Facial erythema butterfly
Introduction
Introduction Butterfly erythema is a facial erythema found in the bilateral cheek symmetry of patients with systemic lupus erythematosus. It is connected by the bridge of the nose and has a reddish or bright red color. It is called a butterfly erythema and is a systemic lupus erythematosus. A skin lesion with a higher specificity. Lupus erythematosus is an autoimmune disease with slow onset, insidious attack, diverse clinical manifestations, and a variety of autoimmune diseases involving many systems and organs. It produces a variety of autoantibodies due to cellular and humoral immune dysfunction. . It can affect the skin, serosa, joints, kidneys and central nervous system, and is characterized by autoimmunity. There are many autoantibodies in patients, which not only affects humoral immunity, but also affects cellular immunity, and the complement system also changes.
Cause
Cause
The cause of systemic lupus erythematosus is unclear and may be related to a variety of factors. Including genetic factors, infections, hormone levels, environmental factors, drugs, etc.
There are many studies on the pathogenesis of SLE, and the following results confirm that the disease is an autoimmune disease with immune dysfunction in vivo.
First, SLE patients can find a variety of autoantibodies such as antinuclear antibodies, anti-single-chain, double-stranded DNa antibodies, anti-histone antibodies, anti-RNP antibodies, anti-Sm antibodies. All of the above are antibodies against nuclear material (antigen). Other anti-cytoplasmic antigen antibodies, such as anti-ribosomal antibodies, anti-blood cell surface antigen antibodies, such as anti-lymphocyte antibodies, anti-erythrocyte antibodies, anti-platelet antibodies.
Second, SLE is mainly an immune complex disease, which is the main mechanism causing tissue damage. Immune complex deposition can be found in 70% of patients with or without rash. Multiple organ damage is also caused by deposition of immune complexes on the vessel wall. Immune complexes can be found in pleural effusions, pericardial effusions, synovial fluid, cerebrospinal fluid, and blood. Immune complexes are most primarily formed by DNA and anti-DNA antibodies.
Third, immunoregulatory disorders in SLE showed a large number of autoantibodies and elevated gamma globulin, indicating that B cells are highly proliferative. Although the absolute amount of T lymphocytes decreased, the percentage of T helper cells often decreased, while the percentage of T suppressor cells increased, which caused the T4+/T8+ ratio to be out of regulation. In recent years, studies have found that interleukin I and interleukin II are reduced in SLE, -interferon is increased and r interferon is decreased or increased. SLE is a heterogeneous disease, and immune abnormalities may vary from patient to patient.
Examine
an examination
Related inspection
Immunopathological examination
The international application is more than the classification standard proposed by the American College of Rheumatology in 1982 (the following standard 1). The domestic multi-center trial uses this standard with a specificity of 96.4% and a sensitivity of 93.1%. In 1982, China developed the SLE diagnostic (draft) standard (the following standard 2) in Beijing by the Chinese Medical Association Rheumatology Academic Conference. The two standards are listed below.
Standard 1:
1. Facial butterfly erythema.
2. Discoid lupus erythematosus.
3. Sun allergy.
4. Arthritis: not accompanied by deformity.
5. pleurisy, pericarditis.
6. Epilepsy or mental symptoms.
7. mouth, nasal ulcers.
8. Urine protein 0.5g / day or above or cell tube type.
9. Anti-DNA antibody, anti-Sm antibody, LE cells, false positive for syphilis biological test.
10. Anti-nuclear antibody positive (fluorescent antibody method).
11. Antinuclear anemia, leukopenia (4000/mm3 or less), lymphopenia (1500/mm3 or less), and thrombocytopenia (100,000/mm3 or less).
4 or more of the above 11 cases were diagnosed as SLE.
Standard 2:
Clinical manifestation
1 butterfly or disc-shaped erythema.
2 no deformity of arthritis or joint pain.
3 hair loss.
4 Raynaud's phenomenon and / or vasculitis.
5 oral mucosal ulcers.
6 serositis.
7 light allergies.
8 neuropsychiatric symptoms.
