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Ocular cicatricial pemphigoid:diagnosis and systemic management
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作者 Claudia Castiblanco 《Annals of Eye Science》 2024年第2期38-52,共15页
Ocular cicatricial pemphigoid(OCP)is a subcategory of mucous membrane pemphigoid(MMP)where the conjunctiva is the main site of inflammation.It is a chronic and autoimmune disease characterized by acute and chronic con... Ocular cicatricial pemphigoid(OCP)is a subcategory of mucous membrane pemphigoid(MMP)where the conjunctiva is the main site of inflammation.It is a chronic and autoimmune disease characterized by acute and chronic conjunctivitis that can progress to severe conjunctival cicatrization,corneal opacification,ocular surface keratinization,and eyelid abnormalities.OCP can lead to structural damage that can result in visual impairment,visual loss,and blindness,and can have a significant impact in a patient’s quality of life.Patients may manifest with varying symptoms,degrees of severity and may have different rates of progression.Early diagnosis and appropriate systemic immunosuppression are of utmost importance for prompt and adequate disease control.Various systemic immunomodulatory therapies(IMTs),including anti-metabolites,alkylating,and biologic agents have been utilized to achieve inflammation control and remission.Careful monitoring of disease progression is important to assess response and to modify and escalate therapy if needed.Treatment to alleviate symptoms of dry eye disease and address trichiasis and other eyelid abnormalities is recommended as well.A multidisciplinary approach to optimize clinical care is recommended in the management of patients with OCP.This review will address the immunopathogenesis,clinical features,keys to diagnosis and staging of patients with OCP.It will highlight the current immunomodulators utilized for disease management and proposed stepladder strategies.This review will discuss the updated roles of combination therapy,novel use of biologics as well as the recent use of adrenocorticotropic hormone(ACTH)analog in severe recalcitrant cases. 展开更多
关键词 Ocular cicatricial pemphigoid(OCP) mucous membrane pemphigoid(MMP) basement membrane zone(BMZ) symblepharon immunomodulatory therapy(IMT)
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Bullous Pemphigoid Induced by Doxycycline: Case Report and Literature Review
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作者 Mishal Al Mebayadh Rahaf Aldawish +1 位作者 Sohail A. Butt Abdulrahman Saad Aljahhan 《Case Reports in Clinical Medicine》 2024年第5期187-192,共6页
Bullous pemphigoid (BP) is the most prevalent autoimmune sub-epidermal blistering disease that affects mainly the elderly and could lead to serious morbidity. It has numerous risk factors and triggers, including an ag... Bullous pemphigoid (BP) is the most prevalent autoimmune sub-epidermal blistering disease that affects mainly the elderly and could lead to serious morbidity. It has numerous risk factors and triggers, including an aging population with several comorbidities and drug exposure. In the published paper, we reported a case about a 32 years old male patient with unknown medical conditions who presented with erythematous patches and plaques on the scalp, face, and trunk with scattered blisters two weeks after initiating doxycycline treatment for folliculitis. The exact pathogenesis of the drug-reaction in drug- associated bullous pemphigoid (DABP) remains controversial. In conclusion, it is crucial for clinicians to be aware of DABP when prescribing doxycycline. The purpose of this case report is to raise awareness of the possible association between bullous pemphigoid and doxycycline. 展开更多
关键词 Bullous pemphigoid DOXYCYCLINE Drug-Associated Bullous pemphigoid
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Bullous pemphigoid associated with acquired hemophilia A: A case report
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作者 Su-Ye Hu Meng-Can Li +7 位作者 Zi-Jia Hao Xu-Ya Chai Pei-Sai Li Yang Liu Li-Xia Liu Ying Xu Pan-Pan Yang Ling-E Li 《World Journal of Clinical Cases》 SCIE 2025年第4期28-33,共6页
