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Curr Protoc Pharmacol 2019 Feb 20:e55. Epub 2019 Feb 20.

Lübeck Institute of Experimental Dermatology, University of Lübeck, Lübeck, Germany.

Pemphigoid diseases (PDs) are a group of autoimmune bullous diseases characterized and caused by autoantibodies targeting structural proteins of the skin and mucous membranes. Chronic inflammation, subepidermal blistering, and often scaring are the clinical characteristics of PDs. Itching and, in severe cases, disabilities resulting from scaring (i. Read More

Arch Pathol Lab Med 2019 Feb 20. Epub 2019 Feb 20.

From the Department of Dermatology, Saint Louis University, St Louis, Missouri (Dr Abate);the Departments of Dermatology (Dr Battle),and Pathology (Drs Gardner and Shalin),University of Arkansas for Medical Sciences, Little Rock; and the Department of Dermatology, University of Mississippi Medical Center, Jackson (Dr Emerson).

Context.—: Fatal dermatologic diseases and ones with high morbidity can occur in the inpatient setting. In such cases, prompt and accurate assessment of a bedside skin biopsy is required. Read More

Adv Clin Exp Med 2019 Feb 18. Epub 2019 Feb 18.

Department of Dermatology and Immunodermatology, Medical University of Warsaw, Poland.

Background: Bullous pemphigoid (BP) is the most common autoimmune bullous disease associated with higher mortality and coexisting comorbidities. The strongest relationship has been reported with neurological diseases (NDs) but the particular type of ND differed depending on the study. There are some doubts on the prevalence of other comorbidities. Read More

Am J Case Rep 2019 Feb 18;20:212-218. Epub 2019 Feb 18.

Department of Internal Medicine, Mount Sinai Hospital, Chicago, IL, USA.

BACKGROUND The hallmark of bullous pemphigoid (BP) is widespread tense blisters arising on normal or erythematous skin, often with marked pruritus, the diagnosis of which is confirmed by direct immunofluorescence (DIF). BP is an autoimmune process that can be induced, though rarely, by medications. Drug-induced BP often has atypical clinical presentation, which requires a good understanding of other dermatological conditions with similar presentations, in particular, bullous subtype of erythema multiforme. Read More

J Am Acad Dermatol 2019 Feb 13. Epub 2019 Feb 13.

PEDEGO Research Unit, University of Oulu; Department of Dermatology and Medical Research Center Oulu, Oulu University Hospital, Oulu, Finland.

Curr Opin Pharmacol 2019 Feb 12;46:34-43. Epub 2019 Feb 12.

Department of Dermatology, Philipps University, Baldingerstr., Marburg, Germany. Electronic address:

Pemphigoid is the most common autoimmune blistering disease. IgG and IgE autoantibodies against the hemidesmosomal antigens Bullous Pemphigoid (BP) 180 and BP230 are of pathogenic relevance, since autoantibody-antigen binding results in complement activation, immune cells infiltration, impaired hemidesmosomal function, and loss of dermal-epidermal adhesion. Systemic steroids and immunosuppressants are frontline therapies in pemphigoid, but result in substantial morbidity and increased mortality. Read More

Medicine (Baltimore) 2019 Feb;98(7):e14514

Dermatology Unit, Department of Medicine, University of Padua, Via Vincenzo Gallucci 4, Padua, Italy.

Bullous pemphigoid is an autoimmune subepidermal blistering skin disease immunologically defined by autoantibodies directed against basement membrane zone antigens, the main of which is BP180. Laboratory tests are essential for diagnosis and include direct immunofluorescence and serologic assessments with indirect immunofluorescence and ELISA. Serology may be performed on blister fluid, in alternative to blood serum. Read More

Pediatr Dermatol 2019 Feb 14. Epub 2019 Feb 14.

Division of Dermatology, CHU Sainte-Justine, University of Montreal, Montreal, Quebec, Canada.

Localized vulvar bullous pemphigoid of childhood is an excessively rare variant of bullous pemphigoid and affects almost exclusively young girls of 7-12 years of age. In contrast to adult-onset bullous pemphigoid, a prompt response to potent topical corticosteroids is observed in the majority of cases, with a favorable prognosis and rare relapses. We report the case of a 7-year-old girl who presented with this condition. Read More

Front Med (Lausanne) 2019 29;6. Epub 2019 Jan 29.