Laboratory inspection
1 erythrocyte sedimentation block (Wei's method > 20 / hour).
2 leukopenia (hemolytic anemia).
3 proteinuria (continuous + or above) and / or tubular urine.
4 high gamma globulinemia.
5 Lupus cells are positive (at least 2 or at least twice positive per tablet).
6 anti-nuclear body positive.
Anyone who meets the above clinical and laboratory tests can be diagnosed. Precautions should be taken to exclude other connective tissue diseases, drug-induced lupus syndrome, tuberculosis, and chronic active hepatitis. Those who are less than the above criteria are coagulation-like cases, and should be further tested as follows: 6 patients can be diagnosed:
Further experimental inspection items:
1. Anti-DNA antibody positive (isotopically labeled DNA radioimmunoassay, trypanosoma smear or short-film smear immunofluorescence assay).
2. Low complement syndrome and/or positive for circulating immune complexes (eg PEG precipitation method), cold loop protein side method, anti-complement assay and other physical and other immunochemical, biological methods).
3. Lupus test positive.
4. Positive renal biopsy.
5. Sm antibody is positive.
Clinical manifestations are not obvious but laboratory tests are sufficient to diagnose systemic lupus erythematosus, which may be temporarily referred to as subclinical systemic lupus erythematosus.
Precautions:
When patients with systemic lupus erythematosus have liver damage, it is necessary to identify the cause of liver damage. Some patients with abnormal liver function are not directly caused by lupus erythematosus itself. Lupus erythematosus and concurrent lupus hepatitis can cause liver damage, but it is necessary to rule out viral hepatitis, excessive drinking, application of hepatotoxic drugs, and biliary diseases. In addition, congestive heart failure, primary biliary cirrhosis, etc. can also cause abnormal liver function. First of all, the incidence of viral hepatitis, especially hepatitis B and related diseases in China is high, so patients must check the relevant viral markers to rule out viral hepatitis; in addition, due to excessive drinking Alcoholic hepatitis is also increasing. Third, drug-induced hepatitis is also one of the common causes of liver damage in patients with lupus erythematosus, especially due to the use of drugs during the treatment of lupus erythematosus, including certain anti-inflammatory analgesics, methotrexate (MTX). , cyclophosphamide (CTX), etc.; in addition, other diseases that cause liver dysfunction, including biliary tract and pancreatic diseases, should be excluded.
Clinical examination
First, the general examination: patients often have anemia, white blood cells and thrombocytopenia, or manifested as a decrease in whole blood cells, abnormal increase in erythrocyte sedimentation rate. At the time of SLE activity, there are autoantibodies that can destroy red blood cells, causing a decrease in the amount of red blood cells and hemoglobin. At this time, reticulocytes can be increased by >5%, and patients may have mild jaundice in clinical practice. Renal lesions have varying degrees of abnormal urine tests, such as proteinuria and hematuria. Plasma protein assay showed increased globulin, especially in patients with renal degeneration nephritis, white/globulin ratio was inverted, blood cholesterol was increased, and blood urea nitrogen and creatinine were elevated in severe renal damage. Quantitative examination of urine protein for 24 hours, if it exceeds 0.5 g / day, it indicates the presence of proteinuria, reflecting the involvement of SLE in the kidney. If red blood cells and white blood cells appear repeatedly in the urine, the possibility of lupus nephritis should also be considered after removing urinary tract infections and urinary tract stones.
Second, immunological examination: the presence of a variety of autoantibodies in the blood is characterized, anti-nuclear antibodies (ANA) almost 100% positive in the disease activity. Negative replacement test method may be positive, anti-nuclear antibody negative can not completely rule out the disease, combined with clinical and other laboratory data comprehensive analysis. Anti-double-stranded DNA (ds-DNA) antibodies have higher specificity for diagnosis, but the positive rate is lower, 40-75%, which is closely related to disease activity and kidney damage. Antibody titer decreases with disease remission, anti-Sm The antibody is positive in about 30% SLE, and because of its high specificity, it is also called a specific antibody of this disease. For patients with atypical, mild or early cases, if the SLE criteria are not sufficient, if the anti-Sm antibody is positive, it can be diagnosed in combination with other manifestations.