BACKGROUND Acquired hemophilia A (AHA) is a rare and potentially severe bleeding disordercaused by circulating autoantibodies against factor Ⅷ (FⅧ). In approximately50% of the patients, the condition is associated w... BACKGROUND Acquired hemophilia A (AHA) is a rare and potentially severe bleeding disordercaused by circulating autoantibodies against factor Ⅷ (FⅧ). In approximately50% of the patients, the condition is associated with autoimmune diseases,cancers, medication use, pregnancy, and the post-partum period. Bullous pemphigoid(BP) is a chronic autoimmune subepidermal blistering disease associatedwith tissue-bound and circulating autoantibodies against BP antigens 180 (BP180)and 230 (BP230). AHA-associated BP has a high mortality rate;hence, the understandingof this disease must improve.CASE SUMMARY A 69-year-old man presented with erythema, blisters, blood blisters, and crustsaccompanied by severe pruritus for more than 20 days, and ecchymosis andswelling on his left upper arm for 3 days. Pathological examination revealed asubepidermal blister that contained eosinophils. Laboratory tests showed that theBP180 autoantibody levels had increased, isolated activated partial thromboplastintime was notably prolonged (115.6 s), and coagulation FⅧ activity wasextremely low (< 1.0%). Furthermore, the FⅧ inhibitor titer had greatlyincreased (59.2 Bethesda units). Therefore, the patient was diagnosed as having BP associated with AHA, prescribed 0.05% topical halometasone cream, and transferred to a higher-level hospitalfor effective treatment;however, he died after 2 days.CONCLUSION AHA associated BP is rare, dangerous, and has a high mortality rate. Therefore, its timely diagnosis and effectivetreatment are necessary. 展开更多
关键词 Bullous pemphigoid Acquired hemophilia A Acquired hemophilia A FactorⅧ Case report
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Systemic Immune-Inflammation Index (SII) and Neutrophil-Lymphocyte Ratio (NLR) as Systemic Inflammatory Predictors in the Diagnosis of Bullous Pemphigoid and Pemphigus Vulgaris
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作者 Mulubwa Changa Chibesa Mengqi Guan Shanshan Li 《Journal of Cosmetics, Dermatological Sciences and Applications》 2024年第2期211-225,共15页
Introduction: Autoimmune blistering skin disorders such as Bullous Pemphigoid and Pemphigus Vulgaris present diagnostic challenges. The Systemic Immune-Inflammation Index (SII) and Neutrophil-Lymphocyte Ratio (NLR), a... Introduction: Autoimmune blistering skin disorders such as Bullous Pemphigoid and Pemphigus Vulgaris present diagnostic challenges. The Systemic Immune-Inflammation Index (SII) and Neutrophil-Lymphocyte Ratio (NLR), are inflammatory markers used to assess the body’s immune-inflammatory response. Objectives: The study aims to evaluate the significance of hematologic markers, specifically the Systemic Immune-Inflammation Index (SII) and Neutrophil-Lymphocyte Ratio (NLR), as diagnostic predictors of bullous pemphigoid (BP) and pemphigus vulgaris (PV). Methods: A retrospective study of 64 patients (36 with BP and 28 with PV). Patient clinical data: age, gender, complete blood count, autoimmune antibody levels (Dsg1, 3 and BP180, 230), IgE and C-reactive protein, and history of hypertension, diabetes, brain infarction, and coronary heart disease. The data was analyzed using SPSS. Results: The study involved 36 (56.3%) diagnosed with bullous pemphigoid (BP) and 28 (43.75%) with pemphigus vulgaris (PV). The average age in BP was 71 ± 8 and 52 ± 13 in PV. Laboratory findings showed high levels of Dsg1, Dsg3, neutrophil count, and lymphocyte count in PV, while high levels of eosinophils with a significant increase in C-reactive protein (CRP) in BP. Blood biomarkers, including NLR, PLR, SII, MPV, CRP, and IgE, proved an overall of 84.4% in disease prediction. Dsg1, Dsg3, BP180, and BP230 showed an overall of 88.1%. No significant relationship was noted between NLR, SII, and patients with comorbidities. Conclusion: The study highlights the diagnostic potential of SII and NLR in addition to hematologic markers in BP and PV, emphasizing their role in early diagnosis and therapeutic interventions, requiring further validation in larger patient cohorts. 展开更多
关键词 pemphigoid VULGARIS Neutrophil-Lymphocyte IMMUNE-INFLAMMATION Autoimmune
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Rituximab Therapy for Persistent, Severe and Extensive Idiopathic Bullous Pemphigoid
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作者 Shaima Al-Bader Kamel El-Reshaid 《Journal of Cosmetics, Dermatological Sciences and Applications》 CAS 2023年第1期76-83,共8页
