proliferative pemphigus
Introduction
Introduction to proliferative pemphigus Proliferative pemphigus is a benign type of pemphigus vulgaris, which is rare. Patients are generally young people with lower immunity. Skin lesions occur in the armpits, under the breasts, groin, vulva, around the anus, nasolabial folds and limbs. The damage was initially a thin-walled blisters with a positive Nissl sign. After smashing, papillary granulation is gradually proliferated on the erosion surface, and new blisters often appear on the edges, so that the damage area is gradually enlarged. Nie's sign is positive. The wrinkles are warm and humid, and are susceptible to secondary bacterial and candida infections, often with odor. The old damage surface is slightly dry and papillate. The course of the disease is chronic and the prognosis is good. The characteristics of clinical damage often only see a red wound or erosion surface. If the probe can be inserted into the lower surface of the epithelium or under the surface of the adjacent mucosa without resistance, the Nissl's sign is positive, or the positive skin test is positive, which is helpful for diagnosis. However, do not use the peeling test on a large scale to avoid increasing the suffering of patients. The general condition of the patient is characterized by decreased constitution and even sub-quality, which is also helpful for diagnosis. basic knowledge Sickness ratio: 0.002%-0.004% Susceptible people: no specific population Mode of infection: non-infectious Complications: sepsis
Cause
Cause of proliferative pemphigus
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.
Prevention
Proliferative 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 is UV-sterilized once a day for 30 minutes - 1 hour each time.
3 Flatten the patient's nails to avoid scratching the walls.
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 and appropriate removal, there are bulls, first pump Apply blister fluid and apply Chinese medicine.
5 Avoid cold to prevent upper respiratory tract infections, strengthen nutrition and enhance the body's resistance.
6 Observe the changes in body temperature at any time.
7 long-term use of corticosteroids should be supplemented with potassium to prevent hypokalemia. Sufficient sensitive antibiotics or antifungal agents should be given if there is a bacterial or fungal infection.
Complication
Proliferative pemphigus complications Complications sepsis
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
Proliferative pemphigus symptoms Common symptoms Blisters or bullae damage progressive granulomatous ulcer necrosis Skin mild erosion
(1) Oral: Same as the vulgaris, but there is often a significant proliferation in the red line of the lips.
(2) Skin: The bullae are common in wrinkles such as the armpit, umbilicus and anus. The Nissl sign is positive. The base of the blisters is papillary. The upper part is covered with yellow thick sputum and exudate. , conscious pain. There is a narrow red around. The blister can be fused, the range is variable, and the secondary infection has high fever. The patient's body gradually weakened and often died of secondary infection.
(3) The same damage can occur in the nasal cavity, labia, glans, etc.
Examine
Proliferative pemphigus examination
Immunofluorescence
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 sequestration at the junction of the epidermis and dermis, similar to that seen in lupus erythematosus.
Pathology: Intraepithelial decomposing blister, acantic lysis (acantholysis) can be found under electron microscopy to dissociate at the center of the desmosome, and the ability to lose binding between cells. This is also the pathological basis of the Nikolsky's sign.
Thoracic lysis cells (Tzandk cells) can be found in vesicles
There is a villus formation at the base 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 blistering blister occurs in the superficial epidermis (under the horn layer or within the granule layer)
Diagnosis
Diagnosis and prognosis of proliferative pemphigus
diagnosis
The characteristics of clinical damage often only see a red wound or erosion surface. If the probe can be inserted into the lower surface of the epithelium or under the surface of the adjacent mucosa without resistance, the Nissl's sign is positive, or the positive skin test is positive, which is helpful for diagnosis. However, do not use the peeling test on a large scale to avoid increasing the suffering of patients. The general condition of the patient is characterized by decreased constitution and even sub-quality, which is also helpful for diagnosis.
Differential diagnosis
Different from other skin diseases.
