Lupus Erythematosus is an autoimmune disease involving multiple systems and organs. Its clinical manifestations are complex and its course of disease is prolonged and repeated. Lupus erythematosus can be divided into systemic lupus erythematosus (SLE) and discoid lupus erythematosus (DLE). Subacute cutaneous lupus erythematosus (SCLE) is a skin lesion between discoid lupus erythematosus and systemic lupus erythematosus. Discoid lupus erythematosus: it mainly invades the skin and is the lightest type of lupus erythematosus. A few cases may have mild visceral damage, and a few cases (about 5%) may turn into systemic lupus erythematosus. At first, the skin lesions of discoid lupus erythematosus are one or several bright red spots, the size of mung beans and soybeans, with adhesive scales on the surface, and then gradually expand, showing a round or irregular shape, and the marginal pigment is obviously deepened, slightly higher than the center. The center is pale, atrophic and low-lying, and the whole lesion is discoid (hence the name discoid lupus erythematosus). The damage is mainly distributed in sun-exposed parts, such as face, helix and scalp, and a few can involve upper chest, back of hand, forearm, lips and oral mucosa. Most patients have no symptoms, but it is difficult to completely disappear. The new injuries can be gradually increased or unchanged for many years, and the injuries are symmetrically distributed, or they can be merged into blocks, and the injuries in the middle part of the face can be merged into butterflies. Discoid lesions worsen after sun exposure or fatigue. Damage to the scalp can lead to permanent hair loss. Old injuries occasionally develop into squamous cell carcinoma of the skin. Deep lupus erythematosus, also known as lupus panniculitis, is also the intermediate type of lupus erythematosus. Skin lesions are nodules or plaques, located in deep dermis or subcutaneous adipose tissue, with uncertain size and quantity, normal or reddish skin color, solid texture and no activity. Injuries can occur anywhere, the most common are cheeks, hips, arms, followed by calves and chest. After chronic treatment, it can last for several months to several years, leaving skin atrophy and depression after healing. Deep lupus erythematosus is unstable and can exist alone, and then it can be transformed into discoid lupus erythematosus, systemic lupus erythematosus or both. Pathological phenomenon (1) the immune function of febrile lupus erythematosus is abnormal, and many substances can be produced in the body as heat sources, which makes the body fever. About 80% patients may have fever, mostly high fever, and about 12% patients show low fever. The first symptom of some patients is fever of unknown origin. It should be noted that a young woman has long-term fever for unknown reasons, accompanied by joint pain and swelling, and a rash. It is highly doubtful whether she has the possibility of lupus erythematosus, so we should ask a specialist for further examination to see if there are any autoantibodies to make a diagnosis. Fever often indicates that lupus erythematosus is in the active stage, and measures should be taken in time to avoid the development of the disease. Another feature of fever caused by lupus erythematosus is that the body temperature can quickly subside and return to normal after using glucocorticoid. If you stop using hormones, your body temperature will rise again. But patients with lupus erythematosus, especially after long-term use of hormones, will also have fever. At this time, we should be highly alert to the emergence of infection. Because after hormone use, it inhibits people's immunity and reduces the body's ability to resist diseases and bacterial infections. At this time, bacteria can take advantage of the opportunity to infect the body, the most common is lung infection. In particular, we should be careful about the infection of mycobacterium tuberculosis and use appropriate antibiotics in time to avoid the development of the disease and endanger our lives. (2) More than 80% patients with erythema and rash have skin damage, and erythema and rash present various types. Butterfly erythema on zygomatic face and edema erythema around nails and fingertips are the characteristic manifestations of lupus erythematosus. Butterfly erythema does not exceed nasolabial groove, and irregular erythema can also be seen on the bridge of the nose, forehead and auricle. Erythema of different shapes can also appear on the back