The dermal-epidermal junction consists of a network of several interacting structural proteins which strengthen adhesion and mediate signaling events. This structural network consists of hemidesmosomal-anchoring filament complexes connecting the basal keratinocytes to the basement membrane. The anchoring filaments in turn interact with the anchoring fibrils to attach the basement membrane to the underlying dermis. Read More
Bullous pemphigoid (BP) is the most common autoimmune subepidermal blistering disease in Western countries. Although topical and/or systemic glucocorticoids treatment efficacy is widely recognized, up to 30% of patients with BP may undergo a relapse during the first year of treatment. We investigated the protein expression of the total glucocorticoid receptor and GRβ isoform in the skin biopsy specimens from patients with BP, and wondered whether such investigation at baseline provided a tool to predict disease outcome. Read More
Clin Cosmet Investig Dermatol 2017 23;10:317-324. Epub 2017 Aug 23.
Department of Dermatology, University of California San Diego, La Jolla, CA, USA.
Scabies, a parasitic infestation caused by the mite Sarcoptes scabiei, is diagnosed by observing either the mite, its ova, or its excrement. The mite tracts, known as burrows and a characteristic presentation of the pruritic condition, are typically found on the web spaces between the fingers. Other cutaneous lesions include excoriated papules, pustules, and vesicles. Read More
Studies that report the incidence of bullous pemphigoid from validated nationwide population-based registries are rare. The aim of this study was to estimate the incidence of bullous pemphigoid in Sweden 2005-2012. A population-based open cohort study was designed including all patients diagnosed by a dermatologist with bullous pemphigoid (BP) in Sweden from 2005 to 2012 (n = 3761), identified from the National Patient Register (NPR). Read More
Background: Bullous pemphigoid (BP) is an autoimmune disease characterised by tense blisters that are usually preceded by urticarial eruptions. Affected patients exhibit IgG and/or IgE auto-antibodies against BP180 and/or BP230. Their relative importance in disease pathogenesis has not been fully elucidated. Read More
Department of Dermatology, Nantes University Hospital, France.
Anti-programmed death 1 (PD-1) antibodies are revolutionizing the treatment of many cancers, including melanoma (1) . Cutaneous adverse events (AE) of anti-PD-1 antibodies are common (20%) and mainly non-specific. Bullous pemphigoids (BP) are very rare immune-related AEs induced by anti-PD-1 antibodies, with only 12 cases previously published. Read More
Department of Dermatology, Hokkaido University Graduate School of Medicine.
Mucous membrane pemphigoid (MMP) is a rare autoimmune blistering disease targeting various autoantigens, including the C-terminus of collagen XVII (COL17) and laminin332. Bullous pemphigoid (BP) is the most common autoimmune blistering disease, affecting the mucosae in 10-20% of cases. COL17, particularly, the non-collagenous (NC)16A domain is the pathogenic epitope for BP. Read More
Int J Dermatol 2017 Oct 30;56(10):996-1002. Epub 2017 Aug 30.
Department of Dermatology, Medical University of Warsaw, Warsaw, Poland.
Background: Scalp is a common location of autoimmune bullous diseases. Trichoscopy is a noninvasive method for diagnosing hair and scalp diseases. Data on trichoscopy in autoimmune bullous diseases are limited to the studies on pemphigus including a small number of patients. Read More
Int J Dermatol 2017 Oct 30;56(10):1010-1016. Epub 2017 Aug 30.
Department of Dermatology, Tel Aviv Sourasky Medical Center, Tel Aviv, Israel.
