Digestive EndoscopyThe role of upper endoscopy in identifying oesophageal involvement in patients with oral pemphigus vulgaris
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
References (37)
Pemphigus
J Am Acad Dermatol
(1988)- et al.
Pemphigus and bullous pemphigoid
Lancet
(1999) - et al.
Oral pemphigus: clinical significance of esophageal involvement
Oral Surg Oral Med Oral Pathol
(1997) Immunopathology of oral mucosal ulcerative desquamative and bullous diseases: selective review of literature
Oral Surg Oral Med Oral Pathol
(1994)- et al.
Lack of mucosal involvement in pemphigus foliaceus may be due to low expression of desmoglein 1
J Invest Dermatol
(1998) - et al.
Esophageal involvement in pemphigus vulgaris: a clinical, histologic and immunopathologic study
J Am Acad Dermatol
(1991) - et al.
Esophageal involvement in pemphigus vulgaris. Report of two cases and review of the literature
Lancet
(1999) - et al.
Esophageal impairment in adult coeliac disease with steatorrea
Am J Gastroenterol
(1998) Prednisone and azathioprine in treatment of patients with vesiculoerosive oral diseases
Oral Surg Oral Med Oral Pathol
(1981)- et al.
Pemfigus vulgaris with exclusive involvement of the esophagus: case report and review
Gastrointest Endosc
(2004)
Influence of treatment on the clinical course of pemphigus vulgaris
J Am Acad Dermatol
Mucosal and mucocutaneous (generalized) pemphigus vulgaris show distinct autoantibody profiles
J Invest Dermatol
Oesophageal involvement in pemphigus vulgaris
Lancet
Esophageal involvement in pemphigus vulgaris
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
Cited by (32)
Non eosinophilic chronic stricturing esophagitis: Lessons from thirty eight cases
2023, Clinics and Research in Hepatology and GastroenterologyEsophageal dissecans: A rare life-threatening presentation of recurrent pemphigus vulgaris
2015, American Journal of Emergency MedicineCitation Excerpt :The pathogenesis of EDS appears to be immune mediated due to immunoglobulin G antibodies against the desmoglein 3 proteins, although rare variants of immunoglobulin A against desmoglein 1 [1] and Intercellular Adhesion Molecule 1 have also been reported. The most common presentation of EDS include dysphagia (57.1%) and odynophagia (21.4%) [2]. Other manifestations include intractable heartburn, nausea, and vomiting, sometimes bringing out the entire esophageal mucosal cast.
Non-reflux esophagitis: A review of inflammatory diseases of the esophagus exclusive of reflux esophagitis
2014, Seminars in Diagnostic PathologyCitation Excerpt :Various autoimmune bullous dermatidides can involve the esophageal mucosa, with pemphigus vulgaris being the most common. More than 50% of patients with vesicobullous disease have esophageal symptoms and endoscopic esophageal abnormalities.71. Identification of esophageal involvement by pemphigus vulgaris may be the first step to diagnosing asymptomatic pemphigus vulgaris in some patients.72–74
Oral mucosal disease: Pemphigus
2008, British Journal of Oral and Maxillofacial SurgeryTechnical aspects in endoscopic biopsy of lesions in esophageal pemphigus vulgaris
2007, Digestive and Liver DiseaseCitation Excerpt :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.