[Pemphigus vulgaris. A case report] (original) (raw)
Related papers
Pemphigus vulgaris—a case report and detailed review of literature
Indian Journal of Dentistry, 2011
Pemphigus vulgaris is a common oral mucosal disease characterized by the occurrence of chronic multiple ulcerative lesions. Although exact etiology is not known, the underlying mechanism responsible for causing intra-epithelial lesion of pemphigus vulgaris is binding of IgG autoantibodies to desmoglein 3, a transmembrane glycoprotein adhesion molecule. The characteristic oral lesions of pemphigus vulgaris are thin-walled fl accid bullae arising on otherwise normal skin or mucosa. The bullae rupture and extend peripherally, giving a large area of denuded skin or mucosa. Here, we report a case of a 56-year-old female patient suffering from exacerbations and remissions of pemphigus vulgaris since last 2 years, with a detailed review of literature. The present case report emphasizes a need of detailed case history, thorough oral and systemic examination, investigative measures, and a proper treatment regimen.
Oral Pemphigus Vulgaris: A case report
Journal of Chitwan Medical College, 2020
Pemphigus vulgaris is an autoimmune blistering disease clinically presenting as vesicles, bullae and erosion on the skin and mucous membrane. Histopathologically, it is characterized by acantholysis and supra basilar split. The underlying mechanism causing intra epithelial split in pemphigus vulgaris is binding of IgG autoantibodies to desmoglin 3 which belongs to a transmembrane glycoprotein adhesion molecule. Here we present a case of 27 years old male patient suffering from pemphigus vulgaris. This Case report highlights the importance of detailed case history, thorough clinical examination and appropriate investigation to reach a diagnosis of vesiculobullous lesion.
International Archives of BioMedical and Clinical Research, 2016
Pemphigus is a life-threatening autoimmune blistering disease targeting skin and mucous membranes. It is clinically characterized by flaccid blisters and erosions, while histologically shows intraepithelial acantholysis. The disruption of desmoglein-dependent cell adhesion by autoantibodies is the basic pathophysiology in blister formation of pemphigus. The clinical and histological spectrum of pemphigus is complex and differs in various variants of pemphigus. This review offers an answer to why the splits associated with pemphigus foliaceus occur in the superficial layer of the epidermis, while those of pemphigus vulgaris occur deep in the epidermis. With the help of desmoglein compensation theory, it logically explains why oral erosions develop in patients with pemphigus vulgaris, but not in patients with pemphigus foliaceus and why some patients with pemphigus vulgaris have only oral involvement, but others have extensive lesions on both skin and mucous membranes. Learning objective: After completing this article, readers shall be familiar with the clinical presentations, histologic findings, immunopathology of classical pemphigus and its variants. It discusses the desmoglein compensation theory of pathogenesis. along with the management of pemphigus.
Pemphigus Vulgaris: An Insight on Conventional and Emerging Treatment Modalities
International Research Journal of Pharmacy, 2013
According to Sir William Osler, Mouth is the mirror of the body which reflects systemic diseases. The oral mucosa may be affected by a variety of mucocutaneous diseases and oral lesions may occur first or very early in several muco-cutaneous disorders. Pemphigus is a group of potentially life threatening, chronic auto immune disorder characterized by epithelial blistering affecting muco-cutaneous surfaces. Autoantibodies in pemphigus vulgaris are directed against desmoglein in epithelial desmosomes, and the immune deposits result in intra-epithelial splitting (acantholysis). Oral lesions in the form of blisters and erosions are the first to manifest, and diagnosis is made by positive nikolsky's sign, coupled with characterstic histopathology and immunofluorescent features. Corticosteroids form the mainline of treatment, but adjuvant and newer therapies are also been used at an increasing rates. This article deals about the etiopathogenesis, clinical characterstics, diagnosis and treatment strategies for pemphigus vulgaris.
A Comprehensive Review on Pemphigus Vulgaris
BJSTR, 2017
Pemphigus vulgaris is a life threatening chronic autoimmune disease characterized by the formation of intraepithelial blisters on the skin and mucous membranes. Pemphigus vulgaris initially manifests in the form of intraoral lesions which spread to other mucous membranes and the skin. Pemphigus vulgaris (PV), the most common and important variant, is an autoimmune blistering disease characterized by circulating pathogenic IgG (immunoglobin) antibodies against desmoglein 3 (Dsg3), about half the patients also having Dsg1 autoantibody. Oral lesions are initially vesiculobullous but readily rupture, new bullae developing as the older ones rupture and ulcerate. Biopsy of perilesional tissue, with histological and immune staining examinations, is essential to the diagnosis. The diagnosis is based on clinical findings and laboratory analyses, and it is usually treated by the combined administration of corticosteroids and immune suppressants. Detection of the oral lesions can result in an earlier diagnosis. The current review focuses on the etiopathogenesis, diagnosis and current treatment of pemphigus vulgaris.