Background: Bullous pemphigoid (BP) is the most prevalent autoimmune blistering skin disease in Western countries and in Israel. Initial disease control is achieved in 60-90% of BP patients within 1-4 weeks of corticosteroid therapy. In the remainder of patients, recalcitrant disease is controlled with additional immunosuppressive treatment. Read More
Importance: Pneumocystis pneumonia (PCP) is a potentially lethal opportunistic infection that primary prophylaxis can help prevent. The risk of prophylactic therapy must be weighed against the incidence of PCP in the patient population. Prophylaxis most frequently involves trimethoprim-sulfamethoxazole, with second-line therapies, including atovaquone, dapsone, and pentamide. Read More
Bullous pemphigoid (BP) is associated with higher mortality and coexisting comorbidities, some of them affecting poor prognosis. The aim of the study was to identify prognostic factors causing greater mortality both in the 1st and 3rd year of follow-up and to determine the 1-, 2-, 3-year mortality rates, standardized mortality ratio (SMR) in Polish BP patients. All patients with BP (a cohort of 205 patients, mean age 76. Read More
Bullous pemphigoid (BP) is the most common autoimmune subepidermal blistering skin disease and is characterized by the presence of autoantibodies directed against the hemidesmosomal proteins BP180 and BP230 that can be detected in the skin and serum of BP patients. Histologically, the dermal infiltration of eosinophils is obvious. The objective of this review is to present evidence that eosinophils play a key role in the pathogenesis of BP. Read More
Pemphigoid diseases (PD) are a subgroup of rare acute or chronic autoimmune skin disorders characterized and caused by autoantibodies directed against distinct structural components of the dermal-epidermal junction. Binding of autoantibodies to their targets leads to the formation of blisters and erosions in patients. PDs comprise eight disorders for which the molecular target antigens have been identified. Read More
A 78-year-old man presented with cutaneous blisters of the limbs and abdominal distension. He had been treated for various diseases, including liver cirrhosis. He had begun receiving sitagliptin, a dipeptidyl peptidase-4 (DPP-4) inhibitor, for diabetes mellitus three years before the hospitalization. Read More
Departments of *Dermatology ‡Pathology §Oncology, Johns Hopkins University School of Medicine and Sidney Kimmel Comprehensive Cancer Center ∥The Brady Urologic Institute, Johns Hopkins University School of Medicine, Baltimore, MD Departments of †Medicine, Thoracic Oncology Service ¶Pathology #Dermatology, Memorial Sloan Kettering Cancer Center, New York, NY.
Cutaneous eruptions are among the most common immune-related adverse events (irAEs) associated with anti-programmed cell death protein 1/programmed cell death ligand 1 therapy, and are often clinically and histologically characterized as lichenoid. Nonlichenoid patterns may also occur and are likely to be encountered by surgical pathologists, given the increasing clinical use of these agents. The purpose of this study is to describe the histopathologic features of nonlichenoid cutaneous irAEs from patients receiving anti-programmed cell death protein 1/programmed cell death ligand 1 therapies for a variety of underlying advanced malignancies. Read More
Direct immunofluorescence (DIF) on frozen tissue (DIF-F) is the method of choice for the identification of immune deposits present in skin and other tissues. DIF can also be performed on formalin-fixed paraffin-embedded tissue (DIF-P) after antigen retrieval with proteases and has proven to be of value in renal pathology. However, its utility in skin biopsies has not been fully examined. Read More
Itch which is one of the major, subjective symptoms in a course of bullous pemphigoid and dermatitis herpetiformis makes those two diseases totally different than other autoimmune blistering diseases. Its pathogenesis is still not fully known. The aim of this research was to assess the role of IL-31 in development of itch as well as to measure its intensity. Read More
Background: Autoimmune bullous disorders (AIBDs) are a heterogeneous group of diseases which are rarely seen in children. Studies concerning the immunobullous diseases in pediatric patients are scarce.