Department of Dermatology, Venerology and Allergology, Charité- Universitätsmedizin Berlin, Berlin, Germany.

Mucous membrane pemphigoid (MMP) is a rare autoimmune bullous disease of the mucous membranes, which can cause irreversible scarring and is discussed to be associated with cancer, if laminin-332-autoantibodies are present. MMP with severe ocular and laryngeal involvement is difficult to treat and can be treatment-refractory to conventional immunosuppressant therapy. A 67-year-old man with a history of prostate cancer presented to our clinic with sore throat, intraoral bullae, odynophagia, dysphonia, exertional dyspnea, and erosions of the glans penis. Read More

Nihon Ronen Igakkai Zasshi 2019 ;56(1):43-50

Department of Diabetes, Metabolism, and Endocrinology, Tokyo Metropolitan Geriatric Hospital.

Aim: Bullous pemphigoid (BP) is an autoimmune skin disorder characterized by the production of autoantibodies. Several recent reports have described the occurrence of BP in diabetic patients treated with dipeptidyl peptidase-4 (DPP-4) inhibitors. However, the clinical features of BP in diabetic patients, particularly in those treated with DPP-4 inhibitors, have not yet been examined. Read More

J Dermatol 2019 Feb 13. Epub 2019 Feb 13.

Department of Dermatology, Kyoto University Graduate School of Medicine, Kyoto, Japan.

Eur J Case Rep Intern Med 2018 21;5(3):000724. Epub 2018 Mar 21.

Serviço de Anatomia Patológica, Centro Hospitalar Vila Nova de Gaia/Espinho, Portugal.

Little has been documented about hypersensitivity reactions caused by treatment with rivaroxaban. This paper reports a bullous pemphigoid-like skin eruption that occurred in a 76-year-old female patient during rivaroxaban treatment. This case highlights the vigilance required by healthcare workers in recognising potential adverse effects of newly marketed drugs and in making medication changes when necessary. Read More

Front Immunol 2019 24;10:34. Epub 2019 Jan 24.

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

Mucous membrane pemphigoid (MMP) is a mucous membrane-dominated autoimmune subepithelial blistering disease that is caused by autoantibodies against various autoantigens in basement membrane zone (BMZ) proteins, including collagen XVII (COL17). Clinicians face diagnostic problems in detecting circulating antibodies and targeted antigens in MMP. The diagnostic difficulties are mainly attributed to the low titers of MMP autoantibodies in sera and to heterogeneous autoantigens. Read More

J Am Acad Dermatol 2019 Feb 6. Epub 2019 Feb 6.

Division of Dermatology, Lahey Hospital and Medical Center, 67 S Bedford Rd, Burlington, MA 01803.

J Am Acad Dermatol 2019 Feb 6. Epub 2019 Feb 6.

PEDEGO Research Unit, University of Oulu; Department of Dermatology and Medical Research Center Oulu, Oulu University Hospital, Oulu, Finland. Electronic address:

Eur J Dermatol 2019 Feb 8. Epub 2019 Feb 8.

Department of Dermatology and National Reference Center for Bullous Diseases -MALIBUL, 2 Avenue Martin Luther King, Limoges, 87 000, France.

J Eur Acad Dermatol Venereol 2019 Jan 31. Epub 2019 Jan 31.

Department of Dermatology, Hospital NECKER ENFANTS-MALADES, Institut Imagine, centre de référence MAGEC, University Paris Descartes- Sorbonne Paris Cité, Paris, France.

Background: Autoimmune bullous dermatosis (AIBDs) in children are uncommon and their long-term evolution remains unknown.

Objective: The aim of this retrospective study was to characterize the long-term prognosis of AIBDs that started during childhood.

Methods: We conducted a monocentric retrospective study, in the French dermatology center, by including all children affected by AIBDs. Read More

J Drugs Dermatol 2019 Jan;18(1):103-104

Novel immunotherapies including antibodies to programmed death ligand 1 (PD-1) and cytotoxic T-lymphocyte-associated antigen 4 (CTLA-4) have become common therapies for neoplasms including metastatic melanoma and non-small cell lung cancer (NSCLC). Dermatologic toxicity is the most common adverse event associated with these immunotherapies. We report a case of bullous pemphogoid (BP) in a patient receiving combination durvalumab and tremelimumab, two newer immunotherapy checkpoint inhibitors under investigation in phase III trials. Read More

Exp Ther Med 2019 Feb 5;17(2):1132-1138. Epub 2018 Dec 5.