of elbow, palm, knuckle, knuckle back, palm, sole of foot and other parts of upper arm. The shapes are discoid erythema, annular erythema, edematous erythema and polymorphic erythema. Discoid erythema is flaky, with clear boundary, slightly flat in the middle of the protuberance, with keratin debris stuck on it, which can not be wiped off. It is connected with the lower hair follicle and is a hair follicle corner plug, which can leave atrophic scars and slight depressions in the future. Rash includes red papules and maculopapules, which are generally not itchy or slightly itchy, and can occur in all parts of the body, especially the face, neck and limbs. A few people have blisters and blood bubbles. After erythema and blisters subside, epidermal atrophy, pigmentation and keratinization may occur. Photosensitive, about 1/3 patients appear flushed as soon as they are exposed to the sun. They are mainly sensitive to ultraviolet rays. In summer, in the shade of the house, their faces will turn red due to radiation, and some patients will have allergic rashes after ultraviolet radiation. (3) Mucosal ulcer and alopecia About 65,438+0/5 patients have mucosal damage, involving lips, tongue, cheeks, nose and cavity. And painless mucosal ulcer appeared. If there is a secondary infection, there may be pain. Patients with lupus erythematosus are prone to hair loss. In addition to hair loss caused by inflammation in the rash, hair loss can also occur in other parts, not only hair, but also eyelashes. Eyebrows and body hair will also fall off. There are two forms of hair loss: one is diffuse hair loss, the residual hair is sparse, dull or yellow, the hair is dry, sparse and easy to break, resulting in sparse or alopecia areata; Another kind of hair loss is concentrated on the forehead, which is often called "flowing sea". Hair is sparse, yellow and easily broken, and the length of hair is uneven, forming "lupus hair". Alopecia caused by lupus erythematosus is completely different from seborrheic alopecia, and its pathological basis is also different. Lupus alopecia is mainly caused by vasculitis under the skin, which leads to the disorder of nutrient supply of hair follicles and affects the growth of hair. It exists as a clinical manifestation of lupus erythematosus, and the first symptom of some patients with lupus erythematosus is alopecia, so it should be noted that alopecia in lupus erythematosus is more common. Generally, hair can be regenerated after the disease is controlled. It is particularly noteworthy that alopecia in patients with lupus erythematosus may be the first symptom of disease recurrence, which should attract the attention of doctors and patients. (4) More than 90% of patients with joint pain have joint pain, which may occur at all stages. Some joint pains have appeared several years before the onset, and some soft tissues around the joints are swollen, tender and edematous, showing acute arthritis. Proximal phalangeal joints, metacarpophalangeal joints, wrist, elbow, knee and toe joints, etc. Usually symmetrical. Some patients are stiff in the morning. Some patients have a long course of joint disease, while others have a short course of joint disease, and even have short-term joint pain. Most X-rays can't show worrying bone changes and deformities. After taking glucocorticoid for more than 5 years, about 5% patients can have aseptic osteonecrosis. (5) Decreased blood cells are reduced due to the existence of autoantibodies, and red blood cells, white blood cells and platelets are all destroyed by themselves. Anemia is mostly positive cell anemia, anti-erythrocyte antibodies are mostly lgG type, and hemoglobin is also reduced. White blood cells are generally granulocytes or lymphopenia. Thrombocytopenia and short survival time. Platelets are self-destructive because they have lgG antiplatelet on their surface. (6) About 65,438+0/4 patients with heart damage have pericarditis, which is mild and asymptomatic, with only obvious precordial pain, chest tightness and temporary pericardial fricative sound, which may not be found clinically. Echocardiography or B-ultrasound and X-ray chest film need routine examination, which can show pericardial effusion. 34. 