Pathological causes
The disease causes have not been clarified, most scholars believe that may be an autoimmune disease, because indirect immunofluorescence examination, found that patients with spine in the anti-epidermal cell material specific antibody , the main Is IgG, pemphigus antibody in the serum and the severity of the disease in parallel. Pemphigus antibody response on the part of the pathology of the disease pemphigus site . The role of antibodies in the epidermal cells with the Department.
Clinical performance
Pemphigus vulgaris is the special-shaped, good skin lesions occurred in Toumian, armpits, harness, etc.. Relaxation is the beginning of the blister, Nikolsky's sign positive, Pokui after the formation of erosion, nipple V hyperplasia, surface contamination, Jiejia, stench.
Often violated oral, nasal, labia minora, the anus, etc. mucosa, blister easily into a surface erosion.
Symptoms was not obvious, sometimes accompanied by high fever and other systemic symptoms. Lesion at the light at the heavy, often for years.
Some people think that the proliferation of pemphigus can be divided into two types: ① heavy and multi-phase transitional pemphigus vulgaris. ② light had no blisters in only a small part of wiping the pustules and papillary proliferation. Clinical and value-added dermatitis Nanyiqubie, using direct immunofluorescence examination revealed the disease in the epidermal cells between IgG calmly.
Diagnosis and differential
1. Anetoderma bullosa, a review Jiejia, or the erosion of refractory.
2. Mucosa in particular, often find oral mucosa of the early symptoms of pemphigus.
3.Nikolsky Levy's positive.
4. Blister blisters at the end of scratching film, you can see for pemphigus cells
5. Immunofluorescence inspection
a, the direct method
: Lesions of the epidermal cells between IgG and C3 calmly. In addition about 25-30% of the patients that have IgA and IgM. Non-lesion about 60% of the Department of IgG and C3 calmly.
b, indirect method: in the serum of patients, approximately 100% pemphigus antibodies. Antibody titers and conditions are generally parallel.
Erythematosus pemphigus addition to the above findings, there are anti-nuclear antibody positive, the epidermis and dermis and occupies a IgG and C3 calm, similar to lupus erythematosus seen.
6. Pathology: skin blisters in the spine of melting, melting spike in the electron microscope can be found desmosome center of dissociation due to the loss of cell capacity. This is also Nikolsky's sign of pathological basis.
In blister spine release can be found within the cells
Blisters formed at the bottom of a villi, leather mild inflammatory cell infiltration.
In addition to the proliferation of pemphigus the above changes, the epidermal hyperplasia showed that a false-like change, a majority of eosinophils in the epidermal cells small abscess formation.
Defoliating pemphigus and erythema pemphigus spike melting of blisters in the skin of shallow .
Treatment
1. Glucocorticoid: The main use of prednisolone or prednisone 40-60mg / day, depending on condition can also be used hydrogenated de-300 mg intravenous drip, after the disease control plan by the slow drug. Until prednisolone or strong pine 10-15 mg / day of capacity.
2. Immunosuppressant: The main choice of cyclophosphamide 100 mg / day , to be in stable condition after the gradual reduction to zero. Or azathioprine 50 mg / 2 times / day.
3. Kim therapy: the general treatment of difficult therapy, especially for Jiuzhibuyu the mucosal erosion, the therapy can be used as replacement therapy. The main use of glucose thiosulfate for the use of intramuscular injection once every week or every other day, gradually increase.
No. 1 → the first four weeks last 10 mg. No. 5 → 8a 25 mg. No. 9 → the first 12 weeks of a 50 mg →.. 13 weeks after a 50 mg. The total up to 1000 mg.
4. Removing plasma : This therapy is aimed at patients with the abnormal protein component in particular, antibodies and immune complexes and other harmful substances to remove the non-proliferation and replacing it with fresh plasma of healthy, fresh frozen plasma Or albumin preparation, this law because of side effects from corticosteroid is a complementary therapy. 1000ml / day for three consecutive days for a course of treatment, a course of a week, for three courses.
5. Chinese medicine treatment:
a, Chi Sheng-toxic heat: Law to Qingrejiedu, serum cooler business. The use of carbon situ. Carbon double flowers, lotus heart, Rhizoma Imperatae, pollen, and the small, the Health and sticks, the fleas off, the Health and licorice, Chuen Lian, Sheng Shigao, high fever and rhino horn Butui to 0.5 grams, dry stool to increase the yellow.
b, Xinhuo spleen wet-type: Law to Qingxin Xiehuo. Side with Fuling, white tricks, Cangzhu, Huang Ling, situ, Zexie, the Health and licorice, Forsythia, Yuanming Fen, Deng Xin, bamboo leaves, Fructus Aurantii. High fever, and Dai run, Sheng Shigao, Xinhuochisheng lotus seeds to increase heart, berberine, oral mucosa erosion, and Golden Lotus, Jin Quehua, dry stool to increase the yellow.
c, Qiyin two injury-type: Law to Yiqi Yin, the legacies of the Qing. American ginseng, with North and South Shashen, Dendrobium, black yuan Senate and bergamot Senate, the Health Effects, dry situ, Salvia, double flowers, the British public, the winter and Yuzhu.
6. Illness as the need to strengthen support for anti-infection therapy and treatment.
7. Partial treatment: good for exposure, can be used anti-infection of paint or two percent of Long Danzi. Oral mucosal surface damage can contain the narcotic drug agents and antibiotics, such as one percent can be reached Ronning, one percent of Soukou neomycin.
