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Bullous pemphigoid

Hiroaki Iwata, Mayumi Kamaguchi, Hideyuki Ujiie, Machiko Nishimura, Kentaro Izumi, Ken Natsuga, Satoru Shinkuma, Wataru Nishie, Hiroshi Shimizu
Macropinocytosis is an endocytic pathway that is involved in the nonselective fluid uptake of extracellular fluid. Bullous pemphigoid (BP) is an autoimmune subepidermal blistering disease associated with autoantibodies to type XVII collagen (COL17), which is a component of hemidesmosome. When keratinocytes are treated with BP-IgG, COL17 internalizes into cells by way of the macropinocytosis. We investigated the mechanism of COL17 macropinocytosis using DJM-1 cells, a cutaneous squamous cell carcinoma cell line...
October 24, 2016: Laboratory Investigation; a Journal of Technical Methods and Pathology
Mirjana V Milinković, Slavenka Janković, Ljiljana Medenica, Miloš Nikolić, Vesna Reljić, Svetlana Popadić, Janko Janković
BACKGROUND AND OBJECTIVES: While most previous surveys on the clinico-epidemiological features of autoimmune bullous diseases (AIBDs) have predominantly focused on a single disease entity or just one disease group, there have been only few studies examining the incidence of various AIBDs. In the present study, we set out to determine the spectrum of AIBDs, to estimate the incidence of the most common AIBDs, and to examine their temporal trends in Central Serbia over a period of 20 years...
October 2016: Journal der Deutschen Dermatologischen Gesellschaft, Journal of the German Society of Dermatology: JDDG
Hisashi Nomura, Takeru Funakoshi, Ayumi Baba, Akiko Tanikawa, Kazuhito Hayakawa, Masayuki Amagai
No abstract text is available yet for this article.
October 20, 2016: European Journal of Dermatology: EJD
Kyle T Amber, Christine M Panganiban, Dorota Korta, Sebastien de Feraudy, Kristen M Kelly, Sergei A Grando
The association of bullous pemphigoid with melanoma remains controversial and poorly understood. Recent studies report the presence of the bullous pemphigoid antigen, BP180, in melanoma cells, yet not normal melanocytes, suggesting an underlying mechanism for cases of melanoma-associated bullous pemphigoid. We report on an 88-year-old woman who showed a temporal relationship between the development of bullous pemphigoid and melanoma. The patient did not receive programmed death ligand 1 inhibitor therapy and improved rapidly following complete excision of her melanoma, with clobetasol, doxycycline, and niacinamide...
October 18, 2016: Melanoma Research
Justin C Chia, P Régine Mydlarski
PURPOSE: Omalizumab is a recombinant humanized monoclonal antibody that inhibits the binding of IgE to the high-affinity IgE receptor (FceRI) on the surface of mast cells and basophils. Omalizumab has been approved for use in asthma, and new reports show promise in a variety of dermatologic diseases. Herein, we review the literature on omalizumab in dermatology and discuss the safety, efficacy and mechanisms of action for this emerging therapy. MATERIALS AND METHODS: PubMED, MEDLINE, and Embase databases were searched for the period January 1, 1990, to September 1, 2016...
October 19, 2016: Journal of Dermatological Treatment
Katarzyna Osipowicz, Agnieszka Kalinska-Bienias, Cezary Kowalewski, Katarzyna Wozniak
Haemodialysis is the most frequent form of renal replacement therapy (RRT) in patients with end-stage renal disorder (ESRD). Patients with ESRD frequently develop skin problems, mainly xerosis, pruritus and hyperpigmentation, as well as bullous diseases, mainly porphyria or pseudoporphyria and, in some cases, bullous pemphigoid (BP). BP is the most common autoimmune sub-epidermal blistering disease, and it predominantly affects elderly people. Clinically, BP is characterised by generalised pruritic, bullous eruptions and urticaria-like lesions...
October 17, 2016: International Wound Journal
Minhee Kim, Luca Borradori, Dédée F Murrell
Elderly patients are more susceptible to the development of autoimmune blistering disorders such as bullous pemphigoid, mucous membrane pemphigoid, epidermolysis bullosa acquisita, and paraneoplastic pemphigus. This article focuses on the clinical aspects of the aforementioned autoimmune blistering diseases and highlights the important factors involved in treating elderly patients. It is essential for clinicians to offer individualized treatment plans for these patients to optimize outcomes, as elderly patients often have multiple co-morbidities, polypharmacy, and suboptimal socioeconomic status that can adversely influence adequate compliance...