Department of Dermatology, 'Iuliu Hațieganu' University of Medicine and Pharmacy, 400006 Cluj-Napoca, Romania.

Pemphigoid nodularis (PN) is a rare clinical variant of bullous pemphigoid characterized by the presence of nodular prurigo-like lesions and pemphigoid blisters. The diagnosis is confirmed by direct immunofluorescence (DIF)/ indirect immunofluorescence (IIF) and immunoserology tests. For some patients, with long mean duration of symptoms, the correct diagnosis of PN is delayed because the disease is not recognized. Read More

JAMA Otolaryngol Head Neck Surg 2019 Jan 24. Epub 2019 Jan 24.

Division of Otolaryngology-Head & Neck Surgery,Department of Surgery, University of California, San Diego.

Br J Dermatol 2019 Jan 23. Epub 2019 Jan 23.

Department of Dermatology, Hospital Clinic de Barcelona, Barcelona, Spain.

Dipeptidyl peptidase-4 inhibitor-associated bullous pemphigoid (DPP4i-BP) has been linked to a characteristic non-inflammatory BP phenotype and to the presence of autoantibodies directed against the midportion of BP180 ectodomain. However, DPP4i-BP cases with clinical and immunological findings of classical inflammatory BP have also been reported. In the September issue of the BJD, Mai et al reported three cases of DPP4i-BP. Read More

BMC Dermatol 2019 Jan 23;19(1). Epub 2019 Jan 23.

Dermatology Department, College of Medicine, King Saud University, Riyadh, Saudi Arabia.

Background: Mucous membrane pemphigoid (MMP) is an autoimmune blistering disease that is notoriously difficult to treat. Nail involvement in MMP is rare.

Case Presentation: We report on a 58 years old man with severe MMP who presented with onychomadesis. Read More

J Eur Acad Dermatol Venereol 2019 Jan 21. Epub 2019 Jan 21.

Department of Medicine, Division of Experimental Allergology and Immunodermatology, University of Oldenburg, Germany.

Background: Bullous pemphigoid (BP) is the most common subepidermal autoimmune blistering disease with an increased incidence particularly amongst the elderly. Several studies have recently reported an association between BP and neurological disorders.

Objective: To evaluate the association between BP and neurological disorders in a single center in Germany. Read More

Dermatol Ther 2019 Jan 18:e12829. Epub 2019 Jan 18.

Dermatology and Venereology Department, Hospital Universitario 12 de Octubre, Universidad Complutense de Madrid, Madrid, Spain.

Bullous pemphigoid (BP) is an acquired autoimmune bullous disease characterized by autoantibodies against the hemidesmosomal proteins found in the basal keratinocytes of the basement membrane zone (BMZ): a 180 kDa protein (type XVII collagen) mainly and the 230 kDa antigen. There is such evidence that the antibodies against the BMZ components are not only of IgG type, but also this bullous disease may have IgE antibodies directed to the BMZ that contribute to the pathogenesis of the disorder. IgE is not only thought to contribute to the pathogenesis of BP, it has also been suggested that eosinophils play a role in the development of the first signs associated with BP. Read More

Dermatol Ther 2019 Jan 19:e12833. Epub 2019 Jan 19.

Department of Dermatology, Beijing Children's Hospital, Capital Medical University, Beijing, China.

Pityriasis lichenoides et varioliformis acuta (PLEVA) pemphigoides is an uncommon skin disease, which is characterized by the appearance of blistering skin lesions in patients with PLEVA. We present a 3-year-old boy, who was diagnosed with PLEVA pemphigoides. Combined treatment of oral methotrexate and corticosteroids was more effective than corticosteroids alone on this patient. Read More

Clin Exp Dermatol 2019 Jan 16. Epub 2019 Jan 16.

Department of Dermatology and Immunodermatology, Medical University of Warsaw, Warsaw, Poland.

