Delayed Diagnosis of Pemphigus Vulgaris Initially Presenting as an Oral Ulcer: A Case Report (original) (raw)
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Pemphigus Vulgaris as Oral Mucosal Ulcer Progressive to Skin Lesion
Update Dental College Journal, 2014
Among all the life threatening diseases, autoimmune diseases are among most notorious and complicated diseases. As these diseases have no specific cause or responsible organism, it is therefore very difficult to distinguish and diagnose and thus stands as a major threat for public health. It is assumed that the reason for these diseases may be from genetic to environmental factors, from stress to idiopathic origin. Pemphigus Vulgaris(PV) is such an autoimmune blistering disease that frequently affects the mucous membrane and skin. Very often it starts from oral cavity and Dentists therefore the first to recognize. This paper describes the case of a patient presenting with a one-year history of painful ulcerated gingiva, tongue, floor of the mouth and even on GIT who is finally diagnosed as having PV spreading to generalized skin. DOI: http://dx.doi.org/10.3329/updcj.v3i2.17999 Update Dent. Coll. j: 2013; 3 (2): 43-47
Oral ulcer and skin lesions: a tell-tale sign of pemphigus vulgaris
Pediatria i Medycyna Rodzinna
Aim: The aim of this case presentation was to highlight the importance of the awareness of two different coexisting pathologies, as well as emphasise that early diagnosis and prompt management are essential to manage pemphigus vulgaris. Pemphigus vulgaris is a rare, life-threatening autoimmune disease characterised by the presence of blisters on the surface of oral mucosa caused by antibodies against adhesion molecules on the cell surface of keratinocytes. Coexistence with various conditions, notably herpes simplex virus, is noteworthy. Case report: We report a case of a middle-aged female who presented with non-healing oral ulceration that turned out to be herpes simplex virus, consequently triggering pemphigus vulgaris. Histopathology and immunofluorescence were suggestive of pemphigus vulgaris, but immunohistochemistry and polymerase chain reaction were indicative of herpes simplex virus infection. Conclusion: The coexistence of a viral infection with pemphigus vulgaris poses a t...
2020
Background: Pemphigus is a rare group of life-threatening muco-cutaneous autoimmune blistering diseases. Frequently, oral lesions precede the cutaneous ones. Sometimes oral clinical features could be misdiagnosed as aphthous ulcer especially when cutaneous lesion could not be found. Histopathology examination is essential to diagnose any vessicobullous diseases. Case report: A case of 44-year-old man with chief complaint recurrent oral ulceration since two years ago, working diagnosed as suspect tuberculosis cutis orificalis was reported. Histopathological examination showed suprabasilar blisters contained of acantholytic cells. Basillary cells remained attached to the basement membrane and lamina propria, arranged like "thombstonesappearance". Lymphocytes and neutrophills were the main inflammatory cells in dermis. Discussion: Clinically, it may be difficult to differentiate recurrence aphthouslike ulcer as oral pemphigus, especially when oral lesion was the main clinical feature. To make diagnose of pemphigus, histopathological examination is necessary and must be done. In some case when both direct and indirect immunofluorescence could not be performed, immunohistochemistry is one of alternative to determine the autoantibodies that involve in disease. Unfortunately this examination is not available in our department and several studies still ongoing in purpose to replace immunofluorescence with immunohistochemistry.
Pemphigus vulgaris: a case-based update
Journal (Canadian Dental Association), 2005
Pemphigus vulgaris (PV) is an autoimmune disease accounting for 80% of all cases of pemphigus. Before the advent of corticosteroid therapy, pemphigus was fatal, with a mortality rate of up to 75% in the first year. It is still a serious disorder, but the 5% to 10% mortality rate is now primarily due to the side effects of therapy. In 75% to 80% of cases, PV lesions first appear in the oral cavity. Dentists are therefore in a unique position to recognize the oral manifestations of the disease, allowing early diagnosis and initiation of treatment. The diagnosis is based on pathological examination and immunofluorescence testing. Systemic corticosteriods and steroid-sparing agents are the mainstays of treatment; topical corticosteroids may also be used to accelerate healing of persistent oral lesions. This article describes a 71-year-old woman with multiple chronic ulcers in the oral cavity, in whom PV was diagnosed 4 months after the symptoms first appeared. The article also reviews t...