October 13, 2016: Drugs & Aging
J Loget, J Plee, F Antonicelli, P Bernard
Predisposing factors for bullous pemphigoid (BP), the most common autoimmune blistering disease, include neurological disorders, chronic use of certain drugs (spironolactone, loop diuretics, psycholeptics).(1) The coexistence of psoriasis with classical BP is occasionally observed, whereas half of the patient population with anti-laminin γ1 pemphigoid of Japanese origin have coexisting psoriasis.(2-3) We report for the first time a case of relapsing BP associated with psoriasis, in which both dermatoses completely disappeared after treatment with ustekinumab, a monoclonal antibody that targets the p40 subunit of IL-12/23...
October 12, 2016: Journal of the European Academy of Dermatology and Venereology: JEADV
Marta Wieczorek, Annette Czernik
Paraneoplastic pemphigus (PNP) is a fatal autoimmune blistering disease associated with an underlying malignancy. It is a newly recognized blistering disease, which was first recognized in 1990 by Dr Anhalt who described an atypical pemphigus with associated neoplasia. In 2001, Nguyen proposed the term paraneoplastic autoimmune multiorgan syndrome because of the recognition that the condition affects multiple organ systems. PNP presents most frequently between 45 and 70 years old, but it also occurs in children and adolescents...
2016: Clinical, Cosmetic and Investigational Dermatology
T Hashimoto, A Ohzono, K Teye, S Numata, S Hiroyasu, D Tsuruta, T Hachiya, K Kuroda, M Hashiguchi, T Kawakami, N Ishii
BACKGROUND: IgE autoantibodies are considered to be involved in pathogenesis of bullous pemphigoid (BP), particularly inflammatory and erythematous phenotypes. OBJECTIVE: To develop reliable ELISAs for detection of IgE autoantibodies to both BP180 and BP230 in BP sera, and to compare the ELISA results with clinical features. METHODS: We used commercially available IgG ELISAs to develop IgE ELISAs for both BP180 and BP230. To determine the influence of excess amount of IgG autoantibodies, all normal and BP sera were tested before and after IgG adsorption...
October 7, 2016: British Journal of Dermatology
Ulla Derhaschnig, Jim Gilbert, Ulrich Jäger, Georg Böhmig, Georg Stingl, Bernd Jilma
BACKGROUND: Innovative trial designs are sought to streamline drug development in rare diseases. Basket- and integrated protocol designs are two of these new strategies and have been applied in a handful oncologic trials. We have taken the concept outside the realm of oncology and report about a first-in-human integrated protocol design that facilitates the transition from phase Ia in healthy volunteers to phase Ib in patients with rare complement-mediated disorders driven by the classical pathway...
October 4, 2016: Orphanet Journal of Rare Diseases
Kahori Nasu, Norio Hanafusa, Masaomi Nangaku
Bullous pemphigoid (BP) is an autoimmune blistering skin disorder characterized by circulating serum IgG antibodies against two hemidesmosomal proteins: BP180 and BP230. Fundamentally, immunosuppressive therapies are administered to treat this disease, but plasmapheresis can be added for refractory patients. We experienced the case of a 63-year-old patient with refractory BP for which we administered double filtration plasmapheresis (DFPP). His skin lesions improved along with decreased IgG BP180 antibodies, but factor XIII (FXIII) and fibrinogen were also reduced by DFPP repetition...
October 6, 2016: Journal of Clinical Apheresis
Ana Sofía Ayala-Cortés, Sylvia Aide Martínez-Cabriales, Oliverio Welsh, Gloria Rosales-Solis, Osvaldo Vázquez-Martínez, Jorge Ocampo-Candiani
No abstract text is available yet for this article.
September 30, 2016: Journal of Pediatrics
S Goetze, A K Dumke, D Zillikens, U C Hipler, P Elsner
Anti-laminin γ1-pemphigoid (formerly known as anti-p200 pemphigoid) is a rare subepidermal autoimmunobullous disorder first described by Zillikens et al. in 1996 (1,2). Clinically, it mimics bullous pemphigoid, linear IgA dermatosis, dermatitis herpetiformis, pompholyx or the inflammatory variant of epidermolysis bullosa acquisita, respectively (1,3). The laminin γ1-chain has been identified as the major targeting antigen (1,4,5). Histologically, a subepidermal split is seen accompanied by neutrophils and eosinophils as well as linear deposits of IgG and/or C3 along the dermoepidermal junction in direct immunofluorescence microscopy (1)...