Background: Idiopathic Bullous Pemphigoid (IBP) is a rare blistering autoimmune disease. Its morbidity and mortality have remained high owing to complications of extensive skin involvement as well as its conventional ... Background: Idiopathic Bullous Pemphigoid (IBP) is a rare blistering autoimmune disease. Its morbidity and mortality have remained high owing to complications of extensive skin involvement as well as its conventional steroid therapy. We reviewed the medical literature and found indicators of an autoimmune etiology for its pathogenesis triggering genetically predisposed patients. Objective: to evaluate, prospectively, the role of Rituximab (R) therapy in its persistent, severe and extensive form. Patients and methods: A total of 12 patients, with disease duration of 6 ± 1 months, were treated with yearly R infusions (1 g followed by 1 g 2 weeks later). Results: Significant clinical improvement was achieved as documented by decrease in total score of Bullous Pemphigoid Disease Area Index from 60 ± 3 to 6 ± 2 that persisted for 26 ± 11 months of follow up. Moreover, IBP autoantibodies (anti-BP 180 and anti-320 IgG) levels fell from to 91 ± 3 and 81 ± 2 to 8 ± 2 and 9 ± 2, respectively. Conclusions: R is a safe and effective treatment for severe IBP and such response further confirms its autoimmune pathogenesis. 展开更多
关键词 Bullous pemphigoid RITUXIMAB Treatment BP Autoantibodies Bullous pemphigoid Disease Area Index
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Urticarial Bullous Pemphigoid: A New Case Report
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作者 Waqas S. Abdulwahhab Fatima M. Al Qaydi 《Journal of Cosmetics, Dermatological Sciences and Applications》 CAS 2022年第3期145-152,共8页
Background: Bullous pemphigoid (BP) is the most common autoimmune bullous disease and it primarily affects the elderly population. It typically presents with tense bullae and severe pruritus. Non-bullous pemphigoid is... Background: Bullous pemphigoid (BP) is the most common autoimmune bullous disease and it primarily affects the elderly population. It typically presents with tense bullae and severe pruritus. Non-bullous pemphigoid is a subtype of BP characterized by lacking the typical bullae formation with different presentations including erythematous, eczematous, urticarial, polycyclic, targetoid, nodular, lichenoid, vesicular and erythrodermic. Aim: to document a new case presentation of urticarial BP who was treated for several years as chronic urticarial and chronic eczema. Case Report: A 56-year-old male patient presented with a history of recurrent severely pruritic urticarial wheals for 4 years duration involving the lower abdomen, lower back, upper and lower extremities associated with excoriations, keratosis, and post-inflammatory hyperpigmentations on resolved sites, diagnosed as urticarial BP on histopathology & direct immunofluorescence study (DIF) and was improved on systemic doxycycline therapy. Conclusion: BP can be presented with atypical manifestation. Urticarial BP is a rare variant of non-bullous pemphigoid that should be taken into consideration in the differential diagnosis of an atypical urticarial wheal not responding to conventional therapy. 展开更多
关键词 Bullous pemphigoid Non-Bullous pemphigoid Urticarial Bullous pemphigoid Direct Immunofluorescence DOXYCYCLINE
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Pseudopemphigoid as caused by topical drugs and pemphigus disease 被引量:1
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作者 Laura C Huang James R Wong +5 位作者 Javier Alonso-Llamazares Carlos H Nousari Victor L Perez Guillermo Amescua Carol L Karp Anat Galor 《World Journal of Ophthalmology》 2015年第1期1-15,共15页
Pseudopemphigoid can cause a chronic cicatricial conjunctivitis that is clinically identical to the manifestations seen in mucous membrane pemphigoid, a disorder with a common clinical phenotype and multiple autoimmun... Pseudopemphigoid can cause a chronic cicatricial conjunctivitis that is clinically identical to the manifestations seen in mucous membrane pemphigoid, a disorder with a common clinical phenotype and multiple autoimmune links. For the purpose of this review, we will describe pseudopemphigoid as caused by topical drugs, the most common etiology with ocular manifestations, and as caused by the pemphigus disease, a more rare etiology. Specifically, we will discuss the ophthalmological features of drug-induced cicatricial conjunctivitis, pemphigus vulgaris, and paraneoplastic pemphigus. Other etiologies of pseudopemphigoid exist that will not be described in this review including autoimmune or inflammatory conditions such as lichen planus, sarcoidosis, granulomatosis with polyangiitis(Wegener's granulomatosis), erythema multiforme(minor, major, and Stevens-Johnson syndrome), bullous pemphigoid, skin-dominated linear IgA bullous dermatosis, and skin-dominated epidermolysis bullosa acquisita. Prompt diagnosis of the underlying etiology in pseudopemphigoid