15% SLE patients were found to have pericardial effusion. (VII) Lung damage Many SLE patients have two punctate nodular shadows and cord-like or reticular shadows in the basal segment of the lung on the X-ray chest film, which are interstitial changes, and most of them are asymptomatic. Pleurisy can be asymptomatic, only found in the following examinations, that is, B-ultrasound and X-ray chest films have a small to moderate pleural effusion, sometimes coexisting with pericardial effusion. LE cells can be seen in pleural effusion. (8) Clinically, about 75% of SLE patients have kidney damage, and urine tests show protein, red blood cells and white blood cells, and a few patients have casts. Lupus nephritis occurs in the early stage. If the lesion persists for many years, it may have extensive damage and evolve into uremia due to renal insufficiency. (9) SLE with brain injury has various manifestations of nervous system damage. Psychopathy is manifested as schizophrenia, and various mental disorders such as irritability, insomnia, hallucinations, suspicion, delusion, obsessive-compulsive ideas, etc. Brain injury is common in the terminal stage of chronic SLE or severe cases of acute SLE attack. A few cases of brain injury were born slowly, with mild illness, often accompanied by headache, dizziness and abnormal EEG changes. In rare cases, it is the first symptom of acute phase. Generally speaking, after timely treatment, brain injury is reversible, as are abnormal changes in EEG and brain CT scanning. Severe intracranial hemorrhage and cerebral hernia can lead to death. (X) About 50% SLE patients with lymphatic reticular system have local or systemic lymphadenopathy, especially in the neck, submandibular and axillary lymph nodes, which are soft and have different activities, and generally have no tenderness. Many patients have swollen tonsils and pain, which often leads to the onset of systemic lupus erythematosus. Antibacterial and anti-inflammatory effect is not good. About13 patients have hepatomegaly, but SLE has no special liver lesions. A few patients can have a slight increase in GPT and GOT, which rarely causes jaundice and cirrhosis. About 20% patients have splenomegaly. Spleen enlargement is usually found in B-ultrasound. For example, it has been increased by 1- 1.5 times that can be felt under the ribs in physical examination. Very few patients have splenomegaly. (1 1) Irregular menstruation and menstrual disorder are common in early and active SLE patients. Menstruation is advanced, delayed, decreased or menstrual flow is obviously increased. If anticardiolipin antibody (anti-Acl) is positive, there may be stillbirth or abortion. B-ultrasound can occasionally find pelvic effusion, suggesting the existence of pelvic serositis. (12) Raynaud phenomenon Raynaud phenomenon is a peripheral circulation disease. Stimulated by cold or emotional tension, the digital (toe) arteriole suddenly spasms. Typical symptoms of Rp include: several fingers or toes turn pale after catching cold or nervous, then turn blue, rub or keep warm, then turn ruddy, and Rp may be accompanied by local numbness or tingling. Rp most often affects fingers, but toes, ears, nose and tongue are also affected. Persistent or frequent attacks of Rp can lead to ischemic ulcer or necrosis of toes. Rp can occur in the absence of basic diseases, even if it cannot be diagnosed as a certain disease after years of follow-up, it is also called primary Rp. However, connective tissue diseases are often accompanied by Rp. For example, more than 90% of scleroderma and mixed connective tissue disease, 20% ~ 60% of lupus erythematosus can be accompanied by Rp. What is more striking is that Rp can be used as an early initial symptom of connective tissue diseases, which appears several months or years earlier than other clinical symptoms. It can be seen that Rp, as a clinical manifestation, is of great significance for the diagnosis of connective tissue diseases (including lupus erythematosus), especially for early diagnosis. (13) Vasculitis A large number of petechiae can appear on both hands and feet, which is caused by embolic vasculitis and peripheral necrotizing vasculitis caused by the accumulation of immune complexes into macromolecules blocking tiny blood vessels, and can also cause depression, ulcer and necrosis of fingertips and toes. Very few cases can cause arteritis obliterans of dorsum of foot, accompanied by severe pain. Netted and flaky blue spots can appear on both legs. Cyanosis reticularis, like fishnet, is caused by spasm of central arteriole in subcutaneous tissue, and cyanosis caused by dendritic branches of capillaries is reticular around the middle ischemic pale area. It is most common in the inner thigh, and reticular green spots can also be seen in the inner