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JAMA Dermatol 2022 May 25. Epub 2022 May 25.

Harvard Medical School, Boston, Massachusetts.

Importance: There is limited information on immune checkpoint inhibitor-induced bullous pemphigoid (ICI-BP) in patients with cancer, with most existing studies being case reports or small case series from a single institution. Prior review attempts have not approached the literature in a systematic manner and have focused only on ICI-BP secondary to anti-programmed cell death 1 (PD-1) or programmed cell death ligand 1 (PD-L1) therapy. The current knowledge base of all aspects of ICI-BP is limited. Read More

J Eur Acad Dermatol Venereol 2022 May 25. Epub 2022 May 25.

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

Background: The association of autoimmune bullous diseases (AIBDs) with thyroid disorders remains to be profoundly investigated.

Objective: To evaluate the epidemiological association between six AIBDs and thyroid disorders.

Methods: A population-based cross-sectional study enrolled patients with bullous pemphigoid (BP), mucous membrane pemphigoid (MMP), epidermolysis bullosa acquisita (EBA), pemphigoid gestationis (PG), pemphigus vulgaris (PV), and pemphigus foliaceus (PF). Read More

CMAJ 2022 May;194(20):E705

Departments of General Internal Medicine (Kano) and Dermatology (Kato), Tokyo Metropolitan Tama Medical Center, Fuchu, Tokyo, Japan.

Dermatol Ther 2022 May 24:e15595. Epub 2022 May 24.

Department of Dermatology, First Hospital of Shanxi Medical University, Taiyuan, People's Republic of China.

J Invest Dermatol 2022 May 18. Epub 2022 May 18.

Department of Dermatology, Johns Hopkins University School of Medicine, Baltimore, MD; Department of Oncology, Sidney Kimmel Comprehensive Cancer Center at Johns Hopkins University, Baltimore, MD. Electronic address:

Cutaneous immune-related adverse events (cirAEs) are the most prevalent complication to arise from immunotherapy and cause significant morbidity. We aimed to determine the spectrum, timing, clinical features, and outcomes of cirAEs by conducting an observational pharmacovigilance study using VigiBase, the World Health Organization's global database of individual case safety reports from over 130 member countries (ClinicalTrials.gov, number NCT04898751). Read More

Curr Drug Saf 2022 May 18. Epub 2022 May 18.

Department of Pharmacy Practice College of Pharmacy, Sri Ramakrishna Institute of Paramedical Sciences Coimbatore, Tamil Nadu, India.

Introduction: Bullous pemphigoid is the most common chronic recurrent autoimmune subepidermal blistering disorder most prevalent in geriatric population. It varies widely in clinical presentation ranging from tense bullae to intense generalized pruritus. It is immunologic in origin with the presence of IgG antibodies. Read More

Front Immunol 2022 3;13:887279. Epub 2022 May 3.

Department of Experimental and Clinical Medicine, University of Florence, Florence, Italy.

Bullous pemphigoid (BP) represents the most common autoimmune bullous disease and is characterized by IgG autoantibodies targeting collagen XVII (BP180). BP has reportedly been occurred in association with other inflammatory skin diseases. Here, we describe the unusual occurrence of BP in a female patient with a concomitant history of generalized morphea (localized scleroderma, LoS) and cutaneous and genital lichen sclerosus (LiS). Read More

J Adv Res 2022 Apr 4. Epub 2022 Apr 4.

Max Planck Institute for Evolutionary Biology, August-Thienemann-Str. 2, 24306 Plön, Germany; Section of Evolutionary Medicine, Institute for Experimental Medicine, Kiel University, Arnold-Heller-Str. 3, 24105 Kiel, Germany. Electronic address:

Introduction: Bullous pemphigoid (BP) is the most common autoimmune blistering disease. It predominately afflicts the elderly and is significantly associated with increased mortality. The observation of age-dependent changes in the skin microbiota as well as its involvement in other inflammatory skin disorders suggests that skin microbiota may play a role in the emergence of BP blistering. Read More

Am J Clin Dermatol 2022 May 17. Epub 2022 May 17.

Department of Dermatology, National Taiwan University Hospital and National Taiwan University College of Medicine, 15F, No. 7, Chung-Shan South Road, Taipei, Taiwan.

Background: Rituximab is a potential initial adjuvant therapy for bullous pemphigoid, yet clinical experience is scarce.

Objective: We aimed to examine the clinical outcomes and safety of initial combination therapy with systemic corticosteroids and adjuvant rituximab for the treatment of bullous pemphigoid.

Methods: A retrospective cohort study was performed on 84 patients with bullous pemphigoid, who received systemic corticosteroids with or without initial adjuvant rituximab therapy (defined as rituximab use within 12 weeks after initiation of systemic corticosteroids). Read More

Cureus 2022 Apr 13;14(4):e24126. Epub 2022 Apr 13.

Dermatology, Rutgers Robert Wood Johnson Medical School, New Brunswick, USA.