is paramount to the patient's outcome as certain diseases are associated with a more severe clinical course, increased ocular involvement, and differential response to treatment. A completehistory and ocular examination may find early cicatricial changes in the conjunctiva that are important to note and evaluate to avoid progression to more severe disease manifestations. When such cicatricial changes are noted, proper diagnostic techniques are needed to help elucidate a diagnosis. Lastly, collaboration between ophthalmologists and subspecialists such as dermatologists, pathologists, immunologists, and others involved in the care of the patient is needed to ensure optimal management of disease. 展开更多
关键词 Pseudopemphigoid Mucous membrane pemphigoid CICATRICIAL CONJUNCTIVITIS PEMPHIGUS vulgaris PARANEOPLASTIC PEMPHIGUS Drug-induced CONJUNCTIVAL cicatrization
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Clinical features and in vivo confocal microscopy assessment in 12 patients with ocular cicatricial pemphigoid 被引量:2
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作者 Qin Long Ya-Gang Zuo +3 位作者 Xue Yang Ting-Ting Gao Jie Liu Ying Li 《International Journal of Ophthalmology(English edition)》 SCIE CAS 2016年第5期730-737,共8页
AIM: To describe the clinical features and microstructural characteristics assessed by in vivo confocal microscopy(IVCM) in patients with ocular cicatricial pemphigoid(OCP).· METHODS: A descriptive, uncontr... AIM: To describe the clinical features and microstructural characteristics assessed by in vivo confocal microscopy(IVCM) in patients with ocular cicatricial pemphigoid(OCP).· METHODS: A descriptive, uncontrolled case series study. Patients diagnosed with OCP were examined by clinical history, slit-lamp biomicroscopy features and IVCM images. The results of direct immunofluorescence(DIF) biopsies and indirect immunofluorescence(IIF) were also recorded. Local and systemic immunosuppressive therapy were administered and adjusted according to response.·RESULTS: A total of 12 consecutive OCP patients(7male, 5 female; mean age 60.42 ±10.39y) were recruited.All patients exhibited bilateral progressive conjunctival scarring and recurrent chronic conjunctivitis was the most frequent clinical pattern. The mean duration of symptoms prior to diagnosis of OCP was 2.95 ±2.85y(range: 5mo to 10y). The Foster classification varied from stage I to IV and 20 eyes(83%) were within or greater than Foster stage Ⅲ on presentation. Two of the 12patients(17%) demonstrated positive DIF; 3 of the 12(25%) patients reported positive IIF. The mean duration of the follow-up period was 20.17 ±11.88mo(range: 6 to48mo). IVCM showed variable degrees of abnormality in the conjuctiva-cornea and conjuctival scarring was detected in all the involved eyes. Corneal stromal cell activation and dendritic cell infiltration presented asocular surface inflammation, ocular surface keratinization along with the destroyed Vogt palisades was noted in eyes with potential limbal stem cell deficiency. After treatment, remission of ocular surface inflammation was achieved in all the patients, 18 eyes(75%) remained stable, 6 eyes(25%) had recurrent conjunctivitis and cicatrization in 2 eyes(8%) was progressing.· CONCLUSION: As an autoimmune disease, OCP manifests as variable degrees of clinical and laboratory abnormalities with both local and systemic immunosuppressive treatment playing important roles in disease therapy. IVCM can be as a valuable non-invasive technique to assess ocular surface changes in a cellular level with a potential value for providing diagnostic evidence and monitoring therapeutic effects during follow-up. 展开更多
关键词 ocular cicatricial pemphigoid ocular surface disease in vivo confocal microscopy
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Incidence, prevalence, and demographic characteristics of ocular cicatricial pemphigoid in Colombia: data from the National Health Registry 2009-2019
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作者 Carlos Cifuentes-González Stefania Amaris-Martínez +2 位作者 Juliana Reyes-Guanes Pilar Uribe-Reina Alejandra de-la-Torre 《International Journal of Ophthalmology(English edition)》 SCIE CAS 2021年第11期1765-1770,共6页