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.
Journal of Clinical & Experimental Dermatology, 2010
Pemphigus vulgaris is a chronic autoimmune mucocutaneous disease characterized by the formation of intraepithelial blisters. It results from an autoimmune process in which antibodies are produced against desmoglein 1 and desmoglein 3, normal components of the cell membrane of keratinocytes. The first manifestations of pemphigus vulgaris appear in the oral mucosa in the majority of patients, followed at a later date by cutaneous lesions. The diagnosis is based on clinical findings and laboratory analyses, and it is usually treated by the combined administration of corticosteroids and immunosuppressants. Detection of the oral lesions can result in an earlier diagnosis. We review the oral manifestations of pemphigus vulgaris as well as the differential diagnosis, treatment, and prognosis of oral lesions in this uncommon disease.
Blistering mucocutaneous diseases of the oral mucosa--a review: part 2. Pemphigus vulgaris
PubMed, 2006
Oral mucous membranes may be affected by a variety of blistering mucocutaneous diseases. In this paper, we review the clinical manifestations, typical microscopic and immunofluorescence features, pathogenesis, biological behaviour and treatment of pemphigus vulgaris. Although pemphigus vulgaris is not a common disease of the oral cavity, its potential to cause severe or life-threatening disease is such that the general dentist must have an understanding of its pathophysiology, clinical presentation and management.
Oral pemphigus vulgaris: A case report with direct immunofluorescence study
Journal of Oral and Maxillofacial Pathology, 2016
Pemphigus vulgaris (PV) is a chronic, autoimmune, intraepidermal blistering disease of the skin and mucous membranes. The initial clinical manifestation is frequently the development of intraoral lesions, and later, the lesions involve the other mucous membranes and skin. The etiology of this disease still remains obscure although the presence of autoantibodies is consistent with an autoimmune disease. These antibodies are targeted against the adhesion proteins of keratinocytes, leading to acantholysis (disruption of spinous layer, leading to intraepidermal clefting) and blister formation. Because only oral lesions are present initially, the chances of misdiagnosing the disease as another condition are increased, leading to inappropriate therapy. In this article, we report a case of PV with only oral manifestations in a 36-year-old male.
Oral pemphigus vulgaris: a case report with review of the literature
Journal of Oral Science, 2008
Pemphigus vulgaris is a chronic autoimmune mucocutaneous disease that initially manifests in the form of intraoral lesions, which spread to other mucous membranes and the skin. The etiology of pemphigus vulgaris is still unknown, although the disease has attracted considerable interest. The pemphigus group of diseases is characterized by the production of autoantibodies against intercellular substances and is thus classified as autoimmune diseases. Most patients are initially misdiagnosed and improperly treated for many months or even years. Dental professionals must be sufficiently familiar with the clinical manifestations of pemphigus vulgaris to ensure early diagnosis and treatment, since this in turn determines the prognosis and course of the disease. Here, we report a case of pemphigus vulgaris that was misdiagnosed in its earliest stage.
Pemphigus vulgaris: update on etiopathogenesis, oral manifestations, and management
Critical Reviews in Oral Biology & …, 2002
Pemphigus is a group of potentially life-threatening diseases characterized by cutaneous and mucosal blistering. There is a fairly strong genetic background to pemphigus with linkage to HLA class II alleles. Certain ethnic groups, such as Ashkenazi Jews and those of Mediterranean origin, are especially liable to pemphigus. Pemphigus vulgaris (PV), the most common and important variant, is an autoimmune blistering disease characterized by circulating pathogenic IgG antibodies against desmoglein 3 (Dsg3), about half the patients also having Dsg1 autoantibodies. 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 immunostaining examinations, is essential to the diagnosis. Serum autoantibodies to either Dsg1 or Dsg3 are best detected by both normal human skin and monkey esophagus or by enzyme-linked immunosorbent assay (ELISA). Before the introduction of cortico...