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 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.

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.

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 Pemphigus Vulgaris- A Case Report

2021 ◽  
pp. 324-328
Author(s):  
Mayur B. Wanjari ◽  
Deeplata Mendhe ◽  
Pratibha Wankhede ◽  
Sagar Alwadkar
Keyword(s):  
Case Report ◽  
Pemphigus Vulgaris ◽  
Entire Body ◽  
Case Presentation ◽  
Transmembrane Glycoprotein ◽  
Mouth Ulcer ◽  
Life Threatening ◽  
History Of ◽  
Epithelial Lesions ◽  
Intraepithelial Lesions

Introduction: Pemphigus is a rare and life-threatening autoimmune disease characterized by blisters and erosion of the skin and mucous membranes throughout the entire body. It mostly affects the mouth, eye, nose, throat as well as genitals. Pemphigus vulgaris is most common type of pemphigus. The epithelial lesions are caused by autoantibodies reacting with desmosomal glycoproteins found on the keratinocyte's cell surface. The binding of immunoglobulin G autoantibodies to desmoglein 3, a transmembrane glycoprotein adhesion molecule found on desmosomes, is the underlying process that causes intraepithelial lesions. Case Presentation: A 40-year-old male patient came to the hospital with a complaint of a mouth ulcer and a genital lesion that had been since 15 years. He is having a history of oral ulcer in oral mucosa and involvement of genital area in glans penis with a history of pain, bleeding, difficulty in swallowing.   Intervention: The patient was admitted to the hospital on 29/07/2021 and taken Inj. Rituximab 1gm in 500Ml Normal Saline in infusion pumps in over 6 Hours. Conclusion: In this case report, we mainly focus on expert dermatological management and excellent nursing care in managing the rare complicated case nicely. Early diagnosis and treatment of pemphigus Vulgaris help determine the course of the disease of the patient and is done by a dermatologist.

Autoimmunity and immunological tolerance in autoimmune bullous diseases

2019 ◽  
Vol 31 (7) ◽  
pp. 431-437 ◽  
Author(s):  
Hayato Takahashi ◽  
Hisato Iriki ◽  
Miho Mukai ◽  
Aki Kamata ◽  
Hisashi Nomura ◽  
...  
Keyword(s):  
Autoimmune Diseases ◽  
Immunological Tolerance ◽  
Tissue Destruction ◽  
Bullous Disease ◽  
Autoimmune Bullous Diseases ◽  
Life Threatening ◽  
Blistering Disease ◽  
Bullous Diseases ◽  
Insight Into

Abstract Autoimmune diseases are devastating conditions in which the immune system is directed against the host, leading to life-threatening destruction of organs. Although autoantigens are ill-defined in most autoimmune diseases, this is not the case in the skin. Autoimmune bullous diseases have been extensively studied with detailed characterization of autoantigens, the epitopes that are targeted, and the mechanisms of action that mediate autoimmune tissue destruction. Pemphigus is an autoimmune bullous disease caused by circulating IgG that targets two desmosomal proteins, desmoglein 1 and 3, which are crucial for cell–cell adhesion of keratinocytes. Binding of auto-antibodies to desmogleins impairs keratinocyte adhesion, leading to severe blistering disease. Mouse models that recapitulate the human disease have been instrumental in elucidating the detailed pathophysiology. Taking advantage of the fact that desmogleins are specifically targeted in pemphigus, studying humoral and cellular autoimmunity against these autoantigens provides us with an opportunity to understand not only the effector mechanisms of B and T cells in mediating pathology but also how autoreactive lymphocytes are regulated during development in the thymus and post-development in the periphery. This review introduces pemphigus and its subtypes as prototypic autoimmune diseases from which recent basic and translational developments should provide insight into how autoimmunity develops.

