acquired epidermolysis bullosa
Introduction
Introduction to acquired bullous epidermis Acquiredpidermolysisbullosa (EBA) is a chronic subepidermal bullous skin disease with anti-type VII collagen immunoreactive antibodies, which is more common in adults. This disease is similar to the "pemphigus" recorded in the medical literature of the motherland. Occurred in adults, skin lesions occur in areas susceptible to trauma and compression, such as the hands and feet, elbow and knee joint extension, skin lesions for blisters, bullae and erosion, leaving atrophic scars and millet rash, the current Its clinical performance classification has not been determined. basic knowledge The proportion of illness: 0.003% Susceptible people: more common in adults Mode of infection: non-infectious Complications: sepsis
Cause
Acquired bullous epidermolysis
(1) Causes of the disease
The cause is still unclear, but there is considerable evidence that the occurrence of this disease is related to autoimmune factors. Direct immunofluorescence revealed IgG deposition in the real epidermal junction of the skin biopsy next to the lesion.
(two) pathogenesis
Recent studies have shown that this autoantibody is resistant to collagen type VII located in anchor fibrils, which binds to the a chain of type VII collagen, resulting in a decrease in the number of anchor fibrils. The mechanism of this reduction is unclear. It has been suggested that the a-chain of the newly synthesized collagen type VII may bind to the EBA autoantibody, which may result in the formation of the triangular helix and the stable anchor fibrils. In vitro experiments have not confirmed that EBA autoantibody IgG can cause skin blisters.
Acquired bullous epidermolysis is not a hereditary disease, but it does have the genetic quality of autoimmunity. In the southeastern blacks of the United States, EBA and bullous SLE patients have high HLA-DR2 phenotype positive rates. This result also suggests that EBA and bullous SLE are immunologically related, and the HLA-DR2 gene is involved in the autoimmune response against anchorage collagen.
Prevention
Acquired bullous epidermolysis
Precaution:
1. Deaf patients take appropriate rest to avoid mental stress.
2. Change hormone therapy for traditional Chinese medicine treatment.
3, tell the patient about the symptoms of hypertension, such as dizziness, headache, vertigo, tinnitus, insomnia, fatigue and so on.
4, if there is elevated blood pressure, follow the doctor's advice to give antihypertensive drugs.
5. It is recommended that the patient purchase a home sphygmomanometer to monitor blood pressure.
6. A method of teaching patients and their families to measure blood pressure.
Complication
Complications of acquired bullous epidermolysis Complications sepsis
The cause of the disease is not clear, may be related to immune factors, and the integrity of the skin is destroyed, it can lead to bacterial infection or fungal infection of the skin, usually secondary to low body, or long-term use of immunosuppressive agents and fungal infections such as nail fungus Patients, such as concurrent bacterial infections, may have symptoms such as fever, swelling of the skin, ulceration, and purulent secretion. Severe cases can lead to sepsis, which should be brought to the attention of clinicians.
Symptom
Congenital bullous epidermolysis symptoms Common symptoms Caesarea dystrophy papules epidermis full layer necrosis and... Spleen deficiency spleen and kidney yang deficiency
Occurred in adults, skin lesions occur in areas susceptible to trauma and compression, such as the hands and feet, elbow and knee joint extension, skin lesions for blisters, bullae and erosion, leaving atrophic scars and millet rash, the current Its clinical performance classification has not been determined, but there are at least four types:
1. Classical manifestations: non-inflammatory bullous lesions are distributed in the extremities, lesions are bullous erosions, and tend to rub the crusting of the surface, such as the back of the hand, the back of the fingers, the elbows, the knees, the tail and the toes, Sometimes blisters can further develop bloody desquamation, crusting and erosion, scarring hair loss and nail dystrophy can be seen. This kind of skin lesions reminds people of delayed skin porphyria, or hereditary bullous epidermis. In spite of the lysis, the hirsutism associated with delayed porphyria, the distribution of exposed parts of the sun and the appearance of scleroderma, and the positive urinary porphyrin test can be identified.
Classical EBA patients can also have hair loss, dislocation, finger fibrosis and esophageal stricture.
2. Bullous pemphigoid-like manifestations: patients with generalized inflammatory bullae and vesicular lesions, located in the trunk and skin wrinkles and limbs, bullous lesions are tension, surrounded by inflammation Urticaria-like skin lesions.
3. Scar-like pemphigus-like damage: Acquired bullous epidermolysis can have mucosal involvement, and in severe cases, it is similar to scar-like pemphigus.
