Correlation Between Glucocorticoid Receptor Gene Polymorphism And Bullous Pemphigus

Bullous pemphigoid(bullous pemphigiod, BP) is a chronic autoimmune mucocutaneous disease. Its treatment has been of concern to everyone. Currently, the system is still glucocorticoid therapy as first-line BP, BP glucocorticoid therapy can effectively control the disease and reduce mortality, it is diagnosed after BP, should be timely, adequate, regular application of glucocorticoid therapy. However, a large number of long-term use of glucocorticoids often cause some adverse reactions, especially for the elderly, and even lead to increased mortality. In clinical work, we found that aging and high-dose corticosteroids are the most important risk factor for fatal, so correctly grasp the initial dose glucocorticoids, timely maintenance treatment of skin lesions subsided after reduction and disease control is particularly important. For BP individualized treatment, according to the severity of the patient’s situation mucosal damage, complications, hospital medical conditions and adverse drug reactions to the development of rational treatment. But for specific patients, especially those associated with more elderly patients with underlying diseases, how to do individualized therapeutic doses worth further exploration.With the extensive use of glucocorticoids, BP cure rate is greatly improved, though there are still some patients difficult to obtain effective treatment, one important reason for the failure is the presence of resistance to drugs, drug resistance and thus generate research mechanisms to improve the cure rate for the disease and prevent complications has important clinical significance. As the drug of choice for the treatment of BP- glucocorticoids(glucocorticoid, GC) of the effect is caused by the glucocorticoid receptor(glucocorticoid receptor, GR) mediated achieved, but there is a big difference in reactivity of GC treatment of different individuals, even treatment-resistant. Thus, to clarify the mechanism of glucocorticoid resistance BP treatment will have a significant impact, the current mechanism for the primary glucocorticoid resistance is not clear, most studies to explore the mechanism of hormone resistance from the perspective of hormone receptors, mainly in study of hormone receptor gene. Studies have reported that glucocorticoid receptor gene(nuclear receptor subfamily 3, group C, member 1, NR3C1) polymorphisms affect GR function, is considered one of the important mechanisms of GC resistance has been found with GC susceptibility genes are mainly: ER22 / 23 EK, N363 S, BC1 I, 9β so on. Part 1 Retrospective clinical analysis of 88 cases of bullous pemphigoidObjective:To summarize the clinical features and treatment experience of bullous pemphigoid(hereafter referred to as BP), so as to explore the best treatment options for BP.Methods:A retrospective analysis of Affiliated Hospital of Hebei University of Engineering Dermatology in January 2006 admitted in December 2013 during the medical records of 88 cases of patients hospitalized BP on age of onset, duration, lesion characteristics, severity, pathology and immunofluorescence pathology, retrospective analysis of laboratory results, serum lgE value, complications, length of hospital stay and treatment programs, clinical efficacy.Results:In 88 patients, male 45 cases, female 43 cases, male: female = 1.05: 1, aged 28 to 95 years, mean age(65.53 ± 16.83) years, of which <40 years of two cases(2.27%) 40 to 60 years of 16 cases(18.2%), ≥60 years of age in 70 cases(79.5%); duration 7d ~ 4 years, the average(5.17 ± 8.19) months <3 months 87 cases, 3-12 March 1 cases, ≥12 months 0 cases. First onset 81 cases, seven cases of recurrence. Clinical cure in 30 cases, improved in 53 cases, healed in 4 cases, 1 death.Conclusion:1 A bullous pemphigoid common in the elderly, patients with moderate to mild age and sex was not statisticallyTotal learning differences.2 bullous pemphigoid associated with underlying diseases, of which the highest rate of cardiovascular disease, neurological diseases associated with BP in patients with severe illness.3 immunofluorescence and histopathological diagnosis is mainly based on the direct immunofluorescence basement membrane zone mainly IgG, C3 linear fluorescent, IIF detection antibody positive rate and severity of skin lesions without parallel4 Severity and mucosal damage is an important factor in the initial amount of hormones and maximum control and other indicators of the impact. In patients with severe combined immune inhibitors can control the disease and reduce the amount of hormones BP, select immunosuppressants should emphasize individual to improve the quality of life of patients.5 For conventional therapy and high-dose corticosteroids in patients without contraindications can be applied corticosteroid therapy.6 GC combined system is still the preferred immunosuppressive therapy BP, but the GC topical efficacy and safety has been recognized by more and more scholars.7 Select the appropriate control dosage according to the lesion area in the acute phase; follow-up period of medication to control the speed of sound. Part 2 Glucocorticoid receptor gene polymorphism and bullous pemphigus correlationObjective: Detection of glucocorticoid receptor(glucocorticoid receptor, GR) gene N363 S, ER22 / 23 EK, BclI, 9β polymorphism and its relationship with bullous pemphigoid(bullous pemphigoid, BP) glucocorticoids(hereinafter referred to as hormone) resistance in. Will clear the mechanism of glucocorticoid resistance, and further predict the response of hormone therapy, to provide a basis for regulating the amount of hormones, early BP hormone resistance may occur in patients using immunosuppressive agents, such as joint measures to prevent blindness caused by high doses of hormones deputy reaction, so that patients get more benefits. Meanwhile, the study also will be filled on the glucocorticoid receptor gene polymorphism and bullous pemphigoid correlation between the gaps.Methods:On January 2006 December 2013 occupancy of 40 cases in our hospital dermatology hormone-sensitive group, 48 cases of hormone resistance group and 42 healthy controls, application SNaPshotSNP typing on three groups GR gene N363 S, ER22 / 23 EK, BclI, 9β polymorphism analysis to determine its correlation with hormone resistance.Results:Hormone-sensitive group, hormone resistance group and the healthy control group were detected BclI there are three kinds of genotypes CC, CG, GG; 3 group GR gene locus N363 S, ER22/23 EK, 9β mutations were not found, there is no multi-state sex. 4 locus genotype comparison, the three groups showed no significant difference(P> 0.05).Conclusion:1 Glucocorticoid receptor gene loci BclI found mutations in this group of patients with bullous blistering day in.2 A glucocorticoid receptor gene loci N363 S, ER22/23 EK, 9β gene mutation was not found in this group of patients with bullous day in blisters.3 Glucocorticoid receptor gene N363 S, ER22/23 EK, BclI, 9β polymorphisms and bullous pemphigoid clinical features, treatment outcome was not statistically associated with prognosis.4 Glucocorticoid receptor gene N363 S, ER22/23 EK, BclI, 9β polymorphism and BP hormone resistance may no significant correlation.