Br J Dermatol 2017 Jul;177(1):13-14

Department of Dermatology, University of Pennsylvania, 421 Curie Blvd., 1009 BRB, Philadelphia, PA, 19104, U.S.A.

J Cutan Med Surg 2017 Jul 1:1203475417722734. Epub 2017 Jul 1.

1 Departments of Dermatology and Skin Science, University of British Columbia, Vancouver, BC, Canada.

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

J Am Acad Dermatol 2017 Aug;77(2):370-372

Department of Dermatology, Rambam Health Care Campus, Haifa, Israel; Rappaport Faculty of Medicine, Technion-Israel Institute of Technology, Haifa, Israel.

Skinmed 2017 1;15(3):175-180. Epub 2017 Jun 1.

Department of Dermatology, Autoimmune Bullous Diseases Research Center, Tehran University of Medical Sciences, Tehran, Iran.

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

Br J Dermatol 2017 Jul 13. Epub 2017 Jul 13.

Department of Dermatology and Allergology, Philipps-University, Marburg, Germany.

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

Eur J Dermatol 2017 Jul 11. Epub 2017 Jul 11.

Department of Dermatology, Hokkaido University Graduate School of Medicine, Sapporo, Japan.

J Dermatol 2017 Jul 7. Epub 2017 Jul 7.

Department of Dermatology, Hokkaido University Graduate School of Medicine, Sapporo, Japan.

Sci Rep 2017 Jul 6;7(1):4833. Epub 2017 Jul 6.

Laboratory of Dermatology, Faculty of Medicine of Reims, EA 7319, IFR 53, University of Champagne-Ardenne, Reims, France.

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

Eur J Dermatol 2017 Jul 5. Epub 2017 Jul 5.

Department of Internal Medicine, Division of Dermatology, The Ohio State University Wexner Medical Center, Gahana, Ohio, USA.

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

Cent Eur J Immunol 2017 8;42(1):85-90. Epub 2017 May 8.

Autoimmune Blistering Dermatoses Section, Department of Dermatology, Poznan University of Medical Sciences, Poznan, Poland.

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

J Dermatol 2017 Jul 5. Epub 2017 Jul 5.

Department of Dermatology, Kyushu University Graduate School of Medicine, Fukuoka, Japan.

Exp Dermatol 2017 Jul 4. Epub 2017 Jul 4.

PEDEGO Research Unit, University of Oulu; Department of Dermatology and Medical Research Center Oulu, Oulu University Hospital, P.O. Box 20, 90029, Oulu, Finland.

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

Proc (Bayl Univ Med Cent) 2017 Jul;30(3):360-361

Texas A&M Health Science Center College of Medicine (Shah, Parekh) and Department of Dermatology, Scott & White Memorial Hospital (Shah, Mohr, Parekh), Temple, Texas.

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

Ann Dermatol Venereol 2017 Jun 28. Epub 2017 Jun 28.

Service de dermatologie, hôpital Saint-Vincent-de-Paul, université catholique de Lille, boulevard de Belfort, BP 387, 59020 Lille, France.

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

J Ultrasound Med 2017 Jun 29. Epub 2017 Jun 29.

Shandong Provincial Hospital for Skin Diseases, Shandong University, Jinan, China.

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

Eur J Dermatol 2017 Jun 28. Epub 2017 Jun 28.

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

Am J Dermatopathol 2017 Jun 20. Epub 2017 Jun 20.

*Department of Pathology, East Tennessee State University, Quillen College of Medicine, Johnson City, TN; †James H. Quillen College of Medicine, East Tennessee State University, Johnson City, TN; and ‡Department of Medicine, East Tennessee State University, Quillen College of Medicine, Johnson City, TN.

Direct immunofluorescence (DIF) using frozen section material from a fresh/preserved perilesional biopsy is the gold standard for the immunopathologic diagnosis of bullous pemphigoid (BP). DIF in BP shows linear dermoepidermal junction (DEJ) staining for C3, with or without staining for IgG. In some situations, only a formalin-fixed lesional biopsy is obtained (with no fresh/preserved perilesional biopsy for DIF). Read More

J Am Acad Dermatol 2017 Jun 20. Epub 2017 Jun 20.

