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Oral pemphigus vulgaris

Isabella Jascholt, Olivia Lai, Detlef Zillikens, Michael Kasperkiewicz
Periodontitis and autoimmune bullous diseases, including pemphigus vulgaris and mucous membrane pemphigoid, are immunoinflammatory disorders leading to microbial plaque- and autoantibody-elicited tissue injury of the oral cavity, respectively. Evidence indicates that these autoimmune conditions may represent a risk factor for periodontitis, but no systematic evaluation exists to corroborate this assumption. A systematic literature review of periodontal status in pemphigus and pemphigoid was conducted. Electronic searches using PubMed from inception to July 2016 identified 10 studies meeting predetermined inclusion and exclusion criteria...
December 28, 2016: Journal of the American Academy of Dermatology
Aneliza de Fatima Moraes da Silva, Daniel Galera Bernabé, Glauco Issamu Miyahara, Eder Ricardo Biasoli, Renata Callestini, Kellen Cristine Tjioe
Oral biopsy of vesiculobullous diseases such as pemphigus vulgaris often raises questions due to some particularities involving this procedure. The adequate selection of the area to be biopsied defines if the final diagnosis will be reached, being the Achiles heel of the proper management of the patient. Here, the authors report a case of a woman who sought for treatment of generalized oral blisters and ulcers that caused severe pain. She had undergone a previous biopsy by other professionals that was inconclusive...
November 2016: Journal of Craniofacial Surgery
Christopher DiMarco
Pemphigus vulgaris (PV), pemphigus foliaceus (PF), and paraneoplastic pemphigus (PNP) are a group of rare and fatal blistering diseases involving autoantibodies that target desmosomal proteins. The pathogenesis of pemphigus involves the production of activated B-cells and IgG with stimulation by IL-4 by T-helper 2 cells. Clinically these diseases present most often with epidermal erosions of the mucosae and skin caused by rapid rupturing of flaccid bullae. These lesions correlate histologically with splits forming in the epidermis, leaving a blister roof composed of a few cell layers...
December 1, 2016: Rhode Island Medical Journal
Eden P Lake, Yu-Hui Huang, Iris K Aronson
Pemphigus vulgaris is an autoimmune blistering disorder treated with systemic steroids and immunosuppressive agents. Treatment of this disorder in young women of childbearing age must take into consideration the patient's desire for pregnancy and effects of the treatment on both mother and child. We report two young women with pemphigus, initially treated with standard immunosuppressive medications, who expressed their wishes for pregnancy. The immunosuppressive agents were tapered and both patients were treated with Rituximab and IVIG, permitting discontinuation of other medications, conception and pregnancy without any oral steroids or immunosuppressive agents...
November 20, 2016: Journal of Dermatological Treatment
Aneliza de Fatima Moraes da Silva, Daniel Galera Bernabé, Glauco Issamu Miyahara, Eder Ricardo Biasoli, Renata Callestini, Kellen Cristine Tjioe
Oral biopsy of vesiculobullous diseases such as pemphigus vulgaris often raises questions due to some particularities involving this procedure. The adequate selection of the area to be biopsied defines if the final diagnosis will be reached, being the Achiles heel of the proper management of the patient. Here, the authors report a case of a woman who sought for treatment of generalized oral blisters and ulcers that caused severe pain. She had undergone a previous biopsy by other professionals that was inconclusive...
October 14, 2016: Journal of Craniofacial Surgery
Sangeetha Jeevan Kumar, S P Nehru Anand, Nandhini Gunasekaran, Rajkumar Krishnan
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...
September 2016: Journal of Oral and Maxillofacial Pathology: JOMFP
A von Köckritz, S Ständer, C Zeidler, D Metze, T Luger, G Bonsmann
BACKGROUND: Pemphigus vegetans is a rare variant of pemphigus vulgaris, accounting for 1-2% of all pemphigus diseases. Systemic corticosteroids are the therapy of first choice in combination with immunosuppressants as steroid-sparing agents. OBJECTIVE: To highlight the exceptional but successful use of minocycline/nicotinamide monotherapy in pemphigus vegetans. METHODS: A review of the literature to date about pemphigus vegetans with special emphasis on therapy was performed...