AIM:To provide an epidemiological characterization of ocular cicatricial pemphigoid(OCP)in Colombia.METHODS:We conducted a cross-sectional study using SISPRO.We applied the specific code of the International Classific... AIM:To provide an epidemiological characterization of ocular cicatricial pemphigoid(OCP)in Colombia.METHODS:We conducted a cross-sectional study using SISPRO.We applied the specific code of the International Classification of Diseases for Ocular Pemphigoid,from 2009-2019 to estimate prevalence,incidence,and the demographic status of the disease in Colombia.RESULTS:The estimated average prevalence was 0.22 per 1000000 inhabitants,and the estimated average incidence was 0.24 per 1000000 inhabitants.With a female predominance of 62.5%,and a male/female ratio of 1:1.6.The group of patients diagnosed with the disease after the age of 80 presented the highest prevalence.The departments with the highest prevalence were Antioquia,Bogotá,and Santander.CONCLUSION:There are important differences between worldwide and Colombian prevalence and incidence data,which may be related to genetic and epigenetic factors,and the possible underdiagnosis of the disease.According to the results,OCP is an extremely rare disease in Colombia.Nevertheless,it is important to encourage awareness of the disease due to its devastating consequences. 展开更多
关键词 ocular cicatricial pemphigoid EPIDEMIOLOGY incidenee ophthalmology Colombia autoimmune diseases
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Cicatricial Pemphigoid in Accompany with Rheumatoid Arthritis:a Case Report
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作者 Di Wu Li-ming Zhang Ying Jiang 《Chinese Medical Sciences Journal》 CAS CSCD 2010年第3期182-184,共3页
CICATRICIAL pemphigoid (CP, also known as benign mucous membrane pemphigoid) is a rare chronic autoimmune subepithelial blister- ing disease, with an incidence of 1 per million,characterized by erosive lesions of mu... CICATRICIAL pemphigoid (CP, also known as benign mucous membrane pemphigoid) is a rare chronic autoimmune subepithelial blister- ing disease, with an incidence of 1 per million,characterized by erosive lesions of mucous membranes and skin that result in scarring. Rheumatoid arthritis (RA) is a symmetric inflammatory arthritis that mainly affects the small joints of hands and feet, with a prevalence of 0.3% in China.3 In this case report we described the diagnosis of and treatment for a patient developing CP 18 years after the onset of RA, a combination rarely encountered or reported so far. 展开更多
关键词 cicatricial pemphigoid SYMBLEPHARON rheumatoid arthritis
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Diagnosis,fetal risk and treatment of pemphigoid gestationis in pregnancy:A case report
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作者 Hai-Ning Jiao Ye-Ping Ruan +2 位作者 Yan Liu Meng Pan Hui-Ping Zhong 《World Journal of Clinical Cases》 SCIE 2021年第34期10645-10651,共7页
BACKGROUND Pemphigoid gestationis(PG)is a rare autoimmune blistering disease that usually presents in the second or third trimester,with an incidence of 1 per 50000 pregnancies.PG tends to recur with an earlier onset ... BACKGROUND Pemphigoid gestationis(PG)is a rare autoimmune blistering disease that usually presents in the second or third trimester,with an incidence of 1 per 50000 pregnancies.PG tends to recur with an earlier onset and a more severe course in subsequent pregnancies.Skin biopsy markers can be confirmed by direct immunofluorescence staining.CASE SUMMARY Our patient was diagnosed with PG at 8 mo of gestation with fresh bullous lesion marks on the abdomen and limbs.Termination of the pregnancy was performed by cesarean section at 37+4 wk of gestation.The patient delivered an infant weighing 3620 gm.The infant had urticaria-like and vesicular skin lesions and was diagnosed with PG.The patient was discharged on prednisolone and in a satisfactory condition.The infant was discharged after anti-inflammatory therapy for one week.CONCLUSION PG is a rarely reported disease,and 10%of newborns develop mild clinical symptoms consisting of urticaria-like or vesicular skin lesions.We intend to remind clinicians to consider this condition when a patient presents with such lesions so that treatment can be started early and neonatal morbidity can be taken into account. 展开更多
关键词 pemphigoid gestationis PREGNANCY NEWBORN PEMPHIGUS Fluorescent antibody techniqu Case report
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Coexistence of ocular cicatricial pemphigoid with Stevens Johnson syndrome
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作者 Hande Taylan Sekeroglu Firas Simsek +3 位作者 Elif Erdem Ibrahim Inan Harbiyeli Meltem Yagmur Reha Ersoz 《International Journal of Ophthalmology(English edition)》 SCIE CAS 2013年第3期411-412,共2页