Department of Dermatology, Rabin Medical Center, Beilinson Hospital, Petach Tikva, Israel; Sackler Faculty of Medicine, Tel Aviv University, Tel Aviv, Israel.

Background: Studies evaluating whether malignancy rate is increased in patients with bullous pemphigoid (BP) have reached conflicting results.

Objective: We sought to determine whether BP is associated with malignancy.

Method: Medline, EMBASE, the Cochrane library, and reference lists of included studies were searched for comparative studies that evaluated the relationship between BP and malignancy. Read More

Int Sch Res Notices 2017 24;2017:6053267. Epub 2017 May 24.

Molecular Dermatology Research Center, Shiraz University of Medical Sciences, Shiraz, Iran.

Background: Bullous pemphigoid (BP) is an autoimmune subepidermal blistering skin disorder which occurs mostly in the elderly. Several studies have reported an association between BP and neurological disorders (ND).

Objective: The purpose of this study was to evaluate the association between BP and neurological disorders in Iranian patients. Read More

Semin Cell Dev Biol 2017 Jun 13. Epub 2017 Jun 13.

Laboratory for Bone Metabolism, Key Laboratory for Space Bioscience and Biotechnology, School of Life Sciences, Northwestern Polytechnical University, Xi'an, Shaanxi 710072, PR China; Shenzhen Research Institution of Northwestern Polytechnical University, Shenzhen, 518057, PR China; Northwestern Polytechnical University-Hong Kong Baptist University Joint Research Centre for Translational Medicine on Musculoskeletal Health in Space, Xi'an, 710072, PR China. Electronic address:

Spectraplakins are multifunctional cytoskeletal linker proteins that act as important communicators, connecting cytoskeletal components with each other and to cellular junctions. Bullous pemphigoid antigen 1 (BPAG1)/dystonin is a member of spectraplakin family and expressed in various tissues. Alternative splicing of BPAG1 gene produces various isoforms with unique structure and domains. Read More

J Eur Acad Dermatol Venereol 2017 Jun 16. Epub 2017 Jun 16.

Department of Dermatology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing, 100730, China.

Turk J Med Sci 2017 Jun 12;47(3):832-840. Epub 2017 Jun 12.

Department of Dermatology, Faculty of Medicine, Dokuz Eylül University, İzmir, Turkey.

Background/aim: The distribution and the demographic, etiological, and clinical features of autoimmune bullous diseases (ABDs) vary according to geographic regions of the world. The limited number of reported studies in Turkey are mostly retrospective and generally include only pemphigus. The aim of our study was to evaluate the demographic data, clinical features, associated triggering factors, and comorbidities in patients newly diagnosed with ABDs. Read More

Mediators Inflamm 2017 10;2017:8980534. Epub 2017 May 10.

Department of Immunopathology, Chair of Allergy, Immunology and Dermatology, Medical University of Lodz, Lodz, Poland.

Dermatitis herpetiformis (DH), bullous pemphigoid (BP), and pemphigus vulgaris (PV) are autoimmune bullous skin conditions with eosinophilic and neutrophilic infiltrations. While cytokines are crucial for the affinity and activation of different leukocyte cells in the inflammation and blister formation, there are no studies concerning a role of IL-36. The goal of the study was to analyze whether interleukin 36 is involved in pathogenesis of DH, BP, and PV. Read More

J Eur Acad Dermatol Venereol 2017 Jun 12. Epub 2017 Jun 12.

Department of Immunology, Hospital Clínico Universitario Virgen de la Arrixaca, Murcia, Spain.

Bullous pemphigoid (BP) is a disease caused by antibodies against components of the basement membrane, mainly two proteins: BP180 and BP230. We present two cases of BP related to chronic renal rejection. This article is protected by copyright. Read More

J Assoc Physicians India 2017 May;65(5):98-100

Bombay Hospital Institute of Medical Sciences, Mumbai, Maharashtra.

