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Bullous pemphigoid

Hui Fang, Yang Zhang, Ning Li, Gang Wang, Zhi Liu
Bullous pemphigoid (BP) is an autoimmune and inflammatory skin disease associated with subepidermal blistering and autoantibodies directed against the hemidesmosomal components BP180 and BP230. Animal models of BP were developed by passively transferring anti-BP180 IgG into mice, which recapitulates the key features of human BP. By using these in vivo model systems, key cellular and molecular events leading to the BP disease phenotype are identified, including binding of pathogenic IgG to its target, complement activation of the classical pathway, mast cell degranulation, and infiltration and activation of neutrophils...
2018: Frontiers in Immunology
Xinhua Yu, Anika Kasprick, Karin Hartmann, Frank Petersen
Skin mast cells (MCs), a resident immune cell type with broad regulatory capacity, play an important role in sensing danger signals as well as in the control of the local immune response. It is conceivable to expect that skin MCs regulate autoimmune response and are thus involved in autoimmune diseases in the skin, e.g., autoimmune bullous dermatoses (AIBD). Therefore, exploring the role of MCs in AIBD will improve our understanding of the disease pathogenesis and the search for novel therapeutic targets. Previously, in clinical studies with AIBD, particularly bullous pemphigoid, patients' samples have demonstrated that MCs are likely involved in the development of the diseases...
2018: Frontiers in Immunology
Mayumi Kamaguchi, Hiroaki Iwata, Hideyuki Ujiie, Ken Natsuga, Wataru Nishie, Yoshimasa Kitagawa, Hiroshi Shimizu
The basement membrane zone (BMZ) consists of multiple components, including collagen XVII (COL17), which is the target of bullous pemphigoid (BP). No research has addressed the differences in BMZ components between the skin and the oral mucosa; therefore, we investigated the BMZ proteins, with a focus on COL17. The mRNA and protein expression levels of COL17 were significantly higher in oral keratinocytes (OKCs) than in skin keratinocytes (SKCs). Hemidesmosomal COL17 expression was markedly higher in OKCs than in SKCs, and its level was associated with adhesion strength...
March 9, 2018: Journal of Investigative Dermatology
M Sawada, T Hida, H Ujiie, H Iwata, H Uhara
Epidermolysis bullosa acquisita (EBA) and anti-p200 pemphigoid are uncommon subepidermal autoimmune bullous diseases caused by autoantibodies against the 200-kDa protein and 290-kDa type VII collagen, respectively. Here we describe a patient with autoantibodies against both 200-kDa and 290-kDa antigens.A 63-year-old-man had itchy tense blisters and edematous erythemas scattered on his trunk, buttocks, extremities and soles (Fig. 1a). There were no ocular or mucosal lesions. Psoriatic skin lesions were not observed...
March 10, 2018: Journal of the European Academy of Dermatology and Venereology: JEADV
Hiroshi Koga, Norito Ishii, Chika Ohata, Takekuni Nakama
No abstract text is available yet for this article.
February 1, 2018: European Journal of Dermatology: EJD
Aniek Lamberts, H Ilona Euverman, Jorrit B Terra, Marcel F Jonkman, Barbara Horváth
Introduction: Rituximab (RTX) is a monoclonal antibody targeting CD20, a transmembrane protein expressed on B cells, causing B cell depletion. RTX has shown great efficacy in studies of pemphigus vulgaris, but data of pemphigoid diseases are limited. Objective: To assess the effectiveness and safety of RTX in pemphigoid diseases. Methods: The medical records of 28 patients with pemphigoid diseases that were treated with RTX were reviewed retrospectively...
2018: Frontiers in Immunology
N Yoshimoto, H Ujiie, M Zheng, H Iwata, H Kosumi, H Hata, H Shimizu
Bullous pemphigoid (BP) is the most common subepidermal autoimmune blistering skin disease, characterized by tense bullae and urticarial erythema with a generalized distribution. BP is induced by autoantibodies to structural proteins of the basement membrane zone (BMZ) such as BP180 and BP2301 . Because the majority of BP skin shows complement deposition2 , complement activation is considered to be important for blister formation. Among IgG subclasses, IgG1 have a high ability to activate complements, whereas IgG2 and IgG4 have a low and no ability, respectively...