Aims And Objectives: In this study, we aimed to investigate the clinical features and treatment outcomes of AIBDs in children. Read More
We thank Dr. Fung and colleagues for their interest in our publication concerning eruptive keratoacanthomas (KAs) and bullous pemphigoid-like disease in patients on anti-programmed cell death-1 (PD-1) immunotherapy.(1) We agree that nivolumab, like pembrolizumab, seems to share the same immune-related adverse event profile, and in fact our second case occurred in a patient on nivolumab (after no visible reduction in metastatic disease on pembrolizumab). Read More
Autoimmune subepidermal blistering diseases of the skin and mucosae constitute a large group of sometimes devastating diseases, encompassing bullous pemphigoid, gestational pemphigoid, mucous membrane pemphigoid, epidermolysis bullosa acquisita, and anti-p200 pemphigoid. Their clinical presentation is polymorphic. These autoimmune blistering diseases are associated with autoantibodies that target distinct components of the basement membrane zone of stratified epithelia. Read More
Bullous pemphigoid (BP) is the most common of pemphigoid diseases caused by autoantibodies against the structures of dermoepidermal junction followed by complement activation, innate immune cell infiltration, neutrophil proteinase secretion and subepidermal blister formation. The first line treatment of BP is topical and systemic glucocorticoids (GC). Regulation of the immune system and inflammatory cells is the main target of GC actions. Read More
Chronic, inflammatory and ulcerating mucocutaneous diseases that can affect the vulvar area are reviewed: lichen sclerosus, lichen planus, plasma cell vulvitis, complex aphthosis, Behcet's disease, pyoderma gangrenosum, metastatic Crohn's disease, dyskeratotic skin diseases (Hailey-Hailey disease and Darier's disease), autoimmune bullous diseases (mucous membrane pemphigoid and pemphigus vulgaris) and hidradenitis suppurativa. Also, vulvodynia and vestibulodynia, zinc and vitamin B deficiency are described. Read More
Background: Pemphigus is a group of autoimmune blistering diseases affecting skin and mucous membranes. While pemphigus is an autoantibody mediated disease, the role of T cells and cytokines in the pathogenesis is being increasingly recognized.
Objective: This study was conducted to observe alterations in the serum cytokine levels of patients with pemphigus vulgaris (PV), pemphigus foliaceous (PF), paraneoplastic pemphigus (PNP) and compare with bullous pemphigoid (BP) and healthy subjects. Read More
Background: Subepidermal autoimmune bullous diseases are a diverse group of diseases with overlapping clinical and immunopathological features. Indirect immunofluorescence microscopy on artificially split skin helps to classify these conditions into those with staining on the epidermal side of the split ("roof-binding") and those with staining on the dermal side ("floor-binding"). Epidermolysis bullosa acquisita is the prototype of "floor-binding" subepidermal autoimmune bullous diseases. Read More
Department of Dermatology, Kyoto University Graduate School of Medicine, Kyoto, Japan; Singapore Immunology Network (SIgN) and Institute of Medical Biology, Agency for Science, Technology and Research (A*STAR), Biopolis, Singapore.
Autoimmune bullous diseases are at the forefront of the research field on autoimmune diseases. Pemphigus and pemphigoid were historical entities in the world of descriptive dermatology for a long time. Recently, however, dermatologists and skin biologists have elegantly explained the novel pathomechanism of pemphigus and pemphigoid diseases. Read More
We herein firstly reported that a patient with thymoma-associated pemphigus (TAP) underwent a robotic-assisted trans-subxiphoid thoracoscopic extended thymectomy and then achieved stable resolution. The patient, a 47-year-old male, was first admitted to our hospital owing to stomatitis and bullae of the trunk after four months' prednisone treatment. On admission, chest computed tomography (CT) revealed an anterior-mediastinal mass and it was initially diagnosed as a thymoma. Read More
BPAG1, also known as Dystonin or BP230, belongs to the plakin family of proteins, which has multiple cytoskeleton-binding domains. Several BPAG1 isoforms are produced by a single BPAG1 genomic locus using different promoters and exons. For example, BPAG1a, BPAG1b, and BPAG1e are predominantly expressed in the nervous system, muscle, and skin, respectively. Read More
Background: It has been postulated that periodic acid-Schiff staining of basement membrane can predict direct immunofluorescence patterns seen in epidermolysis bullosa acquisita and bullous pemphigoid. It has also been suggested that the type of inflammatory infiltrate or presence of fraying of basal keratinocytes may differentiate these two conditions.