Bullous pemphigoid (BP) is an autoimmune blistering disorder of the skin first described in 1953. A decade later, antibodies were described targeting the cutaneous basement membrane zone. The association of Bullous pemphigoid with malignancy is debatable1 but reported in many case reports. Read More

BMJ 2017 Jun 8;357:j2169. Epub 2017 Jun 8.

St Helens and Knowsley Teaching Hospitals NHS Trust, St Helens WA9 3DA, UK

Clin Exp Dermatol 2017 Aug 7;42(6):632-637. Epub 2017 Jun 7.

Department of Dermatology, Ruhr-University Bochum, Bochum, Germany.

Background: Bullous pemphigoid (BP) is the most common autoimmune blistering disease, and is associated with autoantibodies to the hemidesmosomal BP autoantigens BPAG1 and BPAG2.

Aim: We aimed to investigate the significance of T regulatory cells and other lymphocyte subsets in patients with BP.

Methods: In total, 31 inpatients with BP were treated with systemic prednisolone in a tapered dose regimen, while 28 healthy individuals matched for age and sex served as the healthy control (HC) group. Read More

Indian J Dermatol 2017 May-Jun;62(3):262-267

Department of Dermatology, University of California Davis, Sacramento, CA, USA.

Pemphigus and pemphigoid are the prototypical immunobullous diseases. Although it has been well established that they are caused by deposition of autoreactive antibodies directed against adherence proteins within the skin, the specific genetic and environmental factors leading to development of these diseases continue to be an area of investigation. Herein, we discuss several of the potential environmental triggers that may induce patients to develop immunobullous diseases including medications, viral infections, UV exposure or other radiation injury and dietary factors. Read More

Indian J Dermatol 2017 May-Jun;62(3):237-250

Department of Dermatology, KPC Medical College and Hospital, Kolkata, West Bengal, India.

Topical corticosteroids (TCs) are the pillars of dermatotherapeutics. These drugs are the "magic molecules," provided they are used judiciously and appropriately, following a rational prescription. On exhaustive literature search in multiple databases, we found a significant evidence favoring the use of TCs in atopic eczema, localized vitiligo, psoriasis, chronic hand eczema, and localized bullous pemphigoid. Read More

Indian J Dermatol Venereol Leprol 2017 Jul-Aug;83(4):457-461

National Skin Centre; Department of Dermatology, Lee Kong Chian School of Medicine; Department of Dermatology, Tan Tock Seng Hospital, Singapore.

Background: Bullous pemphigoid is the most common subepidermal immunobullous disorder. Studies have reported the association between bullous pemphigoid and various neurological diseases.

Aims: The aim of this study was to evaluate whether bullous pemphigoid is associated with pre-existent neurological diseases and whether specific diseases exhibit this association. Read More

Br J Dermatol 2017 Jun;176(6):1428-1429

Department of Dermatology, St George Hospital, University of New South Wales, Sydney, NSW, Australia.

J Cutan Med Surg 2017 Jun 1:1203475417712247. Epub 2017 Jun 1.

1 Department of Dermatology, Saint-Luc Hospital, Montreal University, Montréal, QC, Canada.

Background: Bullous pemphigoid (BP) is an autoimmune bullous disease requiring immunosuppressive therapy. Kaposi sarcoma (KS) is an angioproliferative tumor associated with the opportunistic viral infection human herpes virus 8 (HHV-8). It is a well-known condition associated with longstanding human immunodeficiency virus infection, but it may also occur in the context of iatrogenic immunosuppression. Read More

J Invest Dermatol 2017 May 30. Epub 2017 May 30.

Department of Physiology, Semmelweis University School of Medicine, 1094 Budapest, Hungary; MTA-SE "Lendület" Inflammation Physiology Research Group of the Hungarian Academy of Sciences and Semmelweis University, 1094 Budapest, Hungary. Electronic address:

The inflammatory form of epidermolysis bullosa acquisita is caused by autoantibodies against type VII collagen (C7), a component of the dermal-epidermal junction. We have previously shown that myeloid Src-family kinases mediate skin inflammation triggered by anti-C7 antibodies. Here we identify the Syk tyrosine kinase as a critical component of autoantibody-induced skin inflammation downstream of Src-family kinases. Read More

Med Hypotheses 2017 Jun 24;103:86-89. Epub 2017 Apr 24.

