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bullous disease

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
Gino A Vena, Maria Concetta Fargnoli, Nicoletta Cassano, Giuseppe Argenziano
The sudden eruption of melanocytic nevi has been associated with a number of conditions, such as bullous skin diseases, immunodeficiency and immunosuppression. The exact mechanisms leading to the development of eruptive melanocytic nevi are unknown. Areas covered: The aim of this article is to review the literature concerning eruptive melanocytic nevi following the administration of immunosuppressive drugs and other medications. Expert opinion: The literature regarding the development of eruptive nevi in association with pharmacological therapies includes a relatively low number of reports...
October 19, 2016: Expert Opinion on Drug Metabolism & Toxicology
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
Armita Kakavand Hamidi, Mohammad Moghaddam, Nasim Hatamnejadian, Ahmad Ebrahimi
OBJECTIVES: Epidermolysis bullosa is one of the most important series of mechano-bullous heritable skin disorders which is categorized into four major types according to the layer that bullae forms within basement membrane zone. In dystrophic form of the disease, blisters are made in the sublamina densa zone, at the level of type VII collagen protein which produce anchoring fibrils. Type VII collagen gene is the only responsible gene for this form. The aim of this study was to survey causative mutations of type VII collagen gene among Iranian patients with epidermolysis bullosa...
August 2016: Iranian Journal of Basic Medical Sciences
Joseph Em van Agteren, Kristin V Carson, Leong Ung Tiong, Brian J Smith
BACKGROUND: Lung volume reduction surgery (LVRS) performed to treat patients with severe diffuse emphysema was reintroduced in the nineties. Lung volume reduction surgery aims to resect damaged emphysematous lung tissue, thereby increasing elastic properties of the lung. This treatment is hypothesised to improve long-term daily functioning and quality of life, although it may be costly and may be associated with risks of morbidity and mortality. Ten years have passed since the last version of this review was prepared, prompting us to perform an update...
October 14, 2016: Cochrane Database of Systematic Reviews
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
Yunhee Im, Saad Farooqi, Adan Mora
Giant bullae often mimic pneumothorax on radiographic appearance. We present the case of a 55-year-old man admitted to a referring hospital with dyspnea, cough, and increasing sputum production; he refused thoracotomy for tension pneumothorax and presented to our hospital for a second opinion. A computed tomography (CT) scan at our hospital revealed a giant bulla, which was managed conservatively as an exacerbation of chronic obstructive pulmonary disease. Thoracic surgery was consulted but advised against bullectomy...
October 2016: Proceedings of the Baylor University Medical Center
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
Zaw Min, Raquel R Garcia, Monika Murillo, Jeffrey M Uchin, Nitin Bhanot
Intravenous vancomycin is a widely used antibiotics, but it causes different types of cutaneous hypersensitivity reactions, ranging from maculopapular rash, red-man syndrome, drug rash with eosinophilia and systemic symptoms, IgA bullous dermatosis, leukocytoclastic vasculitis, Stevens-Johnsons syndrome, to IgE-mediated anaphylaxis. We report an elderly patient with the end-stage renal disease presented with diffuse palpable purpura while receiving IV vancomycin therapy for methicillin-resistant Staphylococcus aureus septicemia...
September 25, 2016: Journal of Infection and Chemotherapy: Official Journal of the Japan Society of Chemotherapy
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
H Zaouri, H Amarouch, N Elmakrini, N Tazi, N Ismaili, L Benzekri, K Senouci, B Hassam
Mastocytosis is a group of diseases related to abnormal accumulation and proliferation of mast cells in one or more organs. They may be associated with an acquired point mutation and the activation of the receptor tyrosine-kinase c-KIT of CFS (mast cell growth factor). The clinical manifestations are varied and secondary to the release of mast cell mediators and/or infiltration of various organs. There are two main types of mastocytosis: pure cutaneous mastocytosis and systemic mastocytosis when more than two organs are involved in mast cell infiltration (bone marrow, gastrointestinal tract, bone, liver and spleen, lymph nodes)...
September 23, 2016: Archives de Pédiatrie: Organe Officiel de la Sociéte Française de Pédiatrie
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
M Larquey, E Mahé
Postinfectious erythema multiforme is an uncommon skin disease in childhood that can have a strong impact, especially in infants if there is involvement of the mucous membranes. The lesion is targeted (central bullous lesions with three concentric circles). Its diagnosis is typically made clinically. Atypical forms are the highly inflammatory, mainly bullous type, with exclusive involvement of the mucous membrane, or recurrent erythema multiforme. The diagnosis of erythema multiforme is frequently suspected in children with urticaria multiforme...
September 20, 2016: Archives de Pédiatrie: Organe Officiel de la Sociéte Française de Pédiatrie
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
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