A Case of Concomitant Pemphigus Foliaceus and Oral Pemphigus Vulgaris

Paula M. Farthing,Ruth Murphy,Alexandra C. Perks,Anne M. Hegarty

doi:10.1007/s12105-017-0884-0

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.

Highlights

Pemphigus is a rare autoimmune mucocutaneous blistering condition with four variants; pemphigus vulgaris, pemphigus foliaceus, IgA pemphigus and paraneoplastic pemphigus
The two major types are pemphigus vulgaris (PV) and pemphigus foliaceus (PF) [2]. Both are characterised by suprabasal acantholysis, 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]
In skin, there is only a low amount of desmoglein 3 (Dsg3) which is expressed in the basal and parabasal layers only, whereas desmoglein 1 (Dsg1) is expressed throughout the entire epidermis and highly in the superficial layers [5,6,7]

Summary

Introduction

Pemphigus is a rare autoimmune mucocutaneous blistering condition with four variants; pemphigus vulgaris, pemphigus foliaceus, IgA pemphigus and paraneoplastic pemphigus. A biopsy from a crusted lesion on the scalp (Fig. 2) in 2003 revealed acantholysis of the keratinocytes in the upper part of the prickle cell layer (Fig. 3), and direct immunofluorescence studies showed IgG positivity around the upper epidermal cells These features were consistent with a diagnosis of PF. More significant than seen previously, were noted at this appointment These consisted of an ulcer on the soft palate and an erosion in the left buccal mucosa (Fig. 5). MMF dose was increased and incisional biopsies of the buccal mucosa for histopathology and direct immunofluorescence were organised This revealed intra-epithelial separation between prickle and basal cell layers (Fig. 6), and positive staining for IgG in the lower third of the epithelium. The dose of MMF has varied depending on symptoms and currently the skin, oral mucosa and genitals are stable on MMF 1 g in the morning and 500 mg in the evening

Discussion

Conclusion