scholarly journals Pemphigus Vulgaris and Bullous Pemphigoid of the Upper Aerodigestive Tract: A Review Article and Novel Approaches to Management

ORL ◽  
2021 ◽  
pp. 1-9
Author(s):  
Mohammed Hassan Hussain ◽  
Faiz Tanweer ◽  
Georgios Sakagiannis ◽  
Manish Mair ◽  
Sara Mahmood ◽  
...  
Keyword(s):  
Bullous Pemphigoid ◽  
Patient Outcomes ◽  
Multimodality Treatment ◽  
Pemphigus Vulgaris ◽  
Review Article ◽  
Upper Aerodigestive Tract ◽  
Systemic Corticosteroids ◽  
Autoimmune Bullous Diseases ◽  
Bullous Diseases ◽  
Inflammatory Drugs

<b><i>Background:</i></b> Autoimmune bullous diseases are rare conditions characterized by blistering of the skin and mucous membranes. The 2 commonest forms are pemphigus vulgaris and bullous pemphigoid. The oral cavity or oropharynx may be the initial site of presentation or often the only site involved. <b><i>Summary:</i></b> These conditions are often misdiagnosed or overlooked leading to poorer patient outcomes. Due to the chronic nature of these conditions and the systemic effects of treatment, there is a significant associated morbidity and mortality. As such, an understanding of the fundamentals of autoimmune bullous diseases is vital to those working in otolaryngology. The mainstay of management in both conditions is topical and systemic corticosteroids. There is also a role for immunomodulating and non-steroidal anti-inflammatory drugs as adjunct or alternative therapies. Surgical intervention may be required to protect the airway. Often multimodality treatment is required involving multidisciplinary input from otolaryngologists, oral surgeons, dermatologists, and rheumatologists. This review article will highlight the aetiology, pathology, clinical features, investigations, and management of both pemphigus vulgaris and bullous pemphigoid including recent advances in management.

scholarly journals Pemphigus Vulgaris and Bullous Pemphigoid: Update on Diagnosis and Treatment

2020 ◽  
pp. e2020050 ◽  
Author(s):  
Vito Di Lernia ◽  
Dahiana M. Casanova ◽  
Mohamad Goldust ◽  
Cinzia Ricci
Keyword(s):  
Bullous Pemphigoid ◽  
Pemphigus Vulgaris ◽  
Screening Tools ◽  
Enzyme Linked Immunosorbent Assay ◽  
Pemphigus Foliaceus ◽  
Line Treatment ◽  
First Line ◽  
Systemic Corticosteroids ◽  
Autoimmune Bullous Diseases ◽  
First Line Treatment

Autoimmune bullous disorders are a heterogeneous spectrum of skin disorders characterized by the production of autoantibodies against adhesion molecules of the skin. The 2 major groups of diseases are “pemphigus diseases” and “autoimmune bullous diseases of the pemphigoid type.” Pemphigus diseases are a group of autoimmune blistering diseases of the skin and mucous membranes characterized by intraepithelial cleft and acantholysis. The main subtypes of pemphigus include pemphigus vulgaris, pemphigus foliaceus, and paraneoplastic pemphigus. Diagnosis is based on clinical manifestations and confirmed with histological, immunofluorescence, and serological testing. Recently multivariant enzyme‐linked immunosorbent assay systems have been developed as practical screening tools for patients with suspected autoimmune bullous dermatoses. The current first-line treatment of pemphigus is based on systemic corticosteroids that are often combined with immunosuppressive adjuvants, such as azathioprine, mycophenolate mofetil, and the anti-CD20 monoclonal antibody rituximab, usually at initiation of treatment. Rituximab efficacy is higher when it is administered early in the course of the disease. Therefore, it should be used as first-line treatment to improve efficacy and reduce cumulative doses of corticosteroids and their side effects. Treatment of bullous pemphigoid is based on disease extension. Localized and mild forms can be treated with superpotent topical corticosteroids or with nonimmunosuppressive agents. In patients with generalized disease or whose disease is resistant to the treatments described above, systemic corticosteroids are preferred and effective. Adjuvant immunosuppressants are often combined with steroids for their steroid-sparing effect.

scholarly journals Case Report: Variety of Target Antigens During 1 Year Follow-Up of a Patient Initially Diagnosed With Bullous Pemphigoid

2022 ◽  
Vol 12 ◽  
Author(s):  
Hua Qian ◽  
Zhijun Zhou ◽  
Luhuai Shi ◽  
Huicheng Li ◽  
Weijun Liu ◽  
...  
Keyword(s):  
Case Report ◽  
Bullous Pemphigoid ◽  
Pemphigus Vulgaris ◽  
Disease Course ◽  
Serum Samples ◽  
Dominant Type ◽  
Time Points ◽  
Autoimmune Bullous Diseases ◽  
Bullous Diseases

Autoimmune bullous diseases (AIBDs), presenting cutaneous and/or mucosal bullous lesions, are classified into pemphigus and pemphigoid diseases. A longtime observation for complicated AIBD cases is rarely reported. In this study, serum samples of one AIBD patient were collected at seven different time points during the disease course including a relapse, which were examined by our conventional and newly developed methods for the detection of autoantibodies. Interestingly, we found changes of both the presence and the titers of various autoantibodies in accordance with the changes of clinical features during the whole disease course, which indicated that the patient started as bullous pemphigoid and relapsed as concurrence of bullous pemphigoid and mucosal-dominant-type pemphigus vulgaris.