January 2017: Journal of the European Academy of Dermatology and Venereology: JEADV
Julia Yu-Fong Chang, Chun-Pin Chiang, Yi-Ping Wang, Yang-Che Wu, Hsin-Ming Chen, Andy Sun
BACKGROUND: Desquamative gingivitis (DG) is principally associated with erosive oral lichen planus (EOLP), mucous membrane pemphigoid (MMP), and pemphigus vulgaris (PV). METHODS: Serum autoantibodies including antigastric parietal cell antibody (GPCA), antithyroglobulin antibody (TGA), and antithyroid microsomal antibody (TMA) were measured in 500 patients with DG, 287 EOLP without DG (EOLP/DG(-) ) patients, and 100 healthy control subjects. RESULTS: The 500 patients with DG were diagnosed as having EOLP in 455 (91%), PV in 40 (8%), and MMP in five (1%) patients...
September 7, 2016: Journal of Oral Pathology & Medicine
Ken Muramatsu, Wataru Nishie, Ken Natsuga, Yasuyuki Fujita, Hiroaki Iwata, Tamaki Yamada, Emi Yamashita, Takuya Asaka, Hiroshi Shimizu
Oral lichen planus (OLP) is a chronic inflammatory disorder of the oral mucosa of unknown etiology. Clinically, the erosive type of OLP (erosive OLP) can show features similar to those of pemphigus vulgaris (PV), an autoimmune blistering disorder in which desmoglein (Dsg)3 is targeted. In addition to clinical and histopathological findings, immunological studies, including direct immunofluorescence (IF), indirect IF and enzyme-linked immunosorbent assay (ELISA) that detect autoantibodies to Dsg3, are helpful in differentiating erosive OLP from PV...
November 2016: Journal of Dermatology
Noritaka Oyama, Ari Togashi, Erika Nomura, Fumio Kaneko
Pemphigus vulgaris (PV) is an acquired autoimmune disease in which the disease characteristic antibodies are directed against the desmosomal transmembrane glycoprotein, desmoglein 3 (Dsg 3), resulting in flaccid blisters and erosions of skin and mucous membrane. Among various affected sites, ocular involvement may often persist or relapse even after remission of other mucocutaneous lesions, and also represent a higher morbidity. We describe such an example case of mucosal PV, whose oral and ocular manifestations were responded specifically to oral cyclosporine and mizoribine, respectively...
November 2016: Dermatologic Therapy
Davide Bartolomeo Gissi, Achille Tarsitano, Chiara Baldovini, Andrea Gabusi
This report describes the management of an unusual case of oral pemphigus vulgaris (PV). The patient was referred for a painful single bullous lesion together with a small proliferative area localized in the soft palate. Histology and direct immunofluorescence data were consistent for PV but disclosed unusual signs of high-grade dysplasia in the proliferative area. At surgical removal of the dysplastic area 1 week after the start of cortisone therapy there was no evidence of dysplasia. Histological signs of high-grade dysplasia in oral mucosa are often associated with concurrent or subsequent carcinoma...
December 2016: International Journal of Surgical Pathology
Vinod Kumar Sharma, Neetu Bhari, Somesh Gupta, Kanika Sahni, Neena Khanna, M Ramam, G Sethuraman
BACKGROUND: Pulsed corticosteroids have been used successfully for the management of pemphigus. However, prolonged use of glucocorticoids may be associated with adverse effects and some patients show a poor response to conventional therapy. Biologics have shown a promising role in such cases; however, there is limited data from the Indian subcontinent. OBJECTIVE: The primary objective was to assess the efficacy and adverse effects of rituximab in pemphigus. The secondary objective was to measure the cumulative doses of corticosteroids required for these patients...
July 2016: Indian Journal of Dermatology, Venereology and Leprology
Marcia Ramos-E-Silva, Natalia Regina Martins, George Kroumpouzos
Physiologic alterations of the oral and vulvovaginal mucosal surfaces result from the profound hormonal and immunologic changes of gestation. High estrogen levels are responsible for the vascular changes noted on mucosal surfaces. Gingival hyperemia and edema, gingivitis and pyogenic granuloma are the most common alterations of the oral mucosa during gestation. Physiologic changes of the vulvovaginal area are mainly of vascular nature, and include among others varicose veins. The oral and vulvovaginal mucosal surfaces can be affected by diseases that can worsen or develop in pregnancy...
