperplasia; pulmonary interstitial and alveolar extensive fibrosis, and cystic changes; pulmonary arteriolar wall thickening, alveolar and microvascular basement membrane thickening; renal interlobular artery intimal hyperplasia, glomerular basement membrane thickening, fibrin Like necrosis. In severe cases, glomerular sclerosis and renal cortical infarction can be seen; thyroid may also have interstitial atrophy and fibrosis (Fig. 1, 2, 3).

3. Chinese medicine etiology and pathogenesis

The cause of this disease is mainly congenital deficiency, spleen and kidney yang deficiency, or exogenous damp heat, wet weight in heat, wet evil and yang, or cold and cold inside, or outside is not solid, exogenous wind and cold evil, evil resistance Weiyang The evil hinders the veins, which leads to the disharmony of the camp and the qi and blood. The advancement involves the visceral disorder, the yang deficiency, and the pathological products such as phlegm condensation.

(1) Wind and cold obstruction: Insufficient congenital endowment, defensive gas is not solid, cold and cold attack, injured in the lungs, blocked in the veins, the camp is not harmonious, the veins are impassable, then there may be body pain, swollen limbs, hard skin, cough , coughing, etc.

(2) spleen and kidney yang deficiency, cold coagulation sputum: cold and cold attack, or lack of congenital endowment, spleen and kidney yang deficiency, or excessive labor injury, yang deficiency, cold from endogenous, cold is cited, cold is stagnation, blood It can't flow, and the blood flow is not smooth, so it can lead to blood stasis, collaterals blocked, then the end of the cold, the skin becomes cold and white, purple, hard skin, even muscle and skin dystrophy and muscle thin Thin skin, hair loss, pigmentation.

(3) turbidity and obstruction: cold evil hurts the lungs, lung health is damaged, lungs are not declared, the liquid is difficult to lose, and it is concentrated; or the spleen and kidney are yang, turbidity can not be turbid. If the turbidity is blocked in the skin veins and the skin of the tendons is dying, the disease may occur.

(4) qi stagnation and blood stasis: anger and anger for a long time, emotional uncomfortable, can lead to qi stagnation and blood stasis, blood stasis and collaterals so that blood can not raise skin hair, so the skin is glory and hard and thin, skin changes Hard to open mouth is difficult, Qi Yu can not transport blood to reach the end of the fourth limbs, cold, body pain, and even ribs and rush.

(5) Damp heat block: the initial feeling of damp heat, wet weight in heat, wet evil damage yang, gradually appearing warm and insufficient skin tightening, so the joint changes from redness to white swelling, the beginning of another situation of the disease .

The disease begins at the beginning of the disease, but the evil stays for a long time to block the air, the blood flow is not smooth, and gradually the lungs, spleen and kidneys are involved, and the main cause is damp heat, cold coagulation, and collateral stagnation. Yin, yin and yang can be strained. Those who are damaged by the viscera are more dangerous.

an examination

Related inspection

Skin fungal microscopy skin smear microscopy

Skin manifests early hand and finger swelling, but also affects the forearm, foot, lower limbs and face, but the lower limbs are less affected. The swelling period can last for weeks, months, or even longer. Edema can be concave or non-concave and can be accompanied by erythema. Skin lesions progress from the distal end of the limb to the proximal end. The skin gradually hardens, thickens, and finally adheres to the subcutaneous tissue (hardening period).

Differential diagnosis

(1) Localized scleroderma needs to be identified with the following diseases:

1. Early damage of plaque atrophy is of different sizes, skin-colored or blue-white, dimples or bulges, wrinkles on the surface, and hard to touch.

2. Atrophic sclerosing mossy skin lesions are lavender-shining flat papules, which vary in size and often aggregate, but do not fuse with each other. There are hair follicle horny plugs on the surface, sometimes blisters, and skin atrophy gradually occurs.

(2) Systemic sclerosis needs to be identified with the following diseases:

Adult scleredema

Skin lesions often develop from the head and neck to the shoulder and back, and the deep dermis is swollen and stiff. Local non-pigmentation, no atrophy and hair loss, and self-healing tendency.

2. Mixed connective tissue disease

The patient has a mixed manifestation of systemic lupus erythematosus, scleroderma, dermatomyositis or polymyositis, including Raynaud's phenomenon, non-recessed edema of face and hands, swollen fingers, fever, non-destructive Arthritis, muscle weakness or myalgia. Both leaching nuclear antigen (ENA) and RNP antibodies are highly titer positive.

3. Scleroderma-like syndrome caused by chemicals and poisons

People with contact with chemicals such as polyvinyl chloride and benzene, as well as those who consume toxic oils or certain drugs and who receive silicone breast augmentation, may develop some other symptoms of crust and scleroderma. However, these people have no typical scleroderma manifestations, no specific autoantibodies in the serum, stop contact, and the symptoms can gradually disappear, which is easy to distinguish from scleroderma.

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