Objective: In this study, we aimed to confirm these observations. Read More
Autoimmune bullous diseases (ABDs) are uncommon but significant skin disorders with relatively high morbidity and mortality. Some surveys have been carried out to describe the spectrum of ABDs in a region, but this is the first that has focused on ABDs in elderly patients. This study was conducted to determine the clinicoepidemiologic features of ABDs in elderly patients. Read More
Background: Epidermolysis bullosa acquisita is a rare, potentially devastating autoimmune disease of the skin. IgG autoantibodies directed against type VII collagen (Col7), the major component of anchoring fibrils, induce skin fragility leading to cutaneous and mucocutaneous blister formation which is mostly of a scaring phenotype. Thus, powerful and reproducible diagnostic assays are critical to establish the diagnosis of EBA early to avoid irreversible sequelae. Read More
Bullous Pemphigoid (BP) is an inflammatory rare autoimmune bullous dermatosis, which outcome cannot be predicted through clinical investigations. Eosinophils are the main immune infiltrated cells in BP. However, the release of Major Basic Protein (MBP), Eosinophil Derived Neurotoxin (EDN), and Eosinophil Cationic Protein (ECP) upon eosinophil activation has still not been evaluated with respect to BP development. Read More
Studies suggest an association between neurological disorders and bullous pemphigoid. The goal of this systematic review was to characterize the occurrence of neurological disorders in patients with bullous pemphigoid. We performed a systematic review of the current English literature from 1984 to June 1(st), 2015 for documented cases of coexistent BP and neurological disorders. Read More
Recent studies postulated the association between bullous pemphigoid (BP) and neurodegenerative disorders (ND). The autoantibodies to BP180 and/or BP230 may be present not only in BP, but also in ND as neuronal isoforms of these proteins are identified in the central nervous system. However, there are only scant data about the precise pathogenetic mechanisms interlinking ND and BP as well as the immunologic profile in these patients. Read More
In elderly patients, bullous pemphigoid (BP) is associated with several comorbidities; the strongest association occurs between BP and neurological diseases. Different types of dementia, Parkinson's disease, cerebrovascular disorders and epilepsy all have a significant association with BP but patients with multiple sclerosis have the highest risk of BP. An existing neurological disorder appears to increase the risk for subsequent BP, but an increased risk for developing some neurological diseases has also been reported following BP diagnosis. Read More
Linear IgA bullous dermatosis (LABD) is an autoimmune disease characterized by blisters on the skin and mucous membranes. Polycyclic, grouped bullae on cutaneous skin are the most characteristic clinical feature of LABD. Lesions are often indistinguishable from bullous pemphigoid or may resemble dermatitis herpetiformis. Read More
Background: The inflammatory myopathies are a heterogeneous group of muscle diseases and comprise polymyositis, dermatomyositis (DM), myopathies associated with cancers, necrotising myositis and inclusion body myositis. DM occasionally exhibits few or no muscular signs: i.e. Read More
Bullous pemphigoid and pemphigus vulgaris, which belong to the group of subepidermal and intraepidermal bullae, respectively, are two potentially devastating blistering skin diseases. We used high-frequency ultrasound (US) in 3 cases of these diseases as prototypes to study the value of high-frequency US in discriminating blister locations. Our findings showed that high-frequency US has a strong correlation with histomorphometric findings because of its high resolution, and we hope that it will be helpful for differentiating blister locations. Read More
University François Rabelais, Boulevard Tonnelé, 37044 Tours Cedex 9, France, Department of Dermatology, Hospital of Tours, Avenue de la République, 37044 Tours Cedex 9, France, Laboratory "Biologie des Infections à Polyomavirus", ISP 1282, INRA-University François, Rabelais, Tours, France.
Iatrogenic Kaposi sarcoma (KS) has previously been reported in patients with bullous pemphigoid (BP), in relation to systemic steroids. To report three cases of previously unreported cutaneous KS during treatment with superpotent topical steroids (STS) and methotrexate (MTX). All patients were elderly men with BP treated with STS for 2 to 32 months (cumulative doses: 2,700-9,150 g) before MTX was introduced (dosage: 10-12. Read More