As the onset of novel variants of the severe acute respiratory syndrome coronavirus 2 virus pushes policy-makers to push widespread vaccination efforts, it is likely that an increased number of severe cutaneous adverse reactions (SCARs) will present. Therefore, it is important to understand the presentation of possible SCARs. However, data are limited regarding which SCARs are most likely to be found following vaccination, and specific presentations in certain demographic groups, such as postmenopausal women, remain widely unknown. Read More

JAAD Case Rep 2022 Jun 3;24:29-31. Epub 2022 Feb 3.

University of Texas Southwestern Medical Center, Dallas, Texas.

J Dermatol 2022 May 10. Epub 2022 May 10.

Department of Dermatology, Saitama Medical Center, Saitama Medical University, Saitama, Japan.

We report a patient with bullous pemphigoid (BP) who was successfully treated with dupilumab monotherapy. To clarify the underlying mechanism of this effective treatment, we investigated the dynamics of a variety of cytokine-producing T cells before and after treatment in the circulation and in blister fluid using flow cytometry. The patient was a 72-year-old woman who had a pruritic eruption consisting of erythema and tense blisters on the whole body. Read More

Br J Dermatol 2022 May 9. Epub 2022 May 9.

Emory Department of Dermatology, Atlanta, Georgia, 30322, USA.

Proc (Bayl Univ Med Cent) 2022 4;35(3):352-353. Epub 2022 Feb 4.

Department of Dermatology, Texas Tech University Health Sciences Center, Lubbock, Texas.

Lichen planus pemphigoides (LPP) is a rare autoimmune subepidermal blistering disease characterized by lichenoid and bullous lesions. LPP is generally thought to be idiopathic, possibly related to medication or malignancy, or potentially the result of long-standing lichenoid inflammation damaging the basement membrane zone leading to epitope spreading. The histological appearance of lichenoid and bullous lesions in LPP resembles findings of lichen planus and bullous pemphigoid, respectively. Read More

Front Immunol 2022 20;13:874108. Epub 2022 Apr 20.

Department of Dermatology and Referral Center for Autoimmune Bullous Diseases (MALIBUL), Avicenne Hospital, Hôpitaux Universitaires de Paris Seine-Saint-Denis, AP-HP, Université Sorbonne Paris Nord, Bobigny, France.

The role of IgE autoantibodies has been demonstrated in the pathogenesis of bullous pemphigoid for many years. Recently, omalizumab (OMZ), a humanized monoclonal anti-IgE antibody that depletes total serum IgE, has been used off-label in a few case series of bullous pemphigoids demonstrating a rapid efficacy and allowing significant improvements or complete remission as add-on therapy in first-line treatment-resistant patients. Herein, we report the largest retrospective study to evaluate OMZ effectiveness in patients with subepidermal autoimmune blistering diseases. Read More

Eur J Dermatol 2022 02;32(1):3-18

University of Genova, DiSSal Section of Dermatology, San Martino Polyclinic Hospital IRCCS, Largo R. Benzi 10 16132 Genova, Italy.

Eur J Dermatol 2022 02;32(1):146-166

Department of Dermatology, Hospital General Universitario Gregorio Marañón, Madrid, Spain.

J Med Case Rep 2022 May 5;16(1):184. Epub 2022 May 5.

Northwest Cancer Centre, Tamworth Hospital, Dean Street, Tamworth, NSW, 2340, Australia.

Background: Bullous pemphigoid is an uncommon dermatologic manifestation seen in squamous cell lung cancer, and evidence guiding optimal treatment, especially in the elderly population, is limited. We report herein a case of squamous cell lung cancer diagnosed after being investigated for refractory bullous pemphigoid showing marked response to carboplatin-based chemotherapy. This is the first case report that shows carboplatin can be used as an effective alternative in treatment of malignancy-associated bullous pemphigoid. Read More

Dermatol Ther 2022 May 5:e15555. Epub 2022 May 5.

Dermatology Unit, IRCCS Azienda Ospedaliera-Universitaria di Bologna, Bologna, Italy.

Clin Cosmet Investig Dermatol 2022 27;15:783-790. Epub 2022 Apr 27.

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

Purpose: Mucous membrane pemphigoid (MMP) is a very rare autoimmune bullous disease, affecting predominantly the mucosae and characterized by autoantibodies to the epithelial basement membrane components. Laminin 332 (Ln-332) is one of the most probable antigens with association with malignancy. The laboratory diagnosis of Ln-332-mediated autoimmunity is troublesome. Read More

JAMA Dermatol 2022 May 4. Epub 2022 May 4.

Department of Dermatology, Center of Blistering Diseases, European Reference Network-Skin, University Medical Center Groningen, University of Groningen, Groningen, the Netherlands.

Importance: Bullous pemphigoid is a difficult-to-treat autoimmune blistering skin disease that predominantly affects older adults and is associated with an increased mortality rate.

Objective: To examine the safety and therapeutic potential of nomacopan, an inhibitor of leukotriene B4 and complement C5, in patients with bullous pemphigoid.