4.Brusting-Perry scar-like pemphigus-like performance: clinically similar to Brusting-Perry scar-like pemphigoid, IgG antibodies against dense anchoring fibrils can be found in serum, similar to scar-like blister The EBA of the sore is rare, less than 10% of EBA patients.
Examine
Examination of acquired bullous epidermis
Histopathology: The lesions were characterized by subepidermal bullae and true epidermis. The degree of inflammatory cell infiltration was consistent with the clinical appearance. Lymphocytes, monocytes, neutrophils and eosinophils were seen in the inflammatory cells.
Observation by electron microscopy: The number of anchor fibrils is reduced, and the amorphous electron dense band under the dense plate may be formed in the gap between the basement membranes where the dense plates are separated.
Direct immunofluorescence: IgG, IgA, IgM, B factor, etc. are deposited in the true epidermal junction site, which is the most common type of immunoglobulin, showing a linear fluorescent band with a basement membrane. Indirect immunofluorescence can detect EBA patients. The serum has an IgG autoantibody against the true epidermal binding site.
Immunoelectron microscopy: The location of immunoprecipitation in the true epidermal junction is the gold standard for EBA diagnosis. The immunoprecipitation of EBA patients is located below the dense plate of the basement membrane zone. This is completely different from bullous pemphigoid, and its immunodeposition is located. The hemidesmosome region or the basement membrane transparent plate and the target antigen of the scar-like pemphigoid are confined to the transparent plate.
Indirect immunofluorescence test for salt-separated skin: Incubation of normal human skin in 1 M NaCl allows the clear epidermal junction to be clearly separated from the clear plate site, which allows the bullous pemphigoid (BP) antigen to be located on the epidermal side. While other basement membrane components are located on the isolated dermal side, this isolated skin can be used to identify EBA and BP sera. If the serum antibody is IgG and is labeled on the epidermal side, the diagnosis of BP should be considered. Conversely, antibody binding To isolate the dermal side of the skin, the patient should be considered EBA or bullous SLE.
Direct immunofluorescence assay of saline-separated skin: Skin lesions were isolated from true epidermal binding with 1 M cold saline, and staining by direct immunofluorescence showed that the immunoprecipitates were located on the dermal side of the isolated skin.
Western immunoagglutination test: The serum antibody of EBA patients can bind to the 290kDa band protein containing type VII collagen human basement membrane. This protein band is the 2 chain of type VII collagen. Recently, the enzyme-specific immunosorbent assay has been used to determine the serum specific resistance of EBA patients. Type VII collagen antibodies are more sensitive than immunofluorescence and Western immunoagglutination assays.
Diagnosis
Diagnosis and differentiation of acquired bullous epidermis
Diagnostic criteria
This diagnostic standard was first proposed by Yaoit et al in 1981 and has been improved as follows:
1 clinical damage similar to dystrophic bullous epidermolysis, including skin fragile and vulnerable, trauma caused by bullous damage, atrophic scar, millet rash, nail malnutrition.
2 no family history of bullous epidermolysis, adult onset.
3 histopathology showed subepidermal blisters.
4 IgG is deposited at the true epidermal junction.
5 Direct immunoelectron microscopy IgG deposition was localized to the lower dense plate and/or dense plate under the true epidermal junction.
TCM pathogenesis and syndrome differentiation
1. Chinese medicine pathogenesis
It is believed that the disease is mostly due to congenital loss, insufficient fetal elements, unsatisfactory endowment, spleen and kidney yang deficiency; or due to sputum in the cell, residual heat, legacy, and external friction.
2. TCM syndrome differentiation
(1) spleen deficiency wet type:
Main card: The general health condition is acceptable, the blister is not the same size, the tension is full, the content is serous, there is no inflammation around, the stool is thin, the tongue is light, the tongue is fat and has tooth marks, the white or white greasy moss, and the pulse is slow.
Dialectical: spleen and dampness, water and wet overflow.
(2) spleen and kidney yang deficiency type:
Main card: more common in infants and children, children are thin, thin hair, soft or hair loss, poor teeth development, soft or falling nails, hands and feet are not warm, or often cyanosis, often have more diarrhea, skin blisters Or blisters, pale tongue or fat body, white or less moss, fine pulse.
Syndrome differentiation: spleen and kidney yang deficiency, lack of blood and blood (this type is more common in dominant dystrophy type).
Differential diagnosis
Different from bullous epidermolysis; no family history, adult onset is the key.