Dear Sir, I am Dr. Hande Taylan Sekeroglu, from the Ophthalmology Department of Cukurova University Faculty of Medicine. I want to present a case of recalcitrant ocular cicatricial pemphigoid (OCP) which was coinciden... Dear Sir, I am Dr. Hande Taylan Sekeroglu, from the Ophthalmology Department of Cukurova University Faculty of Medicine. I want to present a case of recalcitrant ocular cicatricial pemphigoid (OCP) which was coincidently diagnosed with Stevens 展开更多
关键词 Coexistence of ocular cicatricial pemphigoid with Stevens Johnson syndrome ORAL OCP FIGURE
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Topical use of ozone effectively alleviates the acute symptoms and quality of life of patients with moderate to severe bullous pemphigoid: a randomized controlled trial
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作者 Li Li Li-Yun Sang +4 位作者 Wen-Guang Zhang Tong Wang Shu-Ping Guo Hong-Zhou Cui Hong-Ye Liu 《Frontiers of Nursing》 2021年第3期233-240,共8页
Objective:To evaluate the adjuvant treatment of acute stage symptoms and quality of life of patients with bullous pemphigoid(BP)with ozone.Methods:We included 74 patients with moderate and severe BP hospitalized from ... Objective:To evaluate the adjuvant treatment of acute stage symptoms and quality of life of patients with bullous pemphigoid(BP)with ozone.Methods:We included 74 patients with moderate and severe BP hospitalized from July 2018 to December 2020.The participants were randomly divided into the control group and the ozone group,with 37 patients in each group(n=37).The control group used tap water to wash the whole body,and mupirocin ointment was used for the erosions.The ozone group used ozone water to wash the whole body,and the erosion place was coated with ozone oil.The bullous pemphigoid disease area index(BPDAI)score of BP disease was used to observe the clinical symptoms and skin lesions of the two groups before and on day 3,7,and 14 after treatment.Pruritus and sleep were observed before and on day 3,7,and 14 after treatment.The Dermatology Life Quality Index(DLQI)was used to observe the quality of life before and after treatment and the adverse reactions were recorded.Results:The total BPDAI score and erythema score in the ozone group were significantly different from those in the control group on day 3,7,and 14 after treatment(P<0.050).The scab drying time and erosion drying time of ozone group were shorter than that of control group(P<0.001).There were statistically significant differences between the ozone group and the control group in pruritus score and sleep score on day 3,7,and 14 after treatment(P<0.001).There was statistically significant difference in skin disease quality of life between the two groups 14 days after treatment(P<0.001).No significant adverse reactions and complications were observed in both groups.Conclusions:Ozone external use can rapidly improve the acute stage of edema erythema,erosion,and itching of BP,improve the quality of life of patients,is an effective treatment method,and wor thy of clinical promotion. 展开更多
关键词 bullous pemphigoid erosion ERYTHEMA external use ITCH moderate-severe OZONE quality of life
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Expression of MMP-2 and MMP-13 in Bullous Pemphigoid
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作者 Haixiang Zhang Xiaoxu Shi +4 位作者 Lanying Qin Zishen Zhao Guojun Fu Jun Peng Wenjing Ye 《Journal of Clinical and Nursing Research》 2020年第1期7-10,共4页
Objective:To investigate the expression and significance of matrix metalloproteinase-2(MMP-2)and matrix metalloproteinase-13(MMP-13)in bullous pemphigoid(BP)skin lesions.Methods:Immunohistochemical SP method was used ... Objective:To investigate the expression and significance of matrix metalloproteinase-2(MMP-2)and matrix metalloproteinase-13(MMP-13)in bullous pemphigoid(BP)skin lesions.Methods:Immunohistochemical SP method was used to detect the expression of MMP-2 and MMP-13 in 32 BP skin lesions,and compared with 15 normal skin tissues.Results:The expression of MMP-2 in the case group was significantly increased(38.56±10.06)compared to the normal control group(21.20±5.98);the expression of MMP-13 in the case group was significantly augmented(18.62±5.90)compared to the normal control group(11.47±8.484).The expressions of MMP-2 and MMP-13 in the skin lesions of patients with bullous pemphigoid were statistically different from those of normal people(both P<0.05).Compared with the expression of MMP-2 and MMP-13 in bullous pemphigoid,the expression of MMP-2 and MMP-13 was moderately correlated(correlation coefficient was 0.523).Conclusion:The expression of MMP-2 and MMP-13 is significantly increased in bullous pemphigoid skin lesions,suggesting that they may play an important role in the pathogenesis of BP.There is a certain correlation between the expression of MMP-2 and MMP-13,suggesting that the high expression of MMP-13 may play a role in the mechanism that further leads to the high expression of MMP-2. 展开更多