Department of Dermatology, Kaohsiung Chang Gung Memorial Hospital and Chang Gung University College of Medicine, Kaohsiung, Taiwan. Electronic address:

The number of elderly patients with chronic pruritus has been gradually increasing in aging countries. Bullous pemphigoid, a common autoimmune blister disease in the elderly, is always heralded by pruritic eczematous dermatitis and is often associated with neurodegenerative diseases. We hypothesized that chronic eczematous dermatitis in patients with neurodegenerative diseases may be an early marker of bullous pemphigoid. Read More

Case Rep Dermatol Med 2017 16;2017:6134752. Epub 2017 Apr 16.

Department of Dermatology and Venereology, Akdeniz University School of Medicine, Antalya, Turkey.

Background. Bullous pemphigoid is an autoimmune subepidermal blistering skin disease in which autoantibodies are directed against components of the basement membrane. The disease primarily affects the elderly people and in most of the patients inducing factors cannot be identified. Read More

Acta Med Port 2017 Mar 31;30(3):243-246. Epub 2017 Mar 31.

Serviço de Medicina Interna. Unidade de Doenças Autoimunes. Centro Hospitalar de Trás-os-Montes e Alto Douro. Vila Real. Portugal.

Bullous pemphigoid is an autoimmune disease characterized by subepidermal blister formation. Can be self-limiting but when necessary treatment includes the use of corticosteroids, immunosuppressants, and biological agents. We present the case of a 37 year old patient with a severe and refractory presentation of bullous pemphigoid. Read More

J Dermatol 2017 May 23. Epub 2017 May 23.

Department of Dermatology, National Taiwan University Hospital, Taipei, Taiwan.

Clin Exp Dermatol 2017 Jul 22;42(5):576-578. Epub 2017 May 22.

Department of Dermatology, Nagoya University, Nagoya, Aichi, Japan.

Am J Clin Dermatol 2017 May 22. Epub 2017 May 22.

Department of Dermatology, Northwestern University Feinberg School of Medicine, Suite 1600, 676 N. St. Clair St., Chicago, IL, 60611, USA.

Background: Psoriasis, atopic dermatitis or eczema (AD-E), pemphigus, bullous pemphigoid (BP), and hidradenitis are chronic inflammatory skin disorders associated with systemic immune activation, considerable symptom burden, stigma, functional disturbances, and mental health symptoms. All of these might increase cardiovascular risk.

Objective: The objective of this study was to determine whether these inflammatory skin diseases are associated with increased cardiovascular/cerebrovascular risk and/or disease. Read More

Pan Afr Med J 2017 13;26:136. Epub 2017 Mar 13.

Université de Sousse, Tunisie.

Cicatricial Pemphigoid is a subepithelial bullous dermatosis which essentially involves the mucous membranes with cicatricial evolution We report the case of a 66-year old patient hospitalized with erosive gingivitis associated with dysphagia, dyspnea and blurred vision. Dermatologic examination showed erosive lesions involving the palate and the pharynx. Ophthalmologic examination showed symblepharons, ectropion and bilateral cataract. Read More

Harefuah 2016 Oct;155(10):593-595

Department of Dermatology, Ha'Emek Medical Center, Afula, Israel.

Introduction: Bullous pemphigoid is the most common autoimmune bullous disease, treated with low dosage local or systemic corticosteroids. This is a case report of a 65 years-old female patient with a month long history of a bullous disease before admission to the dermatology department with a generalized bullous rash. Clinical examination and histopathology revealed bullous pemphigoid. Read More

J Diabetes Investig 2017 May 18. Epub 2017 May 18.

Department of Diabetes, Endocrinology and Nutrition, Kyoto University Graduate School of Medicine, Kyoto, Japan.

Bullous pemphigoid (BP) is an autoimmune blistering skin disorder. Recently, BP induced by dipeptidyl peptidase-4 (DPP-4) inhibitors has been a concern. Although DPP-4 inhibitors are commonly used in the Asian population because of their safety and efficacy, BP associated with DPP-4 inhibitors is sometimes seen in clinical settings. Read More

CEN Case Rep 2017 May 4;6(1):50-54. Epub 2016 Nov 4.

