The role of upper endoscopy in identifying oesophageal involvement in patients with oral pemphigus vulgaris

doi:10.1016/j.dld.2004.10.005

Digestive and Liver Disease

Volume 37, Issue 3, March 2005, Pages 195-199

https://doi.org/10.1016/j.dld.2004.10.005 Get rights and content

Abstract

Background and aims.

The involvement of oesophagus in pemphigus vulgaris is still debated. The aims of this study were to evaluate the prevalence of oesophageal involvement and the gastro-duodenal mucosa appearance before and after high-dose corticosteroid therapy in a group of patients with oral pemphigus vulgaris.

Methods.

We prospectively studied 28 consecutive patients with oral pemphigus by oesophageal symptom standardised questionnaire, upper gastro-intestinal endoscopy, exfoliative cytology and histological biopsy. After clinical remission, all patients underwent new endoscopy.

Results.

The prevalence of oesophageal symptoms was 57.1%. Endoscopic examination revealed oesophageal involvement with different degrees of severity in 67.8% of patients. After corticosteroid therapy, endoscopy showed normal oesophageal–gastro-duodenal mucosa. No examination-related exacerbations of the oesophageal lesions were seen.

Conclusions.

The upper gastro-intestinal endoscopic examination, in oral pemphigus vulgaris patients with oesophageal symptoms, is safe in skilled hands technique and a useful diagnostic tool prior to starting therapy.

Introduction

Pemphigus vulgaris (PV) is a rare autoimmune blistering disease involving skin and mucous membranes without sex differences, but with a potentially fatal outcome [1], [2]. The skin and the oral mucosa are the most frequently involved sites: oral lesions precede skin lesions in 70% of cases, and when skin is already involved, there is an oral comorbidity in 90% of cases [3]. Nevertheless, any stratified squamous epithelium can be involved.

In PV, acantholysis occurs low in the stratum spinosum with a superbasal cleft [4]. A study reported that, as in the epidermis, the cell adhesion molecule desmoglein 3 (the target antigen of pathogenetic PV antibodies) is strongly expressed in oesophageal epithelia [5], [6].

The prevalence of oesophageal involvement is still debated. Some authors suggest that it is rare [7], [8], [9], [10], [11], [12], [13]; others reported higher prevalence [3], [14]. Because patients with oesophageal involvement may be asymptomatic and endoscopy may not be routinely performed, such involvement may be under-recognised.

The aims of this study were to evaluate in 28 consecutive patients with oral PV:

(1)
the prevalence of oesophageal involvement, and
(2)
the gastro-duodenal mucosa appearance before and after high-dose corticosteroid therapy.

Section snippets

Population

From November 1991 to December 2002, we prospectively studied 28 consecutive patients, referred from Department of Oral Medicine of Federico II University, with oral PV newly diagnosed on the basis of histology, indirect (IIF) and direct (DIF) immunofluorescence [8]. Details on IIF and DIF technique have been presented in the original article [3]. IIF was scored on the basis of titre (1:20–1:40, weakly positive; 1:80–1:160, positive; 1:160–1:640, highly positive). DIF was positive in all

Methods

Oesophageal symptoms were scored by a standardised questionnaire, dealing with the presence or absence of dysphagia, odynophagia, heartburn, regurgitation and chest pain. Any other symptom was specified in an open box in the questionnaire [15]. In each patient, we scored the presence of one or more symptoms as cumulative score (0–5).

All patients underwent upper gastro-intestinal endoscopy (UGE) (standard pre-medication by diazepam 10 mg and hyscine bromure 20 mg i.v.) with histological biopsies

Results

The prevalence of oesophageal symptoms was 57.1% (18/28). The prevalence of each symptom is reported in Table 1. The oesophageal involvement was detected by UGE in 19 (67.8%) patients. The main endoscopic findings were localised erythema (scored as 1), red longitudinal lines (scored as 2), erosions (scored as 3), blisters (scored as 4) and ulcers (scored as 5). When more than one endoscopic finding was found in the same patient, we scored the worst. In all 19 patients with affected oesophagus,

Discussion

In this study, we demonstrated a high prevalence of oesophageal symptoms (57.1%) and oesophageal endoscopic involvement (67.8%) with a strong correlation between them in patients with oral PV. Moreover, no correlation was found between either oral IIF or DIF and oesophageal involvement.

The prevalence of oesophageal involvement in PV is thought to be uncommon [7], [8], [9], [10], [11], [12], [13]. This assumption is based on two large series of autopsy findings in PV patients, both reported in

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Pemphigus defines a group of rare mucocutaneous autoimmune diseases of which pemphigus vulgaris (PV) is the most common.
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It seems to respond to corticosteroid or immunosuppressive therapy [2]. Since oral PV frequently relapses [10,11], and esophageal symptoms could develop again [4], a multidisciplinary surveillance program has been instituted in our University Hospital. The issue of upper endoscopy in patients affected with PV is very important.
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Digestive EndoscopyThe role of upper endoscopy in identifying oesophageal involvement in patients with oral pemphigus vulgaris

Abstract

Background and aims.

Methods.

Results.

Conclusions.

Introduction

Section snippets

Population

Methods

Results

Discussion

J Am Acad Dermatol

Lancet

Oral Surg Oral Med Oral Pathol

Oral Surg Oral Med Oral Pathol

J Invest Dermatol

J Am Acad Dermatol

Lancet

Am J Gastroenterol

Oral Surg Oral Med Oral Pathol

Gastrointest Endosc

J Am Acad Dermatol

J Invest Dermatol

Lancet

Lancet

Desmoglein 3-ELISA: a pemphigus vulgaris specific diagnostic tool

Arch Dermatol

Pemphigus: current concepts

Ann Intern Med

Relapse of pemphigus vulgaris presenting with haematemesis

Dermatology

Digestive Endoscopy
The role of upper endoscopy in identifying oesophageal involvement in patients with oral pemphigus vulgaris