Background: Bullous pemphigoid (BP) is a distressing autoimmune bullous disease strongly associated with severe pruritus; however, data concerning pruritus in BP are still scarce. No clinical research evaluating the effect of BP on sleep quality has been conducted.

Aim: To evaluate the intensity of pruritus measured by nocturnal wrist movements (NWMs) and the sleep quality in patients with BP using actigraphy in comparison with nonpruritic healthy controls (HCs) with subsequent correlations with an itch visual analogue scale (VAS) as a subjective measure, disease severity [Bullous Pemphigoid Disease Area Index (BPDAI), urticaria/erythema, erosions/blisters] and serum total IgE level. Read More

BMC Ophthalmol 2019 Jan 16;19(1):19. Epub 2019 Jan 16.

Department of Ophthalmology, Toho University Ohashi Medical Center, 2-22-36, Ohashi, Meguro-ku, Tokyo, 153-8515, Japan.

Background: To report the first case of human herpesvirus-6 (HHV-6) corneal endotheliitis that developed after intravitreal ranibizumab injections.

Case Presentation: A 63-year-old man with a medical history of diabetes and systemic steroid treatment for bullous pemphigoid had been receiving intravitreal injections of ranibizumab in the left eye for 2 years according to a Pro Re Nata treatment regimen for macular edema associated with branch retinal vein occlusion. Twenty days after the last injection, the patient presented with pain and decreased visual acuity in his left eye. Read More

Am J Clin Dermatol 2019 Jan 16. Epub 2019 Jan 16.

Graduate Institute of Biomedical Informatics, College of Medicine Science and Technology, Taipei Medical University, 250 Wu-Hsing St., Taipei, 110, Taiwan.

Background: Dementia is a syndrome that involves the deterioration of several higher mental functions in advanced age, and psoriasis is an autoimmune disease characterized by skin plaque. Epidemiological studies have indicated an association between dementia and psoriasis; however, to date, no studies in Asia have reported this association.

Objective: This study used a population-based medical dataset to explore the association between previously diagnosed psoriasis and dementia in Taiwan. Read More

Endocrinol Diabetes Nutr 2019 Jan 7. Epub 2019 Jan 7.

Servicio de Endocrinología y Nutrición, Hospital General Universitario Morales Meseguer, Murcia, España.

J Dermatol 2019 Jan 10. Epub 2019 Jan 10.

Department of Dermatology, The University of Tokyo Graduate School of Medicine, Tokyo, Japan.

JAMA Dermatol 2019 Jan 9. Epub 2019 Jan 9.

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

Importance: A substantial number of patients with bullous pemphigoid do not develop skin blisters and may not have received the correct diagnosis. Diagnostic criteria and an optimal diagnostic strategy are needed for early recognition and trials.

Objectives: To assess the minimal requirements for diagnosis of bullous and nonbullous forms of pemphigoid and to evaluate the optimal diagnostic strategy. Read More

JAMA Dermatol 2019 Feb;155(2):166-171

Department of Dermatology, Rambam Health Care Campus, Haifa, Israel.

Importance: The prevalence of mucosal involvement in bullous pemphigoid (BP) is inconsistent. Nonoral mucosal involvement was reported anecdotally in few patients with BP.

Objective: To evaluate the prevalence of mucosal involvement in patients with BP, and to characterize the subgroup of patients with mucosal lesions. Read More

JAMA Dermatol 2019 Feb;155(2):172-177

Department of Dermatology, Bundang CHA Medical Center, CHA University School of Medicine, Seongnam, Republic of Korea.

Importance: Recent studies suggest that dipeptidyl peptidase 4 (DPP-4) inhibitors are associated with an increased risk of developing bullous pemphigoid (BP). Population-based studies on the association between DPP-4 inhibitors and BP are limited.

Objective: To characterize the potential association between the use of DPP-4 inhibitors and an increased risk of developing BP. Read More

Int J Med Inform 2019 Feb 28;122:27-36. Epub 2018 Nov 28.

Bernoulli Institute for Mathematics, Computer Science, University of Groningen, The Netherlands.

