A Case of Concomitant Pemphigus Foliaceus and Oral Pemphigus Vulgaris
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CASE REPORT
A Case of Concomitant Pemphigus Foliaceus and Oral Pemphigus Vulgaris Alexandra C. Perks1,2 · Paula M. Farthing2 · Ruth Murphy3 · Anne M. Hegarty1 Received: 15 October 2017 / Accepted: 29 December 2017 © The Author(s) 2018. This article is an open access publication
Abstract Pemphigus is a chronic autoimmune condition that can affect multiple areas of the body. The two main subtypes of pemphigus are pemphigus vulgaris (PV) and pemphigus foliaceus (PF) which can rarely occur concurrently or even transition from one to the other. The process of transition may be explained by qualitative changes in desmoglein autoantibody profile. We present a rare case of concomitant PF and oral PV and explore the literature on transitions between pemphigus subtypes and whether this case could represent a transition from PF to PV. Furthermore, the realities of multidisciplinary patient management are discussed. Keywords Pemphigus · Desmogleins · Epitopes · Autoimmune disease
Introduction Pemphigus is a rare autoimmune mucocutaneous blistering condition with four variants; pemphigus vulgaris, pemphigus foliaceus, IgA pemphigus and paraneoplastic pemphigus. These all differ in their histological features and target antigens [1]. The two major types are pemphigus vulgaris (PV) and pemphigus foliaceus (PF) [2]. Histologically both are characterised by suprabasal acantholysis, however in PV this occurs in the lower third of the epithelium/epidermis whereas in PF it occurs in the upper third of the epidermis [2, 3]. This acantholysis is as a result of circulating * Alexandra C. Perks [email protected] Paula M. Farthing [email protected] Ruth Murphy [email protected] Anne M. Hegarty [email protected] 1
Oral Medicine Unit, Charles Clifford Dental Hospital, 76 Wellesley Road, Sheffield S10 2SZ, UK
2
Unit of Oral and Maxillofacial Pathology, The School of Clinical Dentistry, University of Sheffield, 19 Claremont Crescent, Sheffield S10 2TA, UK
3
Department of Dermatology, Royal Hallamshire Hospital, Glossop Road, Sheffield S10 2JF, UK
autoantibodies targeting desmosomal cadherins within desmosomes that bind epithelial cells together [2, 4]. PV targets desmosomal cadherins named desmoglein 3 (Dsg3) and desmoglein 1 (Dsg1) whereas PF targets Dsg1 only. Dsg3 and Dsg1 display different expression patterns within mucosa and skin which influences the distribution of blistering [5, 6] (Fig. 1). In mucosa, Dsg3 is expressed highly throughout the entire epithelium, whereas Dsg1 is expressed in much lower amounts and mainly in the superficial layers. Conversely, in skin, there is only a low amount of Dsg3 which is expressed in the basal and parabasal layers only, whereas Dsg1 is expressed throughout the entire epidermis and particularly highly in the superficial layers [5–7]. The method by which these differing expression patterns affects the distribution of blisters is explained by the ‘Dsg compensation theory’ [5–8]. PF has only anti-Dsg1 autoantibodies, therefore in the mucosa the presence of Ds
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