Oral mucosal diseases: the inflammatory dermatoses

Abstract

The oral inflammatory dermatoses is a term used to describe a number of predominantly immune-mediated disorders: lichen planus (LP), erythema multiforme (EM), the vesiculobullous diseases pemphigoid (MMP), pemphigus (PV) and epidermolysis bullosa acquisita (EBA). These conditions are characterized by frequent involvement of the oral mucosa and often associated with extraoral manifestations, particularly of the skin, but can involve the eyes, both the conjunctiva and sclera, the nasal and pharyngeal mucosa, as well as the genitals. Given their frequent, and sometimes initial involvement of the oral mucosa, oral health professionals need to be both familiar with the clinical features and presentations of these conditions, and appreciate their critical role in management.This paper reviews the clinical features and presentation of the oral dermatoses, provides guidance as to the appropriate investigations needed to differentiate and correctly diagnose these conditions, details the aetio-pathology of these diseases and discusses their management.Keywords: Autoimmune, blister, bullae, corticosteroids, dermatoses, desquamative, erosion, immune-mediated, immunosuppression, steroid-sparing, vesicles, vesiculobullous, ulcers.Abbreviations and acronyms: ADCC = antibody dependent cellular cytotoxicity; BMZ = basement membrane zone; CDC = complement dependent cytotoxicity; cGVHD = chronic graft-versus-host disease; DIF = direct immuno-fluorescence; DLE = discoid lupus erythematosus; EB = epidermolysis bullosa; EBA = epidermolysis bullosa acquisita; EM = erythema multiforme; c-IFN = gammainterferon; MMP = mucous membrane pemphigoid; OCP = ocular cicatricial pemphigoid; OLCL = oral lichenoid contact lesions; OLDR = oral lichenoid drug reactions; OLP = oral lichen planus; OMMP = oral mucous membrane pemphigoid; PUVA = psoralen ultraviolet A light; PV = pemphigus vulgaris; SJS = Stevens Johnson syndrome; TEN = toxic epidermal necrolysis; TNF-a = tumour necrosis factor alpha. ORAL LICHEN PLANUSLichen planus is a chronic systemic disease of established immune-mediated pathogenesis.1 It most commonly, and most protractedly, involves the mucosa of the oral cavity, hence it is of interest and concern to dentists, but it can involve other sites, namely the skin, the scalp (with inflammation around and affecting the hair follicles resulting in alopecia), the nails as well as the genital area -the vulval and vaginal mucosa, and the glans penis. There are six recognized oral presentations of lichen planus: (1) reticular; (2) papular; and (3) plaque-form; and the (4) atrophic; (5) ulcerative (erosive) and rare (6) bullous form. These latter three forms can be associated with significant discomfort requiring either topical and ⁄ or systemic immunosuppressive therapy.

Pemphigus Vulgaris with Tense Bullae

“…Histologic examination revealed suprabasal acantholysis (Figure 2), the histologic hallmark of Pemphigus vulgaris (PV) that is essential for diagnosis. 1 Furthermore, direct immunofluorescence showed intercellular staining with immunoglobulin G (IgG) and complement test C3, which was also consistent with PV.…”

Erythema Multiforme

“…Infectious agents are the most common trigger for EM, with HSV-1 implicated in > 70% of cases (1)(2)(3)(4)(5). Other common infectious agents include HSV-2 and Mycoplasma pneumoniae (1)(2)(3)(4).…”

“…In contrast to the Reprints are not available from the authors. RECEIVED: 26 May 2015; ACCEPTED: 4 June 2015 minor variant, EM major is generally more severe, widespread, and associated with prodromal symptoms before the onset of cutaneous and mucosal lesions (1)(2)(3)(4).…”

“…EM is an immune-mediated hypersensitivity reaction most commonly seen in patients between 20 and 30 years of age, and it has a possible female predilection (1)(2)(3)(4). Infectious agents are the most common trigger for EM, with HSV-1 implicated in > 70% of cases (1)(2)(3)(4)(5).…”

“…Other common infectious agents include HSV-2 and Mycoplasma pneumoniae (1)(2)(3)(4). Several medications are considered highly suspicious for triggering episodes of EM, including sulfonamides, nonsteroidal antiinflammatory drugs (NSAIDs), and penicillins (1)(2)(3)(4).…”