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

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.

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 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 Therapeutic Apheresis in Dermatological Diseases

2015 ◽  
Vol 7 ◽  
pp. CMT.S14112
Author(s):  
Rolf Bambauer ◽  
Daniel Burgard ◽  
Ralf Schiel
Keyword(s):  
Porphyria Cutanea Tarda ◽  
Pemphigus Vulgaris ◽  
High Morbidity ◽  
Drug Induced ◽  
Autoimmune Bullous Diseases ◽  
Herpes Gestationis ◽  
Bullous Diseases ◽  
Schonlein Purpura ◽  
Support Treatment ◽  
Autoimmune Blistering Diseases

Since the mid-1970s, membrane modules became available and plasma separation techniques have gained importance. Therapeutic apheresis (TA) has been successfully introduced in a variety of autoantibody-mediated diseases. In dermatology, TA is increasingly applied as a support treatment for severe and/or refractory autoimmune bullous diseases. All autoimmune disease are characterized by autoantibodies mediated against structural proteins of the skin and/or mucous membranes. Autoimmune blistering diseases have a high morbidity and mortality. These diseases include pemphigus vulgaris and bullous pemphigoid among others. Drug-induced pemphigus and other forms, such as dermatitis herpetiformis, herpes gestationis, scleroderma, pyoderma gangrenosum, epidermal necrolysis, Behcet disease, psoriasis vulgaris, Henoch-Schönlein purpura, and porphyria cutanea tarda, are also mentioned. Pathogenetical aspects of autoantibodies in these diseases are clearly demonstrated. TA has been shown to effectively remove the autoantibodies from blood and lead to rapid clinical improvement.

scholarly journals Simultaneous Oral and Umbilical Locations as a First Sign of Pemphigus Vulgaris

2021 ◽  
Vol 2021 ◽  
pp. 1-7
Author(s):  
Eya Moussaoui ◽  
Yassine Oueslati ◽  
Lamia Oualha ◽  
Mohamed Denguezli ◽  
Badreddine Sriha ◽  
...  
Keyword(s):  
Early Diagnosis ◽  
Early Treatment ◽  
Clinical Manifestations ◽  
Pemphigus Vulgaris ◽  
Skin Lesions ◽  
Oral Lesions ◽  
Cutaneous Lesions ◽  
Autoimmune Bullous Diseases ◽  
Bullous Diseases ◽  
Pemphigus Vegetans

The place of pemphigus vulgaris (PV) among autoimmune bullous diseases (AIBD) is well established. It is an acquired chronic, autoimmune, vesiculobullous disease in which IgG antibodies target desmosomal proteins to produce intraepithelial mucocutaneous blistering. The diagnosis is often challenging for the clinicians. It requires a combination of three major features: clinical, histopathological, and immunological. Clinically, oral lesions are the first manifestations of the disease in 50-90% of the patients with widespread blisters affecting the oral mucosa. On the skin, lesions are characterized by flaccid blisters that rapidly progress into erosions and crust formation. Umbilical lesions as a clinical manifestation of PV are peculiar and have rarely been reported, and they are not yet completely elucidated. Umbilical region involvement in patients with pemphigus was assessed in a limited study totalling just 10 patients. This localisation may be a valuable hint easing the diagnosis at the clinical level for patients with oral mucosal blisters. Dentists must be familiar with the clinical manifestations of PV to make an early diagnosis and start an early treatment which determines the prognosis of the disease. To the best of the authors’ knowledge, the coexistence of these lesions with the oral lesions as a first sign of PV in the absence of skin involvement was reported in only one case of pemphigus vegetans (PVe). In this paper, we describe an observation of a female patient that was diagnosed with PV that begun with simultaneous oral and umbilical locations which coexisted for a period of 4 months before the appearance of other cutaneous lesions. We highlight the role of dentists, by being familiar with the clinical manifestations of PV, to make an early diagnosis to start an early treatment which determines the prognosis of the disease and to follow closely the evolution of lesions to change treatment if required. We also discuss the clinical, histological, and immunological features of the disease that enabled the differential diagnosis as well as the appropriate therapeutic management.