Design, Setting, And Participants: This multicenter, single-group, phase 2a nonrandomized controlled trial was conducted in the dermatology departments of universities in the Netherlands and Germany. Read More

Australas J Dermatol 2022 May 2. Epub 2022 May 2.

Department of Dermatology-Venereology, Mohammed V Military Teaching Hospital, Mohammed V University in Rabat, Rabat, Morocco.

Background: Bullous pemphigoid is a common autoimmune blistering skin disease that is significantly associated with a high rate of morbidity and mortality.

Objective: Our aim was to determine the 1-, 2- and 3-year mortality rate and standardized mortality ratio of BP in Moroccan patients, as well as to identify risk factors that influence survival both in the 1st and 3rd year of follow-up.

Methods: All patients with BP diagnosed between January 2008 and December 2017 in a tertiary referral centre at the Mohammed V Military Hospital in Morocco were included retrospectively. Read More

SAGE Open Med Case Rep 2022 21;10:2050313X221093128. Epub 2022 Apr 21.

Division of Dermatology, Hôpital du Sacré-Cœur de Montréal, Université de Montréal, Montreal, QC, Canada.

Bullous pemphigoid is an autoimmune blistering disease that primarily affects the geriatric population. It often presents as urticarial erythematous plaques, which evolve into subepidermal blisters accompanied by pruritus. Although rare, clinical variants of bullous pemphigoid have been documented. Read More

Front Cell Infect Microbiol 2022 14;12:780354. Epub 2022 Apr 14.

Academic Unit of Ophthalmology, Institute of Inflammation and Ageing, University of Birmingham, Birmingham, United Kingdom.

Mucous Membrane Pemphigoid is an orphan multi-system autoimmune scarring disease involving mucosal sites, including the ocular surface (OcMMP) and gut. Loss of tolerance to epithelial basement membrane proteins and generation of autoreactive T cell and/or autoantibodies are central to the disease process. The gut microbiome plays a critical role in the development of the immune system. Read More

Front Immunol 2022 12;13:885023. Epub 2022 Apr 12.

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

Pemphigoid nodularis is a rare form of pemphigoid that joins the clinical picture of prurigo nodularis and the immunological features of bullous pemphigoid, which is therapeutically challenging. Here, we analyze five female patients with a long-lasting course of nodular pemphigoid in terms of clinical and immunological characteristics and therapy. All the patients fulfilled clinical and immunological criteria of nodular pemphigoid. Read More

J Dermatol 2022 May 2. Epub 2022 May 2.

Department of Public Health, Institute of Public Health, National Yang Ming Chiao Tung University, Taipei, Taiwan.

Bullous pemphigoid (BP) has been reported to be associated with an increased risk of venous thromboembolism (VTE). However, the exact time course is unclear, and no previous studies have been reported in the Asian population. This nationwide population-based cohort study examined the risk of VTE among BP patients in Taiwan between 2007 and 2018. Read More

Orv Hetil 2022 May 1;163(18):720-725. Epub 2022 May 1.

1 Semmelweis Egyetem, Általános Orvostudományi Kar, Szemészeti Klinika Budapest, Mária u. 39., 1085 Magyarország.

Összefoglaló. A nyálkahártya-pemphigoid különleges formája a paraneoplasiás pemphigoid, amely primer malignus betegséghez társul; lefolyása szokatlanul gyors, és a klasszikus immunszuppresszív terápiákkal szemben rezisztens lehet. Közleményünkben három eseten keresztül mutatjuk be a paraneoplasiás pemphigoid megjelenését, diagnosztikáját és a terápiás kihívásokat. Read More

Semin Arthritis Rheum 2022 Apr 15;55:152010. Epub 2022 Apr 15.

Harvard Medical School, Massachusetts General Hospital, Boston, MA, United States.

Glucocorticoids (GCs) have been the cornerstone of treating dozens of inflammatory conditions for more than seven decades. GC toxicity is ubiquitous in both clinical trials and clinical practice, and toxicities associated with GC use are central to the experience of most patients being treated for immune-mediated conditions. These conditions span the full range of medical specialties, including rheumatology, nephrology, gastroenterology, neurology, pulmonology, ophthalmology, and others. Read More

J Am Acad Dermatol 2022 Apr 25. Epub 2022 Apr 25.

Dermatology Department, AP-HP, Cochin Hospital, Paris, France; Competence Center for Autoimmune Blistering Diseases, MALIBUL, AP-HP, Henri Mondor Hospital, Creteil; Laboratoire de Biologie Cutanée, Institut Cochin, Inserm U1016, Université de Paris, France.

Background: Bullous pemphigoid (BP) is the most common autoimmune blistering disorder. Its presentation is polymorphic.

Objective: To investigate different clinical and biological profiles of BP. Read More

Dermatitis 2022 May-Jun 01;33(3):179-180. Epub 2022 Apr 28.

From the Department of Dermatology and Cutaneous Biology, Sidney Kimmel Medical College of Thomas Jefferson University, Philadelphia, PA.