Oral pemphigus vulgaris in children and adolescents: a review of the literature and a case report

Ariyawardana, Anura; Tilakaratne, Wanninayake Mudiyanselage; Dissanayake, Manel; Vitanaarachchi, N.; Basnayake, Lihini; Sitheeque, Mohaideen; Ranasinghe, A. W.

doi:10.1111/j.1365-263x.2005.00640.x

Cited by 29 publications

(42 citation statements)

References 38 publications

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“…Most cases are sporadic, with a few rare familial cases, as well as a higher incidence rate in Ashkenazi Jewish and Japanese populations (Joly and Litrowski, 2011, Rocha-Alvarez, et al, 2007). PV affects both men and women, and age of onset is typically between 40–60 years, although a few cases have been observed in children (Ariyawardana, et al, 2005, Kneisel and Hertl, 2011a). Treatment with immunosuppressive agents decreases the otherwise high mortality rate of PV patients to 5–10%.…”

Section: Acquired Desmosomal Diseasesmentioning

confidence: 99%

Desmosomes in acquired disease

Stahley

Kowalczyk

2015

Cell Tissue Res

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Desmosomes are cell-cell junctions that mediate adhesion and couple the intermediate filament cytoskeleton to sites of cell-cell contact. This architectural arrangement functions to integrate adhesion and cytoskeletal elements of adjacent cells. The importance of this robust adhesion system is evident in numerous human diseases, both inherited and acquired, that occur when desmosome function is compromised. This review focuses on autoimmune and infectious diseases that impair desmosome function. In addition, we discuss emerging evidence that desmosomal genes are often misregulated in cancer. The emphasis of our discussion is placed on how human diseases inform our understanding of basic desmosome biology, and in turn, how fundamental advances in the cell biology of desmosomes may lead to new treatments for acquired diseases of the desmosome.

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Section: Acquired Desmosomal Diseasesmentioning

confidence: 99%

Desmosomes in acquired disease

Stahley

Kowalczyk

2015

Cell Tissue Res

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“…PV is a chronic, life-threatening autoimmune mucocutaneous vesiculobullous disease characterized by suprabasal acantholysis, which leads to the formation of blisters that readily rupture, leaving erosions and ulcers of the skin or the mucosa. 5,9,14,15 It affects not only the skin and oral mucosa, but also the mucosa of the nose, conjunctivae, genitals, esophagus, pharynx, and larynx, mainly in middle-aged and elderly patients with intraepithelial immune deposits and loss of cell-to-cell contact (acantholysis), leading to intraepithelial vasiculation. 13 It affects women more than men, although some data show equal prevalence in both genders.…”

Section: Discussionmentioning

confidence: 99%

Implant‐Supported Oral Rehabilitation of a Patient with Pemphigus Vulgaris: A Clinical Report

Altın

Ergün

Katz

et al. 2013

Journal of Prosthodontics

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Pemphigus vulgaris (PV) is a rare mucocutaneous vesiculobullous disease characterized by the development of autoantibodies against the desmosomal proteins. Current treatment is largely based on systemic immunosuppression using systemic corticosteroids. Immunosuppressive drugs used in the treatment of the disease may increase the risk of infection and delayed healing, which are of concern in dental treatment procedures in this group of patients. The clinical outcomes of implants in PV have not been investigated. We present a case of PV rehabilitated with an implant-supported prosthesis with a 32-month follow-up and discuss the important points in the surgical and prosthodontic phases.

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“…4 The process of the pathogenesis in this condition occurs due to the production of auto-antibodies against desmogleins and desmocollins, an integral part of epidermal cell-cell adhesion which causes acantholysis leading to intraepithelial blister formation. 4,5 Exact mechanism causing this acantholysis is not elucidated, but many theories have been put forward. 1,4 Pemphigus vulgaris involves both the oral cavity and the skin.…”

Section: Introductionmentioning

confidence: 99%

Juvenile Pemphigus Vulgaris as a Differential Diagnosis for Chronic Generalized Oral Ulceration in an Adolescent: A Case Report and a Review of Literature

Hettiarachchi¹,

Jayasinghe²,

Gunasena³

et al. 2018

International Journal of Experimental Dental Science

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Pemphigus vulgaris (PV) is an autoimmune disorder involving the mucocutaneous tissues. Pathogenesis of this disease causes auto-antibodies against desmogleins in desmosomes which leads to intraepithelial blister formation. It is a rare but potentially life-threatening disease with a prevalence of 1 to 9 per 1 x 10 9 . The disease has an equal sex predilection and commonly occurs in the 5th and 6th decade of life. Clinically, oral lesions are more evident than skin lesions. Diagnosis of the disease is by clinicopathological correlation with the definitive diagnosis requiring immunofluorescent investigations. The mainstay of treatment involves immunosuppression through the use of corticosteroids and other steroid-sparing agents like dapsone, azathioprine, and methotrexate. Presentation of this entity in an adolescent is a rare occurrence, and this may lead to misdiagnosis of the condition. Quality of life of these patients can be improved by controlling the disease through early diagnosis and necessary management. In this article, we report a case of a 15-year-old male patient diagnosed with Juvenile PV (JPV) and discuss the possibility of pemphigusvulgaris as a differential diagnosis for chronic generalized oral ulceration in an adolescent with a brief review of the literature.

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Oral pemphigus vulgaris in children and adolescents: a review of the literature and a case report

Cited by 29 publications

References 38 publications

Desmosomes in acquired disease

Desmosomes in acquired disease

Implant‐Supported Oral Rehabilitation of a Patient with Pemphigus Vulgaris: A Clinical Report

Juvenile Pemphigus Vulgaris as a Differential Diagnosis for Chronic Generalized Oral Ulceration in an Adolescent: A Case Report and a Review of Literature

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