关键词 MATRIX METALLOPROTEINASE MMP-2 MMP-13 BULLOUS pemphigoid
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Rare bullous pemphigoid during PD-1 inhibitor therapy:a case report
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作者 Zuo-Peng Xiao Meng-Jun Nie Xi Zou 《Cancer Advances》 2022年第6期1-4,共4页
Immunotherapy is an important treatment modality in cancer,but it can also cause adverse reactions,with skin toxicity being the most common.The increasing number of immune checkpoint inhibitors being used in the clini... Immunotherapy is an important treatment modality in cancer,but it can also cause adverse reactions,with skin toxicity being the most common.The increasing number of immune checkpoint inhibitors being used in the clinic will inevitably cause an increase in the rate of adverse skin reactions that markedly affect the patient's quality of life.A 58-year-old patient with intrahepatic cholangiocareinoma developed bullous pemphigoid(BP)nearly a year after using immune checkpoint inhibitors,which is different from what has been reported inthe literature within two weeks of treatment.Pathologically,the skin biopsy diagnosis was epidermal hyperplasia and focal sub-epidermal pustule formation,consistent with drug-induced dermatitis.The patient was treated with methylprednisolone,minocyeline,colchicine,nicotinamide,triamcinolone,and traditional Chinese medicine decoction.No new blisters developed after 1 week of treatment.The medication was gradually discontinued,and BP did not recur.Clinicians should carefully consider the risk-benefit ratio when using PD-1 inhibitors,particularly concerning rash severity.Further studies are needed to investigate the relationship between adverse skin reactions and drug efficacy. 展开更多
关键词 case report immune checkpoint inhibitors bullous pemphigoid adverse skin reactions treatment
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Preliminary Application of High-Frequency Ultrasound in the Differentiation of Pemphigus and Bullous Pemphigoid:An Observational Study
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作者 Yang-Ai Liu Rong Cao +2 位作者 Mei-Hong Da Zheng-Bang Dong Fei Wang 《International Journal of Dermatology and Venereology》 CSCD 2024年第3期155-159,共5页
Objective:Ultrasonography is a diagnostic imaging technique used to visualize subcutaneous body structures for identification of possible pathologies.In this study,we aimed to explore the clinical significance of high... Objective:Ultrasonography is a diagnostic imaging technique used to visualize subcutaneous body structures for identification of possible pathologies.In this study,we aimed to explore the clinical significance of high-frequency ultrasound in differentiating the location of blisters between pemphigus and bullous pemphigoid.Methods:Eighteen patients were recruited in Department of Dermatology,Zhongda Hospital from 2020 to 2021 and divided into a pemphigus group(n=8)and a bullous pemphigoid group(n=10)according to the diagnostic criteria for each.Ultrasonographic images were collected using a 50-MHz high-frequency ultrasound system.The indexes of ultrasonic evaluation were the layer(epidermis or dermis),size,shape,internal echo,and boundary of the blister.Categorical variables are expressed as n(%),and differences were compared using Fisher’s exact test.Results:The ultrasonographic images in the pemphigus group showed an intraepidermal semi-arc or irregular anechoic or hypoechoic areas.The inferior borders were situated above the characteristic thin linear hyperechoic bands of the epidermis.A linear hypoechoic band was present at the dermoepidermal junction in some cases.In the ultrasonographic images of the bullous pemphigoid group,the linear hyperechoic bands of the epidermis were continuous and intact.An oval fluid anechoic area(subepidermal blister)was present immediately below the bands.The location of the blisters in the ultrasonographic images was significantly different between the 2 groups(P<0.001).Conclusion:High-frequency ultrasound can be used as an auxiliary means to differentiate the location of blisters between pemphigus and bullous pemphigoid. 展开更多
关键词 high-frequency ultrasound PEMPHIGUS bullous pemphigoid
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Bullous Pemphigoid With Nail Damage Associated With Kaposi Sarcoma:A Case Report