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 Detection of circulating anti-skin antibodies by indirect immunofluorescence and by ELISA: a comparative systematic review and meta-analysis

2020 ◽  
Vol 58 (10) ◽  
pp. 1623-1633
Author(s):  
Otto Van de gaer ◽  
Petra de Haes ◽  
Xavier Bossuyt
Keyword(s):  
Bullous Pemphigoid ◽  
Indirect Immunofluorescence ◽  
Meta Analysis ◽  
Random Effect ◽  
Random Effect Model ◽  
Pemphigus Vulgaris ◽  
Pemphigus Foliaceus ◽  
Bullous Pemphigoid Antigen ◽  
Autoimmune Bullous Diseases ◽  
Pubmed Database

AbstractBackgroundBoth enzyme-linked immunosorbent assays (ELISAs) and indirect immunofluorescence (IIF) are available for the diagnosis of autoimmune bullous diseases (AIBD). Many studies have reported on the performance of ELISAs and concluded that ELISAs could replace IIF. This study compares the diagnostic accuracy of ELISA and IIF for the detection of autoantibodies to desmoglein 1 (DSG1), desmoglein 3 (DSG3), bullous pemphigoid antigen 2 (BP180) and bullous pemphigoid antigen 1 (BP230) to support the diagnosis of pemphigus vulgaris (PV), pemphigus foliaceus (PF) and bullous pemphigoid (BP).MethodsA literature search was performed in the PubMed database. The meta-analysis was performed using summary values and a bivariate random effect model.ResultsThe five included studies on PV did not demonstrate significant differences between IIF and DSG3-ELISA (sensitivity 82.3% vs. 81.6%, p = 0.9284; specificity 95.6% vs. 93.9%, p = 0.5318; diagnostic odds ratio [DOR] 101.60 vs. 67.760, p = 0.6206). The three included studies on PF did not demonstrate significant differences between IIF and DSG1-ELISA (sensitivity 80.6% vs. 83.1%, p = 0.8501; specificity 97.5% vs. 93.9%, p = 0.3614; DOR 160.72 vs. 75.615, p = 0.5381). The eight included studies on BP showed that BP230-ELISA differed significantly from both IIF on monkey esophagus (MO) and BP180-ELISA with regard to DOR (11.384 vs. 68.349, p = 0.0008; 11.384 vs. 41.699, p = 0.0125, respectively)ConclusionsOur meta-analysis shows that ELISA performs as well as IIF for diagnosing PV, PF and BP.

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 Hemophagocytic lymphohistiocytosis and thrombotic microangiopathy after parvovirus B19 infection and renal transplantation: a case report

2021 ◽  
Vol 22 (1) ◽  
Author(s):  
C. J. Steffen ◽  
N. Koch ◽  
K. U. Eckardt ◽  
K. Amann ◽  
E. Seelow ◽  
...  
Keyword(s):  
Hemophagocytic Lymphohistiocytosis ◽  
Unusual Case ◽  
Kidney Biopsy ◽  
Viral Infections ◽  
Parvovirus B19 ◽  
Autoinflammatory Disorders ◽  
Case Presentation ◽  
Clinical Criteria ◽  
Life Threatening ◽  
Parvovirus B19 Infection

Abstract Background Hemophagocytic lymphohistiocytosis (HLH) is a rare and life-threatening disease characterized by hyperactivation of the immune system that causes hypercytokinemia and potentially multi organ failure. HLH can occur in patients with underlying rheumatic or autoinflammatory disorders. Additionally, HLH can develop in patients during infections or malignancies without a known genetic predisposition. Case presentation We herein report a patient, who presented with fever, both acute kidney and liver injury, anemia, thrombocytopenia and HSV stomatitis. HLH was diagnosed based on clinical criteria and qPCR revealed an acute parvovirus B19 infection as potential underlying infectious trigger. Treatment was started with both IVIG and dexamethasone. Subsequently, kidney biopsy demonstrated TMA. Conclusions In rare cases both HLH and aHUS can occur simultaneously in a patient as a consequence of viral infections. Insights from this unusual case might help physicians understand this complex symptom constellation.

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.

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