March 6, 2018: Journal of the European Academy of Dermatology and Venereology: JEADV
Sana Basseri, Thai Yen Ly, Peter R Hull
BACKGROUND: Dyshidrotic pemphigoid (DP) is a rare variant of bullous pemphigoid (BP) that affects the hands and feet and may resemble an acute vesicular eczema. While it can remain confined to hands and feet, spread that involves the entire body is described. BP and DP are associated with autoantibodies directed against hemidesmosomal proteins BP180 (collagen XVII) and BP230 (dystonin), which are transmembrane and intracellular proteins in the basement membrane zone, respectively. CASE SUMMARY: We present a case of DP in a 78-year-old woman who was diagnosed based on histopathologic and immunofluorescence findings and subsequently successfully treated...
March 1, 2018: Journal of Cutaneous Medicine and Surgery
Yonghu Sun, Hong Liu, Zhenzhen Wang, Xi'an Fu, Chuan Wang, Zihao Mi, Lele Sun, Fangfang Bao, Gongqi Yu, Guizhi Zhou, Furen Zhang
No abstract text is available yet for this article.
February 26, 2018: Journal of Investigative Dermatology
J Bagan, Y Jiménez, J Murillo, L Bagan
OBJECTIVE: To analyze the severity of the oral lesions in low-risk oral mucous membrane pemphigoid (OMMP) measured according to the size of the bullous areas and the number of simultaneously affected oral locations. MATERIAL AND METHODS: A total of 100 cases of low-risk OMMP were studied. The symptoms and location of OMMP in the oral cavity were analyzed. The bullous areas were measured, establishing three grades according to the greatest bullous lesion size (grade 1: < 3 cm in size; grade 2: 3-6 cm; and grade 3: > 6 cm)...
March 2018: Oral Diseases
Inês Raposo, Susana Machado, Rita Sampaio, Manuela Selores
Bullous pemphigoid (BP) is an immune mediated bullous disease that is manifested by urticarial plaques with superimposed subepidermal blisters and significant pruritus. It is generally found in the elderly, but is rare in the pediatric population. A 5-month-old girl previously diagnosed with hand-foot-mouth disease was examined in our dermatology department owing to vesicles and bullae, initially located to the hands and feet, which progressed with new lesions. Tense vesicles and bullae distributed in an annular string of pearls pattern on the abdomen and facial and cervical regions were noted...
July 15, 2017: Dermatology Online Journal
Hui Fang, Shuai Shao, Man Jiang, Erle Dang, Shengxian Shen, Jieyu Zhang, Pei Qiao, Caixia Li, Gang Wang
Bullous pemphigoid is an autoimmune inflammatory disorder characterised by the presence of autoantibodies against bullous pemphigoid autoantigens, leading to dermal-epidermal separation with consequent blister formation. However, whether and how the components of blister fluid exacerbate the progression of bullous pemphigoid is unclear. Exosomes are nanometre-sized vesicles released from cells into the body fluid, where they can transmit signals throughout the body. In the present study, we isolated and characterised exosomes from the blister fluids of patients with bullous pemphigoid, evaluated their proinflammatory role, and identified the underlying molecular mechanisms...
February 22, 2018: Journal of Pathology
Yiman Wang, Xuming Mao, Yanhong Wang, Yueping Zeng, Yidi Liu, Hongzhong Jin, Li Li
Bullous Pemphigoid (BP) is a chronic autoimmune blistering disorder that has a predilection for the elderly. It is characterized by a poor prognosis due to its high mortality rate and the tendency to relapse. The relapse rate of BP ranges from 27.87% to 53% after disease remission, while the majority of relapses occur early (within 6 months) during remission. Clinical interventions aimed to prevent early relapses could reduce potential complications from first-line treatment and make follow-up care easier for clinicians in practice, thus improving the prognosis of BP and the quality of patients' lives...