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作者 Feng-Xia Hu Jun-Qin Liang +2 位作者 Dilinuer Abudoureyimu Cao-Ying Wu Xiao-Jing Kang 《International Journal of Dermatology and Venereology》 CSCD 2023年第4期233-235,共3页
Introduction:Bullous pemphigoid is an acquired autoimmune bullous skin disease that occurs in the elderly,and nail involvement is relatively rare.Kaposi sarcoma(KS) is a rare opportunistic tumor in patients with iatro... Introduction:Bullous pemphigoid is an acquired autoimmune bullous skin disease that occurs in the elderly,and nail involvement is relatively rare.Kaposi sarcoma(KS) is a rare opportunistic tumor in patients with iatrogenic immunosuppression and is rarely associated with autoimmune bullotic disease.Case presentation:The patient was diagnosed with bullous pemphigoid based on her history,bullous pemphigoid antibody,and skin pathological examination.And nail involvement occurred 20 days prior to the recurrence of the disease.Moreover,KS occurred during high-dose glucocorticoid therapy in this patient.Discussion:The patient was admitted to hospital because of a five-month history of erythema and blisters and a one-month history of nodules and plaques.The diagnosis of bullous pemphigoid was followed by treatment with glucocorticoids and intravenous gamma globulin.During treatment,the disease recurred due to discontinuation of glucocorticoids.There were erythema and blisters all over the body,and purple nodules,plaques appeared in the base of some blisters.Pathological examination of nodules was consistent with KS.In addition,nail involvement occurred 20 days before recurrence.The particularity of this case lies in the simultaneous occurrence of nail involvement,bullous pemphigoid,and KS.Conclusion:Patients with autoimmune bullous skin disease may suddenly develop abnormal nails,and such patients should be vigilant about recurrence of the disease.Doctors should be aware of the possibility of KS when rapidly progressing purplish red plaques or nodules appear in patients receiving high doses of corticosteroids or immunosuppressants. 展开更多
关键词 bullous pemphigoid nail damage Kaposi sarcoma TUMOR BLISTER
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临床药师参与1例大疱性类天疱疮、肺曲霉病合并播散性皮疽诺卡菌感染治疗的药学实践
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作者 邓体瑛 林敏 +4 位作者 胡志敏 邹亮 吴志红 刘剑敏 黄蕾 《中国药房》 CAS 北大核心 2024年第16期2038-2043,共6页
目的为大疱性类天疱疮、肺曲霉病合并播散性皮疽诺卡菌感染患者的抗菌药物方案调整、不良反应识别和个体化药学监护提供参考。方法临床药师参与1例大疱性类天疱疮、肺曲霉病合并播散性皮疽诺卡菌感染患者的治疗全过程,结合循证证据协助... 目的为大疱性类天疱疮、肺曲霉病合并播散性皮疽诺卡菌感染患者的抗菌药物方案调整、不良反应识别和个体化药学监护提供参考。方法临床药师参与1例大疱性类天疱疮、肺曲霉病合并播散性皮疽诺卡菌感染患者的治疗全过程,结合循证证据协助临床遴选初期抗皮疽诺卡菌感染的联合治疗方案,并及时沟通微生物室以提供早期药敏数据;在患者出现癫痫时甄别可疑药物,并提醒临床亚胺培南西司他丁钠会影响丙戊酸钠疗效,建议将丙戊酸钠换为左乙拉西坦抗癫痫治疗,并停用亚胺培南西司他丁钠;治疗期间,建议临床对伏立康唑、利奈唑胺进行血药浓度监测,并根据监测结果协助临床及时调整用药剂量。结果医生采纳临床药师建议。患者病情好转,带药出院。结论临床药师基于循证医学证据、药敏试验结果和血药浓度监测数据,协助临床为患者选择敏感的抗感染方案;及时识别不良反应并调整治疗方案,并为患者提供全程用药监护,保证了临床药物治疗的安全性和有效性。 展开更多
关键词 皮疽诺卡菌感染 大疱性类天疱疮 肺曲霉病 癫痫 临床药师 药学监护
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寻常性银屑病合并自身免疫性大疱性皮肤病13例临床分析
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作者 周健 刘宇 +1 位作者 俞晨 王刚 《临床皮肤科杂志》 CAS CSCD 北大核心 2024年第7期387-392,共6页
目的:分析寻常性银屑病合并自身免疫性大疱性皮肤病患者的临床特点、诊断及治疗。方法:回顾性分析2008—2021年西京医院皮肤科确诊寻常性银屑病合并自身免疫性大疱性皮肤病住院患者的临床资料。结果:收集13例寻常性银屑病合并自身免疫... 目的:分析寻常性银屑病合并自身免疫性大疱性皮肤病患者的临床特点、诊断及治疗。方法:回顾性分析2008—2021年西京医院皮肤科确诊寻常性银屑病合并自身免疫性大疱性皮肤病住院患者的临床资料。结果:收集13例寻常性银屑病合并自身免疫性大疱性皮肤病患者,其中男8例,女5例,平均年龄(44.69±8.02)岁。所有患者于银屑病发生后平均(14.85±7.54)年合并自身免疫性大疱性皮肤病。所有患者中7例合并天疱疮(其中1例伴有汞中毒),5例合并大疱性类天疱疮(1例确诊肺腺癌),1例合并成人型线状Ig A大疱性皮病。7例患者采用糖皮质激素和(或)免疫抑制剂治疗,6例予单用免疫抑制剂治疗。出院后随访2个月~13年,10例患者治愈或病情稳定,3例失访。结论:寻常性银屑病可合并多种自身免疫性大疱性皮肤病,临床较为少见。患者多为银屑病病程中出现水疱、大疱性皮损,明确诊断需结合皮损组织病理表现、免疫荧光、疱病自身抗体等检查结果综合判断。提示临床上需警惕合并汞中毒可能,并重视对肿瘤的筛查和随访,根据疱病严重程度制定合理的治疗方案。 展开更多
关键词 银屑病 自身免疫性大疱性皮肤病 天疱疮 大疱性类天疱疮
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白细胞介素-17和白细胞介素-22在大疱性类天疱疮患者皮损及血清中的表达与意义
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作者 崔瑜 吴远慧 +3 位作者 胡彩霞 王文氢 张燕 高顺强 《实用皮肤病学杂志》 2024年第1期25-29,47,共6页
目的检测分析大疱性类天疱疮(BP)患者皮损及血清中白细胞介素(IL)-17和IL-22的表达情况,并探讨其在BP发病机制中的意义。方法选取41例BP患者皮损组织(BP组)及25名健康人正常皮肤组织(对照组),用免疫组化方法检测IL-17和IL-22的表达情况... 目的检测分析大疱性类天疱疮(BP)患者皮损及血清中白细胞介素(IL)-17和IL-22的表达情况,并探讨其在BP发病机制中的意义。方法选取41例BP患者皮损组织(BP组)及25名健康人正常皮肤组织(对照组),用免疫组化方法检测IL-17和IL-22的表达情况。采用酶联免疫吸附试验(ELISA)检测27例BP患者血清中IL-17和IL-22的水平,并以25名健康人作为对照。结果BP组患者皮损中IL-17和IL-22的表达均明显高于对照组,差异有统计学意义(均P<0.01)。且BP组患者皮损组织中IL-17与IL-22的表达呈正相关性。BP组患者血清中IL-17和IL-22的表达水平均高于对照组,差异有统计学意义(均P<0.01)。BP组患者血清中IL-17与IL-22的表达呈正相关性。结论BP患者血清及皮损IL-17和IL-22的表达水平上调,可能参与BP的发病,且两者可能起协同作用。提示3型固有淋巴细胞(ILC3)可能参与BP的致病过程。 展开更多
关键词 类天疱疮 大疱性 白细胞介素-17 白细胞介素-22 固有淋巴细胞
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