September 29, 2016: Journal of the European Academy of Dermatology and Venereology: JEADV
Axelle Bonhomme, Claire Poreaux, Fabienne Jouen, Jean-Luc Schmutz, Pierre Gillet, Annick Barbaud
Nigella sativa oil (NSO), extracted from the plant Nigella sativa, is traditionally used in Africa and Asia for many therapeutic properties. Thymoquinone, its main constituent, has cellular and molecular targets, with pharmacological activities proved against various diseases.(1) We report the succession of an atypical Stevens-Johnson syndrome (SJS)/toxic epidermal necrolysis (TEN) overlap to NSO and bullous pemphigoid (BP). Furthermore, we review the cases of bullous cutaneous adverse drug reactions (CADRs) with traditional herbal medicines (THM)...
September 29, 2016: Journal of the European Academy of Dermatology and Venereology: JEADV
Maryam Daneshpazhooh, Javad Khorassani, Kamran Balighi, Narges Ghandi, Hamidreza Mahmoudi, Hamidreza Tohidinik, Shahin Hamzelou, Cheyda Chams-Davatchi
INTRODUCTION: Neurological diseases are important co-morbidities found in association with bullous pemphigoid. Various neurological conditions (stroke, Parkinson's disease, dementia, epilepsy and multiple sclerosis) have been reported as associations of this bullous disease; whether these are significant has not been definitely proved. However, the presence of neurological conditions is a predictor of poorer prognosis. OBJECTIVES: Our aim was to examine the association of bullous pemphigoid and neurological diseases in Iranian bullous pemphigoid patients...
September 26, 2016: Indian Journal of Dermatology, Venereology and Leprology
Susanna Esposito, Sophie Guez, Annalisa Orenti, Gianluca Tadini, Giulietta Scuvera, Laura Corti, Alessia Scala, Elia Biganzoli, Emilio Berti, Nicola Principi
In order to evaluate the serum anti-skin autoantibodies and cytokine concentrations in patients with different epidermolysis bullosa (EB) types and severity, 42 EB patients and 38 controls were enrolled. Serum anti-skin antibodies were significantly higher in the patients than in the controls (p = 0.008, p < 0.001, p < 0.001, p < 0.001 and p < 0.001 for desmoglein 1 (DSG1) desmoglein 3 (DSG3), bullous pemphigoid 180 (BP180), BP230 and type VII collagen (COL7), respectively). The same trend was observed for interleukin (IL)-1β, IL-2, IL-6, IL-10, tumor necrosis factor-β, and interferon-γ (p < 0...
2016: International Journal of Molecular Sciences
Nina Caca-Biljanovska, Irina Arsovska-Bezhoska, Marija V'lckova-Laskoska
The association between psoriasis vulgaris and bullous pemphigoid is due to the still unclear autoimmune process. The common disease site is the dermo-epidermal junction or basal membrane zone (BMZ), with specific alterations for both diseases. Photochemotherapy (PUVA) is one of the therapeutic modalities for psoriasis and can trigger production of autoantibodies against antigens in the BMZ in patients with subclinical bullous pemphigoid. Furthermore, PUVA therapy can alter the immunological milieu and hence can contribute to the expression of bullous pemphigoid in patients with psoriasis...
August 2016: Acta Dermatovenerologica Croatica: ADC
Nina Kokkonen, Sanna-Kaisa Herukka, Laura Huilaja, Merja Kokki, Anne M Koivisto, Päivi Hartikainen, Anne M Remes, Kaisa Tasanen
Bullous pemphigoid (BP) is a subepidermal blistering skin disease, which has shown a strong association with neurological diseases in epidemiological studies. The BP autoantigens BP180 and BP230 are expressed in the cutaneous basement membrane and the central nervous system. Using BP180 and BP230 ELISA assays and immunoblotting against BP180 we analyzed the IgG reactivity in the sera of 115 patients with Alzheimer's disease (AD) and 40 neurologically healthy controls. BP180 autoantibodies were found in 18 % of AD patients, whereas only 3% of controls had positive results (P=0...
September 17, 2016: Journal of Investigative Dermatology
Matthew D Belcher, Ismail Kaddour-Djebbar, Wendy B Bollag, Loretta S Davis
No abstract text is available yet for this article.
October 2016: Journal of the American Academy of Dermatology
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