Direct immunofluorescence (DIF) microscopy of a skin biopsy is used by physicians and pathologists to diagnose autoimmune bullous dermatoses (AIBD). This technique is the reference standard for diagnosis of AIBD, which is used worldwide in medical laboratories. For diagnosis of subepidermal AIBD (sAIBD), two different types of serrated pattern of immunodepositions can be recognized from DIF images, namely n- and u-serrated patterns. Read More

J Invest Dermatol 2018 Oct 26. Epub 2018 Oct 26.

PEDEGO Research Unit, University of Oulu, Oulu, Finland; Department of Dermatology and Medical Research Center Oulu, Oulu University Hospital, Oulu, Finland. Electronic address:

Bullous pemphigoid (BP) and dermatitis herpetiformis (DH) are autoimmune bullous skin diseases. DH has been described to evolve into BP and the two diseases can have overlapping clinical appearances and diagnostic findings, but the association between DH and BP has not previously been studied in a large population. To evaluate DH and celiac disease as risk factors for BP, we conducted a retrospective case-control study of patients with BP and matched controls with basal cell carcinoma diagnosed in Finland between 1997 and 2013. Read More

Australas J Dermatol 2018 Dec 27. Epub 2018 Dec 27.

Histology and Cytopathology Laboratory, Angers University Hospital, Angers Cedex 09, France.

J Dermatol 2019 Feb 26;46(2):124-130. Epub 2018 Dec 26.

Department of Dermatology, Keio University School of Medicine, Tokyo, Japan.

This was a multicenter study of rituximab, a chimeric monoclonal immunoglobulin G antibody directed against CD20, for the treatment of refractory autoimmune bullous diseases (pemphigus and pemphigoid). Ten patients (three with pemphigus vulgaris, six with pemphigus foliaceus and one with bullous pemphigoid) were treated with a single cycle of rituximab (four weekly infusions at a dose of 375 mg/m of body surface area). The primary end-points were the number of serious adverse events and rate of complete remission at 40 weeks. Read More

Pediatr Dermatol 2018 Dec 19. Epub 2018 Dec 19.

Department of Dermatology, University of Pennsylvania, Philadelphia, Pennsylvania.

Bullous pemphigoid (BP) is the most common autoimmune blistering disease affecting the elderly but is quite rare in childhood. The majority of pediatric cases have been reported during early childhood. Adolescence is divided into three phases: early (10-13 years), middle (14-17), and late (18-21). Read More

Seizure 2019 Jan 5;64:59-64. Epub 2018 Dec 5.

Stichting Epilepsie Instellingen Nederland (SEIN), Achterweg 5, Heemstede, 2103SW, Netherlands; NIHR University College London Hospitals Biomedical Research Centre, UCL Queen Square Institute of Neurology, London, WC1N 3BG, United Kingdom; Chalfont Centre for Epilepsy, Chalfont St Peter, Bucks, SL9 0RJ, United Kingdom.

The risk of epileptic seizures seems increased in several systemic autoimmune disorders including systemic lupus erythematosus, type 1 diabetes mellitus, myasthenia gravis, celiac disease, rheumatoid arthritis, Hashimoto's encephalopathy, psoriasis, multiple sclerosis, neuromyelitis optica, and bullous pemphigoid. Immune dysfunction may be partly responsible for this association. Elevated levels of pro-inflammatory cytokines, autoantibodies seen in these autoimmune disorders and antibodies against neuronal antigens may contribute to the etiopathogenesis of seizures and epilepsy associated to immune conditions. Read More

Expert Opin Biol Ther 2019 Feb 18;19(2):149-156. Epub 2018 Dec 18.

a Unit of Dermatology , University of Padua , Padua , Italy.

Introduction: Autoimmune blistering skin diseases are a group of disorders subdivided according to the location of blister formation: intraepidermal blistering in the pemphigus group and subepidermal in the pemphigoid group. These conditions are clinically heterogeneous and are treated with systemic corticosteroids and/or other forms of immunosuppression on the basis of clinical subtype and disease severity. These approaches may not be effective for the induction and maintenance of clinical response or need to be stopped because of intolerable side effects. Read More

Clin Immunol 2019 Jan 14;198:54-56. Epub 2018 Dec 14.