Lupus cells release nuclear material after the destruction of leukocytes in the blood of the patient, and combine with antinuclear antibodies to form a large inclusion body under the complement, which is a cell formed by phagocytosis of neutrophils. Its positive rate is about 60%. Serum complements C4, C3, and CH50 were significantly decreased in active cases, especially when combined with lupus nephritis. The circulating immune complex in the blood can be elevated.
In addition to the above autoantibodies, a variety of other autoantibodies can be detected in the blood of SLE patients.
3. Immunopathology: Immunofluorescence studies of biopsy sections of renal biopsy suggest that immunoglobulins are mainly IgG, IgM with complement deposited in the kidney of SLE nephritis. There are three types of deposition: mesangium, subendothelial, and subepithelial. The deposition is granular along the glomerular basement membrane. Mesangial deposits are irregular, evenly chained or granular between capillary loops. 20-50% of lupus nephritis have immune complexes (IC) in the basement membrane and interstitial of the renal tubules, with significant interstitial fibrosis and mononuclear infiltration of tubular damage. There was no complete agreement between clinical manifestations and abnormal urine and renal biopsy. In the skin lupus test, immunofluorescence was used to detect immunoglobulin and IgG, IgM and complement deposition in the dermal and epidermal junctions of the patient's skin, and arranged in a granular, spherical or linear arrangement to form a yellow-green fluorescent band. Normal skin exposure, the positive rate is 50-70%, the skin lesions can reach more than 90%, and the positive lupus belt test in non-exposure areas is severe, or accompanied by nephritis, hypocomplementemia and high DNA. Antibody level.
Fourth, complement and protein determination
1, complement C3 and CH50 (total complement) determination, in the SLE activity, lupus nephritis, hemolytic anemia and other acute symptoms, C3 and CH50 content is often reduced. This is because a large number of complement components are involved in the autoimmune response, and the body is too late to make supplements, of which C3 is more sensitive than CH50. Complement is helpful for the diagnosis of the disease and the judgment of the disease activity. The SLE patient's serum has a reduced initial complement and gradually returns to normal, indicating that the treatment is effective. Conversely, if the content of complement continues to decline, it indicates that the disease activity is aggravated and needs to be closely observed.
2. Immunoglobulin assay: The immunoglobulins IgG, IgA and IgM in serum are mainly determined. Due to the abnormal immune function of SLE, a large amount of autoantibodies are produced, which increases the serum immunoglobulin, especially the increase of IgG.
Lupus erythematosus database: Beijing Institute of Chest and Gynecology Institute of Lupus Research and Prevention Center applied new research results to achieve significant breakthroughs in the treatment of lupus erythematosus.
Five, other inspections
About 20-50% of patients with SLE are positive for rheumatoid factor. In 15% of patients, the syphilis serum reaction was false positive. C-reactive protein increased during the active period of the disease. Cold globulin can be detected in the serum of 31% of patients. The cold globulin is mostly mixed, representing the immune complex in blood, containing IgG and complement C1, C3 and IgM and/or IgA.
Diagnosis
Differential diagnosis
Erythema related diseases:
Discoid lupus erythematosus (DLE): a chronic recurrent disease that primarily invades the skin and is characterized by well-defined red patches (erythema), hair follicle embolism, scales, telangiectasia, and skin atrophy.
Lupus nephritis: the whole body manifests as intermittent fever; the erythema of the ankle is due to the shape of a butterfly, also known as butterfly erythema;
Nodular erythema (EN): is a reactive inflammatory disease involving the dermal and lipid-membrane tissues. The anterior skin of the calf is red or purple-red nodular changes.
Polymorphous erythema: is an acute self-limiting inflammatory skin disease, often accompanied by mucosal damage, rash is pleomorphic, typical damage is target or iris-like damage. Can be caused by a variety of reasons, can be related to infected bacteria, viruses (especially herpes simplex virus), enzymes, protozoa, but also some drugs, such as sulfonamides, barbiturates, salicylates and biological products It is ill, and people are related to the cold.