A pen sketch for oral pemphigus vulgaris: A review
Journal of Oral Medicine, Oral Surgery, Oral Pathology and Oral Radiology, 2023
Pemphigus is a rare chronic mucocutaneous autoimmune bullous dermatosis. Based on clinical features and pathophysiology the various subtypes include pemphigus Vulgaris (PV), pemphigus foliaceus (PF), IgA pemphigus, and paraneoplastic pemphigus (PNP). Autoantibodies against desmogleins 1 and 3 cause pemphigus Vulgaris which results in acantholysis, or the loss of cell-to-cell adhesion ultimately causing potentially lethal bullae and erosion formation. 80 to 90% of patients develop oral lesions that are manifested before mucocutaneous lesions in more than half of patients. Dental professionals are pivotal and can thus diagnose the disease and prevent skin involvement through proper therapy. Treatment should include systemic corticosteroids and immunosuppressive drugs. Intravenous pulse therapy is instituted in severe cases of pemphigus. This article is an attempt to present clinical manifestations, pathophysiology, and newer medical treatment modalities of pemphigus. Keywords: Vesiculobullous diseases, Desmogleins, Autoimmune diseases.
Pemphigus vulgaris - approach and management (Review)
Experimental and Therapeutic Medicine
The place of pemphigus vulgaris (PV) among autoimmune bullous dermatoses is well known. In pemphigus, IgG autoantibodies are directed against desmogleins 1 and 3, which are part of the cadherin family of cell-cell adhesion molecules. These structures are responsible for maintaining the intercellular adherence in stratified squamous epithelia, such as the skin and oral mucosa. The incidence of autoimmune bullous dermatoses is steadily increasing, being associated with a high degree of morbidity. The pathophysiology of these dermatoses is very well understood, complemented by recent genetic studies. The gold standard for the diagnosis of pemphigus vulgaris is the detection of autoantibodies or complement component 3 by direct immunofluorescence microscopy of a perilesional biopsy. Early diagnosis and initiation of treatment are necessary in order to achieve a favorable prognosis. Although the first line of treatment is corticotherapy, there are no clear guidelines on dosing regimens, and long-term adverse effects are important. Corticosteroid-sparing adjuvant therapies have been employed in the treatment of PV, aiming to reduce the necessary cumulative dose of corticosteroids. In addition, therapies with anti-CD20 antibodies are used, but antigen-specific immune suppression-based treatments represent the future.
Revisiting pemphigus vulgaris: A case report and review of literature
International Journal of Case Reports and Images
Introduction: Pemphigus is an autoimmune blistering mucocutaneous disease. Production of auto-antibodies against desmosomal glycoproteins is a characteristic feature of the disease. Oral lesions of pemphigus vulgaris are many a times first sign of the disease and hence it is important for a dentist to be familiar with the clinical appearance, diagnosis, and treatment modalities. Case Report: This report describes a case of pemphigus vulgaris in a female patient who presented with ulcers on the gingiva. On the basis of clinical examination and histopathology, we arrived at a diagnosis of pemphigus vulgaris, which was confirmed by immunofluorescence. Conclusion: If left undiagnosed or untreated pemphigus vulgaris may be fatal. Early diagnosis will aid in appropriate treatment and better outcome of the disease.
Atypical clinical appearance of pemphigus vulgaris on the face: case report
Acta dermatovenerologica Croatica : ADC, 2005
Pemphigus vulgaris is an organ-specific autoimmune mucocutaneous disorder. In the majority of cases, the disease manifests initially with oral lesions, and may be limited to a single site for months before spreading. A 78-year-old woman with yellowish crusted areas on her left preauricular region and close to the medial angle of her right eye is presented. Although she described an episode of erosions on her lower lip, the involvement of mucosal surfaces was not noticed on examination. Before she presented to our Department, she was misdiagnosed as an actinic cheilitis and malignant skin tumor. Histopathologic examination and direct immunofluorescence confirmed the diagnosis of pemphigus vulgaris. Immunoblotting of epidermal extracts detected IgG antibodies against desmoglein 3 but not desmoglein 1, which was also confirmed by ELISA test. The patient responded favorably to systemic corticosteroid therapy combined with adjuvant immunosuppressive therapy, with complete clearance of th...