Department of Clinical Immunology, Royal Perth Hospital, Perth, WA 6000, Australia; Institute of Immunology and Infectious Diseases (IIID), Murdoch University, Murdoch, WA 6150, Australia. Electronic address:

Bullous pemphigoid (BP) is a blistering dermopathy and a prototypic antibody-mediated autoimmune disease. Detection of IgG autoantibodies against hemidesmosomal proteins BP180 and/or BP230 are diagnostic and levels can correlate with disease activity. Therapies include corticosteroids and oral immunosuppressants, while intravenous immunoglobulin and rituximab are reserved for treatment resistant cases. Read More

J Invest Dermatol 2018 Dec 10. Epub 2018 Dec 10.

Department of Dermatology, Rouen University Hospital and INSERM U1234, Centre de référence des maladies bulleuses autoimmunes, Normandie University, Rouen.

Dipeptidyl-peptidase-IV-inhibitors have been suspected to induce bullous pemphigoid (BP). The objective of this study was to compare the observed frequency of gliptin intake in a large sample of 1787 BP patients diagnosed between 2012 and 2015 in France, with the expected frequency after indirect age standardization on 225412 individuals extracted from the database of the National Healthcare Insurance Agency. The secondary objective was to assess the clinical characteristics and the course of gliptin-associated BP depending on whether gliptin was continued or stopped. Read More

Front Immunol 2018 20;9:2687. Epub 2018 Nov 20.

Department of Dermatology, Medical Faculty, Medical Center - University of Freiburg, Freiburg, Germany.

Bullous pemphigoid is a subepidermal blistering skin disease, associated with autoantibodies to hemidesmosomal proteins, complement activation at the dermal-epidermal junction, and dermal granulocyte infiltration. Clinical and experimental laboratory findings support conflicting hypotheses regarding the role of complement activation for the skin blistering induced by pemphigoid autoantibodies. In-depth studies on the pathogenic relevance of autoimmune complement activation in patients are largely lacking. Read More

Exp Dermatol 2018 Dec 11. Epub 2018 Dec 11.

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

Exosomes are a type of endosome that can mediate communication between cells and the transport of cellular components such as microRNAs, mRNAs, proteins and DNA. Recently, exosomes have been under investigation for their significant roles in both healthy physiology and disease states. Herein, we review the role of exosomes in chronic inflammatory skin diseases and skin tumors, especially focusing on systemic lupus erythematosus, psoriasis, atopic dermatitis, bullous pemphigoid and melanoma. Read More

J Dermatol 2018 Dec 7. Epub 2018 Dec 7.

Department of Dermatology, Graduate School of Medicine, Mie University, Tsu, Japan.

Eur J Dermatol 2018 Dec 10. Epub 2018 Dec 10.

Division of Dermatology, Department of Medicine of Sensory and Motor Organs, Faculty of Medicine, Tottori University, Yonago, Japan.

J Am Acad Dermatol 2018 Dec 3. Epub 2018 Dec 3.

Loyola Univ Medical Center Dermatology, 321 N La Grange Rd, La, Grange Park, IL. Electronic address:

The incidence of zoster is increased in many autoimmune diseases. This study found a significantly increased zoster incidence in dermatomyositis and cutaneous lupus erythematosus compared with a control group.Physicians should be aware of this risk and consider administering the vaccine against zoster to these patients if not contraindicated. Read More

Hautarzt 2019 Jan;70(1):51-63

Klinik für Dermatologie, Allergologie und Venerologie, Universität zu Lübeck, Ratzeburger Allee 160, 23538, Lübeck, Deutschland.

Autoimmmune bullous diseases are mediated by pathogenetically relevant autoantibodies against components of the epidermis and/or superficial mucous membranes (in pemphigus) and structural proteins of the dermal-epidermal junction (in pemphigoid diseases). Using immunoadsorption (IA), an already well-established procedure in cardiac and rheumatic disorders, antibodies can be removed from the plasma. At present, most data on the adjuvant use of IA in dermatology are derived from patients with severe and/or refractory pemphigus vulgaris or pemphigus foliaceus and also from patients with pemphigoid diseases. Read More

Int J Dermatol 2019 Mar 5;58(3):e66-e67. Epub 2018 Dec 5.

Division of Dermatology, Niigata University Graduate School of Medical and Dental Sciences, Niigata, Japan.