Division of Nephrology, Department of Medicine, School of Medicine, Showa University, Tokyo, Japan.

Bullous pemphigoid (BP) is a common autoimmune blistering disease that can be complicated by autoimmune disorders. We describe a patient with BP who developed membranous glomerulonephropathy (MN). Proteinuria decreased during the clinical course as anti-BP180 antibody titers decreased. Read More

Clin Exp Dermatol 2017 Jul 15;42(5):516-519. Epub 2017 May 15.

Center for Blistering Diseases, Department of Dermatology, Tufts University School of Medicine, Boston, MA, USA.

A 41-year-old white man was treated for bullous pemphigoid (BP) for 4 years, using high-dose prednisone as well as ciclosporin and mycophenolate mofetil. Sustained clinical improvement was not observed. He suffered several serious side effects. Read More

Exp Dermatol 2017 May 13. Epub 2017 May 13.

Department of Dermatology, University of Lübeck, Germany.

Experimental models of bullous pemphigoid (BP), the most frequent subepidermal autoimmune bullous disease, revealed that the immune response leading to blister formation represents an incompletely understood complex process involving different inflammatory cells. In contrast to previous reports commonly focusing on limited molecular and cellular phenotypes of the disease, the aim of this study was to investigate a broad spectrum of markers of cellular immune activation in patients with BP. We found that serum levels of soluble CD4, myeloperoxidase, S100A12, eosinophil cationic protein, and soluble P-selectin were significantly elevated in patients with active BP compared with normal controls. Read More

Front Immunol 2017 27;8:484. Epub 2017 Apr 27.

Department of Pathology, Johns Hopkins University School of Medicine, Baltimore, MD, USA.

Eosinophils are multifunctional granulocytes that contribute to initiation and modulation of inflammation. Their role in asthma and parasitic infections has long been recognized. Growing evidence now reveals a role for eosinophils in autoimmune diseases. Read More

Br J Dermatol 2017 May 11. Epub 2017 May 11.

Department of Dermatology, University of Lübeck, Lübeck, Germany.

Background: Current treatment of bullous pemphigoid (BP) is based on the long-term use of topical and/or systemic corticosteroids associated with a high rate of adverse events and increased mortality.

Objective: To study the corticosteroid-sparing potential of azathioprine and dapsone.

Method: A prospective, multicenter, randomized, non-blinded clinical trial that compares efficacy and safety of two parallel groups of BP patients treated with oral methylprednisolone 0. Read More

Asia Pac Allergy 2017 Apr 26;7(2):97-101. Epub 2017 Apr 26.

Department of Internal Medicine, Seoul National University College of Medicine, Seoul 03080, Korea.

Adverse reactions of subcutaneous low molecular weight heparin or unfractionated heparin could be complications by bleeding, heparin-induced thrombocytopenia, drug-induced liver injury, osteoporosis, and cutaneous reactions. Heparin-induced skin lesions vary from allergic reactions like erythema, urticaria, eczema to intradermal microvascular thrombosis associated with heparin-induced thrombocytopenia. There is a rare cutaneous complication, called bullous hemorrhagic dermatosis. Read More

J Eur Acad Dermatol Venereol 2017 May 9. Epub 2017 May 9.

Department of Dermatology, Singapore General Hospital, Singapore, Singapore.

Background: Bullous pemphigoid (BP) is the most common autoimmune blistering disease in the elderly and is associated with increased mortality. The extent of multimorbidity in patients with BP and its impact on survival are unclear.

Objectives: To describe the extent and spectrum of multimorbidity in patients with BP and to ascertain its impact on survival. Read More

Surg Pathol Clin 2017 Jun 30;10(2):505-512. Epub 2017 Mar 30.

Department of Dermatology, Center for Blistering Diseases, University Medical Center Groningen, University of Groningen, Groningen, The Netherlands.

Autoimmmune bullous diseases of skin and mucosa are uncommon, disabling, and potentially lethal diseases. For a quick and reliable diagnosis immunofluorescence is essential. This article describes two variants of immunofluorescence. Read More