February 19, 2018: Annals of Medicine
Gabriela Cobos, Euphemia Mu, Jeffrey Cohen, Jenna Beasley, Nooshin Brinster, Alisa Femia
Epidermolysis bullosa acquisita (EBA) is a rare, acquired subepidermal blistering disease. EBA is characterized by autoantibodies to collagen VII,which serves to link the epidermis to the dermis. The two most common presentations of EBA are classical noninflammatory EBA and bullous pemphigoid-like EBA. Diagnosis of EBA can be challenging as it sharesclinical and histopathologic features with other blistering diseases. Treatment is often recalcitrant and will often necessitate multiple therapies. We presenta case of a thirty-six-year-old Chinese man with EBA and review the literature...
December 15, 2017: Dermatology Online Journal
E Fulton, F Jan, M J Zimarowski
BACKGROUND: Linear IgA bullous dermatosis (LABD) is an autoimmune subepidermal blistering disease usually with a neutrophil rich inflammatory infiltrate, and characterized by linear IgA deposition at the basement membrane zone (BMZ), and neutrophil predominant dermal inflammation. We report a case of LABD with numerous eosinophils and flame figure formation, a unique histopathologic finding not previously reported. A 69-year-old woman presented with a rapidly progressive, intensely pruritic rash over forearms, breasts, axillae, hips, and thighs...
November 15, 2017: Dermatology Online Journal
Nobuki Maki, Wataru Nishie, Maya Takazawa, Maki Kakurai, Tomoko Yamada, Naoka Umemoto, Masaaki Kawase, Kentaro Izumi, Hiroshi Shimizu, Toshio Demitsu
Bullous pemphigoid (BP) is a common autoimmune blistering disorder with unknown etiology. Recently, increasing numbers of BP cases which developed under the medication with dipeptidyl peptidase-4 inhibitors (DPP4i), widely used antihyperglycemic drugs, have been reported in published works. Here, we report a case of DPP4i (teneligliptin)-associated BP that developed in a 70-year-old Japanese man. Interestingly, the patient had acquired reactive perforating collagenosis (ARPC), which is also known to be associated with the onset of BP...
February 14, 2018: Journal of Dermatology
Abhilash Koratala, William L Clapp, Olanrewaju A Olaoye, Alfonso H Santos
Bullous pemphigoid has been linked to allograft rejection, as well as membranous nephropathy in renal transplant recipients. Although there is a possibility of multiple distinct autoimmune processes, immune stimulation induced by allograft rejection or antibasement zone antibody interactions are possible mechanisms for the simultaneous skin and renal involvement.
February 2018: Clinical Case Reports
T Sach
No abstract text is available yet for this article.
February 2018: British Journal of Dermatology
Outi Varpuluoma, Anna-Kaisa Försti, Jari Jokelainen, Miia Turpeinen, Markku Timonen, Laura Huilaja, Kaisa Tasanen
No abstract text is available yet for this article.
February 7, 2018: Journal of Investigative Dermatology
Maryam Daneshpazhooh, Maryam Ghiasi, Vahideh Lajevardi, Nafise Nasiri, Kamran Balighi, Amir Teimourpour, Hasan Khosravi, Vahide Saeidi, Hamidreza Mahmoudi, Cheyda Chams-Davatchi
Bullous pemphigoid (BP) is an autoimmune bullous disease characterized by autoantibody production against BP180 and BP230. Two scoring systems have been validated for BP including: Bullous Pemphigoid Disease Area Index (BPDAI) and Autoimmune Bullous Skin Disorder Intensity Score (ABSIS). In this study, we investigated correlations between both scoring systems and either anti-BP180 NC16A or anti-BP230 values. BPDAI and ABSIS were used to measure disease activity in 95 BP patients at Razi Hospital in Tehran, Iran...
February 8, 2018: Archives of Dermatological Research
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