scholarly journals Deep pemphigus (pemphigus vulgaris, pemphigus vegetans and paraneoplastic pemphigus) in dogs, cats and horses: a comprehensive review

2020 ◽  
Vol 16 (1) ◽  
Author(s):  
Heng L. Tham ◽  
Keith E. Linder ◽  
Thierry Olivry
Keyword(s):  
Clinical Signs ◽  
Pemphigus Vulgaris ◽  
High Dose ◽  
Paraneoplastic Pemphigus ◽  
Autoimmune Bullous Diseases ◽  
Human Counterpart ◽  
Deep Layers ◽  
Bullous Diseases ◽  
Pemphigus Vegetans ◽  
Autoimmune Dermatoses

AbstractPemphigus is the term used to describe a group of rare mucocutaneous autoimmune bullous diseases characterized by flaccid blisters and erosions of the mucous membranes and/or skin. When the autoantibodies target desmosomes in the deep layers of the epidermis, deep pemphigus variants such as pemphigus vulgaris, pemphigus vegetans and paraneoplastic pemphigus develop. In this article, we will review the signalment, clinical signs, histopathology and treatment outcome of pemphigus vulgaris, pemphigus vegetans and paraneoplastic pemphigus in dogs, cats and horses; where pertinent, we compare the animal diseases to their human homologue. Canine, feline and equine pemphigus vulgaris, pemphigus vegetans and paraneoplastic pemphigus have many features similar to the human counterpart. These chronic and often relapsing autoimmune dermatoses require aggressive immunosuppressive therapy. In animals, the partial-to-complete remission of pemphigus vulgaris and pemphigus vegetans has been achieved with high dose glucocorticoid therapy, with or without adjunct immunosuppressants; the prognosis is grave for paraneoplastic pemphigus.

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.

Autoimmune bullous diseases with skin and eye involvement: Cicatricial pemphigoid, pemphigus vulgaris, and pemphigus paraneoplastica

2016 ◽  
Vol 34 (2) ◽  
pp. 205-213 ◽  
Author(s):  
Karen C. Broussard ◽  
Theresa G. Leung ◽  
Ahmadreza Moradi ◽  
Jennifer E. Thorne ◽  
Jo-David Fine
Keyword(s):  
Pemphigus Vulgaris ◽  
Cicatricial Pemphigoid ◽  
Autoimmune Bullous Diseases ◽  
Eye Involvement ◽  
Bullous Diseases

scholarly journals Cutaneous Type Pemphigus Vulgaris of the Scalp: A Rare Unilesional Presentation

2021 ◽  
Vol 5 (2) ◽  
pp. 170-173
Author(s):  
Stefanie Altmann ◽  
Adam Chahine ◽  
Jarett Casale ◽  
Jessica Forbes ◽  
Sarah Ferrer-Bruker
Keyword(s):  
Rare Variant ◽  
Unusual Case ◽  
Pemphigus Vulgaris ◽  
Cutaneous Involvement ◽  
Autoimmune Bullous Diseases ◽  
Mucosal Involvement ◽  
Mucous Membranes ◽  
Bullous Diseases ◽  
Almost All ◽  
Rare Group

Pemphigus Vulgaris (PV) is the most common subtype of pemphigus, a rare group of autoimmune bullous diseases affecting the skin and mucous membranes. PV can be further subdivided into mucocutaneous and mucosal dominant types, depending on the extent of cutaneous involvement. Almost all cases of PV have mucosal involvement; however, a rare variant of cutaneous-only PV has been reported in the literature. To our knowledge, only two previous accounts of unilesional scalp PV have been reported. We present an unusual case of cutaneous-only PV involving the scalp.

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