pemphigus foliaceus
Introduction
Introduction to defoliating pemphigus Deciduous pemphigus occurs in middle-aged and elderly people. Skin lesions mainly occur in the head, face and chest, upper back, and oral mucosa is rare. Blisters often occur on the basis of erythema. Nissl's sign is positive, compared with the common form. Compared with the disease, the condition is mild, the mucosa is rare and slight, and the blister wall is thinner and more susceptible to rupture. The superficial erosion surface is covered with yellowish brown oily and loose leafy epidermis exfoliation, sputum and scales, such as deciduous, due to the decomposition of the back of the carcass secretion to produce odor, the disease develops slowly, gradually the whole body, patients It can also die due to exhaustion or secondary infection. The main use of Prednisolone (Prednisolone) or Prednisone (Prednisone) 40-60mg / day, depending on the condition of the use of hydrocortisone 300mg intravenous drip, after the disease control plan slow reduction. Until the prednisolone or prednisone 10-15mg / day maintenance. basic knowledge The proportion of illness: 0.005%--0.0065% Susceptible people: good for middle-aged and elderly Mode of infection: non-infectious Complications: sepsis pneumonia
Cause
The cause of deciduous pemphigus
The etiology of this disease has not yet been elucidated. At present, most scholars believe that it may be an autoimmune disease. Because of indirect immunofluorescence, it is found that there are specific antibodies (also known as pemphigus antibodies) in the serum of patients with anti-epidermal interstitial substances. It is IgG, and the serum pemphigus antibody titer is parallel to the severity of the disease. The reaction site of the pemphigus antibody is histopathologically the site of the pemphigus (the site where the spine dissociation occurs). This antibody acts on the junction between epidermal cells.
The etiology of pemphigus is unknown. At present, there are many studies on the cause of autoimmune diseases. It is believed that the stimulation of viral infection, ultraviolet radiation, and certain drugs (such as penicillamine) makes the adhesion substance between the spinous cell layers become self-antigen. It is related to the induction of autoimmune response.
Drug stimulation (30%):
Stimulation of certain drugs (such as penicillamine) can cause the disease. Penicillamine is a metabolite of penicillin, which can be prepared by hydrolyzing penicillin. It is an effective copper-extracting agent for the treatment of hepatolenticular degeneration caused by copper metabolism disorders.
Ultraviolet radiation (30%):
Excessive ultraviolet light causes photochemical reactions, which can cause a series of changes in human functions, especially to the human skin, eyes and immune system.
Virus infection (30%):
May be infected with a certain virus, causing the disease.
Prevention
Deciduous pemphigus prevention
1. Keep the mattress clean, flat, and the area of the skin lesion is large. All cloths are disinfected and used.
2, the hospital room UV disinfection once a day, each 30 minutes -1 hour.
3. Flatten the patient's nails to avoid scratching the wall.
4, skin lesions have erosion, exudate and purulent secretions or stench, according to the doctor can be wet with 1:8000 potassium permanganate solution, thick suede with sterile vegetable oil infiltration after appropriate removal, there are bulls, first Drain the blister fluid and apply Chinese medicine externally.
5, to avoid cold, prevent upper respiratory tract infections, strengthen nutrition to enhance the body's resistance, give high protein, high calorie, high vitamin, low salt diet.
6. Observe the changes in body temperature at any time.
Complication
Deciduous pemphigoid complications Complications sepsis pneumonia
Often accompanied by varying degrees of fever, anorexia, fatigue and so on. Due to large area erosion of the skin, a large amount of body fluid extravasation, protein, electrolytes and body fluids are lost too much, the body is weak, and it is easy to combine secondary infections such as sepsis and pneumonia. Buccal mucosa is the most common site of involvement. The pharyngeal, larynx, and esophageal mucosa may also be affected, and involvement of these sites may result in difficulty in feeding, chewing, and swallowing.
Symptom
Symptoms of deciduous pemphigus Common symptoms Mucosal damage to blister or bullous damage
1. Oral: The mucous membrane is completely normal or slightly red and swollen, and may have superficial erosion.
2, the skin: showed a loose bullae, the blister has yellow-brown scaly sputum, the edge is lifted into a leaf shape.
3, conjunctiva and vulvar mucosa are also often affected.
Examine
Examination of deciduous pemphigus
1. Nie's sign is positive. May be associated with oral mucosal damage.
2. Histopathology showed blistering and acanthosis in the epidermis.
3. Immunopathology showed IgG, IgA, IgM or C3 reticular deposition between the spine cells, and the presence of pemphigus antibody in the serum was detected by indirect immunofluorescence.
4. Cytological examination.
After local disinfection, the early fresh bulls are cut off to the blister top, the blister tissue is lightly scraped, applied to the slide, dried and stained with Giemsa or red hematoxylin-eosin. The typical acantholytic cell is disintegrated. . The nucleus is large and round, with deep staining and less cytoplasm, also known as pemphigus cells or acantholytic cells.
Diagnosis
Diagnosis and identification of deciduous pemphigus
Diagnosis and identification
1. The skin is loose and bullied, covered with crusted, or refractory erosion surface.
2. Mucosa, especially the oral mucosa, is often an early symptom of pemphigus.
3. Nikolsky's sign is positive.
4. The blister blister can be found in pemphigus cells (Tzanck cells).
5. Immunofluorescence check:
a. Direct method: There is IgG and C3 deposition between the epidermal cells of the lesion. In addition, IgA and IgM are seen in approximately 25-30% of patients. About 60% of the non-lesioned parts have IgG and C3 deposition.
b. Indirect method: About 100% of the patient's serum has pemphigus antibodies. Antibody titers and conditions are roughly parallel.
In addition to the above, erythematous pemphigus is also positive for antinuclear antibodies, with IgG and C3 deposition in the junction of the epidermis and dermis, similar to that seen in lupus erythematosus.
6. Pathology: Intraepithelial decomposing blister, anactoholysis can be found under electron microscopy to dissociate the central part of the desmosome, and the ability to lose binding between cells. This is also the pathological basis of the Nikolsky's sign.
In the blisters, it can be found that the spine cells (Tzandk cells) have a villus formation at the bottom of the blister and a mild inflammatory cell infiltration of the dermis.
In addition to the above changes, proliferative pemphigus showed epidermal hyperplasia with pseudoepithelial neoplasia, and most eosinophilic small abscess formation in the epidermis.
Deciduous pemphigus and erythematous pemphigus spine-dissolving blisters occur in the superficial epidermis (under the canopy or within the granular layer).