leg, trunk, upper limbs and hand traction. The flaky purple spots on the legs often coexist with reticular green spots, which are subcutaneous bleeding caused by necrotizing vasculitis of cutaneous arterioles or thrombocytopenia. Some have vascular embolism, which can palpate small nodules, and flaky purple spots are more common in calves, thighs and upper limbs. Embolic phlebitis can also be seen in clinic. The nails have changed. Many patients have onychomycosis, which is related to fungal infection after taking corticosteroids for a long time. There is also peripheral vasculitis, which leads to the thinning, softening and atrophy of nails and looks like onychomycosis. (14) The common symptoms of digestive tract damage include loss of appetite, constipation and abdominal distension in many patients. Some patients have symptoms such as nausea, abdominal pain around navel and frequent defecation. Ascites is related to lupus peritonitis and mesenteritis, and some are related to hypoproteinemia of lupus nephritis. Pancreatitis caused by lupus is rare. This is related to pancreatic blood vessels. (15) About 20%-25% of patients with ophthalmopathy have fundus changes, including fundus hemorrhage and papilla edema. Retinal exudates are oval and round white mixed substances, which are the products of swelling and deformation of retinal nerve fibers caused by small vessel embolism, which can cause visual damage. With the remission of the disease, these changes can disappear. Other vitreous hemorrhage and scleritis. (16) Although the incidence of infectious lupus erythematosus in China is much higher than that in western countries, it is only 70/65438+ 10,000, which is relatively rare compared with other common diseases. Because there are relatively few patients, many people don't know much about this disease. Hearing this name or seeing lupus patients will inevitably lead to such questions: Can lupus be contagious? The answer is no, lupus erythematosus will not be transmitted from one person to another. As far as the pathogenesis of lupus erythematosus is concerned, the etiology is still unknown. From clinical research, it is found that its occurrence may be related to genetic factors, the level of sex hormones in the human body and some environmental factors in which the human body is located, including infection, sunlight exposure, food changes, drug effects and so on. However, these factors may be combined with many unknown factors, reducing human immune tolerance and making human immune function disorder. Forming this autoimmune disease. Therefore, unlike diseases caused by various pathogens, bacteria, viruses, fungi and spirochetes, it can spread from person to person. When normal people contact patients, there is no need to have the fear of being infected by lupus erythematosus, and there is no need to isolate patients. Patients with lupus erythematosus can engage in general work, study and participate in social activities of normal people like normal people during the period of remission and stability. Diagnostic criteria of lupus erythematosus 1. Butterfly erythema or discoid erythema: flat or fixed erythema appears on the neck, which usually does not involve nasolabial groove and discoid erythema. The raised erythema is covered with horny scales and hair follicle damage, and the focus can be skin atrophy 2. Photosensitive x 10'/L or hemolytic anemia: skin allergy caused by sunlight 3. Oral mucosal ulcer: painless ulcer in oral cavity or nasopharynx 4. Non-deformative arthritis or multi-joint pain: Non-erosive arthritis involving two or more peripheral joints, characterized by joint swelling, pain or exudation. Pleurisy or pericarditis: chest pain, pleural fricative sound or. Pericarditis, abnormal electrocardiogram, pericardial fricative sound or pericardial effusion 6. Epilepsy or mental symptoms: caused by non-drug or metabolic disorder, such as uremia, ketoacidosis or electrolyte disorder; Mental illness: non-drug or metabolic disorder, such as uremia, ketoacidosis or electrolyte disorder. 7. proteinuria, tubular urine or hematuria: proteinuria >; 0.5g/dl or 3+; Cell casts can be red blood cells, hemoglobin, granular casts or mixed casts. 8. White blood cells are less than 4x 10'/L or platelets are less than 100 9. Fluorescent antinuclear antibody positive 10. Anti-double-stranded DNA antibody positive or lupus cell positive 1 1. Anti-Sm antibody positive 12. O decrease 13. Skin lupus band.
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