May 2016: Clinics in Dermatology
Mahin Bakhshi, Soheila Manifar, Nemat Azizi, Hamid Asayesh, Parvin Mansouri, Soheila Nasiri, Zeynab Hashemi, Aida Mehdipour
Pemphigus vulgaris (PV) is considered a chronic disease with a potentially fatal outcome. Studies have suggested that environmental factors and personal habits play an important role in the pathogenesis of PV, but more studies are required to elaborate their connection to the disease. The goal of this multicenter case-control study was to determine some of the environmental factors related to PV. Cases (n = 99) were patients with PV, and controls (n = 198) were individuals who did not have PV. Data about demographics, history, clinical findings, occupational exposures to pesticides, smoking status, number of births (parity), herpes simplex virus (HSV) infection, and fast food consumption were collected using a structured questionnaire...
May 2016: General Dentistry
D T Greenblatt, E C Benton, R W Groves, J F Setterfield
Pemphigus vulgaris (PV) is an autoimmune blistering disease affecting the skin and mucous membranes. Rituximab, a CD20 chimeric monoclonal antibody, has efficacy in PV management. We report a case of severe oral PV that showed a progressive response to repeated courses of rituximab, culminating in a rapid response within 4 weeks following severe relapse 4 years after initial therapy. It demonstrates the progressively shorter time to achieve partial or complete remission following rituximab infusions, combined with minimal adjuvant therapy over a 7-year follow-up period...
July 2016: Clinical and Experimental Dermatology
Yi-Xiu Bai, Li-Ming Zhang, Ting Xiao, Hong-Duo Chen
There is a lack of data on treatment and prognosis of pemphigus in China. The aim of this study was to evaluate long-term follow-up and prognosis of pemphigus. Forty-seven inpatients with pemphigus vulgaris (PV) and 22 with pemphigus foliaceus (PF) were recruited in this retrospective study. The average age at onset was 51.6 and 54.9 years in PV and PF, respectively. High-dose systemic steroids were administered in 47 PV and 21 PF, of which 18 PV and 8 PF with adjuvant therapies. CD4 lymphocytopenia was found in 5 PV and 2 PF patients at admission and successfully treated by intravenous thymopentin daily...
March 2016: Dermatologic Therapy
Mihai Radulescu
The oral ulcerations caused by aphtous lesions, herpetic lesions, candidiasis, ulcerative lichen planus, mucous membrane pemphigoid, and pemphigus vulgaris are managed in a step-up approach that can involve topical, intarlesional, and systemic pharmacologic management. This article reviews the common treatment agents, modalities, and dosages. The emphasis is on local pharmacologic therapies, yet systemic conditions that often present with such oral lesions are briefly reviewed, along with the appropriate management...
April 2016: Dental Clinics of North America
Gulmina Saeed Orakzai, Waqar-Un-Nisa, Saima Hanif Orakzai
BACKGROUND: Oral white lesions constitute a major clinical problem in Pakistan and South Asian countries. The study was done with the objective to analyse oral white lesions histologically and clinically, and evaluate association between various risk factors in different ages, gender, ethnic groups, sites and sizes of the lesion. METHODS: A total of 80 patients presenting with oral white lesions were included in this cross-sectional study conducted at Department of Histopathology, Armed Forces Institute of Pathology (AFIP), Rawalpindi...
October 2015: Journal of Ayub Medical College, Abbottabad: JAMC
Yi-Xiu Bai, Jin-Gang Chu, Ting Xiao, Hong-Duo Chen
Autoimmune bullous diseases (AIBDs)-associated interstitial lung disease (ILD) is extremely rare. Pemphigus vulgaris (PV) is an intraepidermal autoimmune blistering disease caused by circulating autoantibodies against desmoglein. To date, PV-associated ILD has rarely been reported in English literature. We report a rare association of PV and ILD. A 53-year-old Chinese female with PV for 8 months developed ILD after a relapse of PV for 2 months due to discontinuation of oral prednisone by herself. She was successfully treated by systemic methylprednisolone...
July 2016: Dermatologic Therapy
Paul R J Ames, Maria Graf, Fabrizio Gentile
BACKGROUND: Several autoimmune skin disorders are characterised by an increased risk of thrombosis, with bollous pemphigoid carrying a higher risk than pemphigus vulgaris (PV). We describe the case of a middle aged gentleman who developed recurrent venous thromboembolism despite adequate oral anticoagulation during very active PV that required escalation of treatment to bring the disease under control. CASE PRESENTATION: In May 2014 a 49 year gentleman was admitted for widespread mucocutaneous blistering diagnosed as PV by histology and immunofluorescence...
2016: Thrombosis Journal
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