scholarly journals Biologics in autoimmune bullous diseases: Current scenario

2021 ◽  
Vol 0 ◽  
pp. 1-10
Author(s):  
Anuradha Bishnoi ◽  
Dipankar De ◽  
Sanjeev Handa ◽  
Rahul Mahajan
Keyword(s):  
Monoclonal Antibody ◽  
Mycophenolate Mofetil ◽  
Adverse Effects ◽  
Pemphigus Vulgaris ◽  
New Developments ◽  
Autoimmune Bullous Diseases ◽  
Current Scenario ◽  
Bullous Diseases ◽  
Anti Cd20

Autoimmune bullous diseases can be intraepidermal (pemphigus group of disorders) or subepidermal (pemphigoid group of disorders). The treatment of these disorders chiefly comprises corticosteroids and immunosuppressant adjuvants like azathioprine and mycophenolate mofetil. Autoantibodies are the main mediators of these diseases. Rituximab, a chimeric anti-CD20 monoclonal antibody targeting B-cells, has emerged as an excellent treatment option for refractory pemphigus vulgaris in the last decade. Since then, many new biologics have been proposed/explored for managing autoimmune bullous diseases. These hold potential for greater efficacy and lesser adverse effects than conventional immunosuppressants. In this review, we discuss the role of various biologics in the treatment of autoimmune bullous diseases, followed by a brief discussion on the drawbacks to their use and new developments in this area.

Coexistence of pemphigus vulgaris and bullous pemphigoid in the upper aerodigestive tract

2006 ◽  
Vol 33 (2) ◽  
pp. 231-233 ◽  
Author(s):  
Keiji Tabuchi ◽  
Masatake Nomura ◽  
Hidekazu Murashita ◽  
Yasuhiro Fujisawa ◽  
Shigeki Tsuji ◽  
...  
Keyword(s):  
Bullous Pemphigoid ◽  
Pemphigus Vulgaris ◽  
Aerodigestive Tract ◽  
Upper Aerodigestive Tract

scholarly journals Development of a Disease Registry for Autoimmune Bullous Diseases: Initial Analysis of the Pemphigus Vulgaris Subset

2015 ◽  
Vol 95 (1) ◽  
pp. 86-90 ◽  
Author(s):  
A Shah ◽  
K Seiffert-Sinha ◽  
D Sirois ◽  
V Werth ◽  
B Rengarajan ◽  
...  
Keyword(s):  
Pemphigus Vulgaris ◽  
Disease Registry ◽  
Initial Analysis ◽  
Autoimmune Bullous Diseases ◽  
Bullous Diseases

IgE-mediated mechanisms in bullous pemphigoid and other autoimmune bullous diseases

2015 ◽  
Vol 12 (3) ◽  
pp. 267-277 ◽  
Author(s):  
Nina van Beek ◽  
Franziska S. Schulze ◽  
Detlef Zillikens ◽  
Enno Schmidt
Keyword(s):  
Bullous Pemphigoid ◽  
Autoimmune Bullous Diseases ◽  
Bullous Diseases ◽  
Ige Mediated

An Unusual Case of Pemphigus Vulgaris

2022 ◽  
pp. 1-3
Author(s):  
Laura Marano ◽  
Gabriella Fabbrocini ◽  
Giuseppe Monfrecola ◽  
Fabrizio Martora
Keyword(s):  
Skin Disorder ◽  
Unusual Case ◽  
Pemphigus Vulgaris ◽  
Pemphigus Foliaceus ◽  
Case Presentation ◽  
Autoimmune Bullous Diseases ◽  
Atypical Manifestations ◽  
Life Threatening ◽  
Bullous Diseases ◽  
Careful History

<b><i>Introduction:</i></b> Pemphigus is a potential life-threatening skin disorder belonging to the group of the autoimmune bullous diseases affecting the skin and mucosa. The most common subtypes are pemphigus foliaceus (PF) and pemphigus vulgaris. <b><i>Case Presentation:</i></b> We present the case of a young woman with scalp manifestations diagnosed as seborrhiasis who came to our office where a more careful history and clinical examination directed us toward another diagnostic suspicion. The histological examination confirmed our suspicion of pemphigus and therefore we believe it is important to report our experience to avoid misdiagnosis. <b><i>Discussion/Conclusion:</i></b> Our case may be useful in the literature to identify cases of PF with atypical manifestations that may mimic other diseases.

scholarly journals New Bullous Eruptions in a COVID-19 Positive Patient in an Intensive Care Unit

2021 ◽  
Vol 5 (3) ◽  
pp. 278-282
Author(s):  
Angela Kim ◽  
Muneeb Khan ◽  
Ann Lin ◽  
Hongbei Wang ◽  
Louis Siegel ◽  
...  
Keyword(s):  
Older Adults ◽  
Intensive Care Unit ◽  
At Risk ◽  
Intensive Care ◽  
Bullous Pemphigoid ◽  
Positive Patient ◽  
Risk Population ◽  
Autoimmune Bullous Diseases ◽  
Bullous Diseases ◽  
New Onset

Bullous pemphigoid (BP) is an autoimmune blistering disorder that typically occurs in older adults. The etiopathogenesis of BP is unclear, although viral triggers have been reported. We present a case of new onset pauci-cellular BP in a patient admitted to Intensive Care Unit from an acute COVID-19 infection. Our case proposes to add the new coronavirus to the list of potential viral triggers of BP. Moreover, clinicians should be aware that the same at-risk population for autoimmune bullous diseases may also be at-risk for COVID-19 infection that is responsible for the current pandemic.

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