Oesophageal pemphigoid: a rare cause of dysphagia

Abstract

Pemphigus vulgaris (PV) is a rare autoimmune bullous disease which affects the skin and mucous membranes. Oesophageal involvement is rare and has previously been limited to case reports and case series. A recent large case series of 477 PV patients showed that 26/477 (5.4%) had symptomatic oesophageal involvement. We present the case of a 54-year-old Somalian lady with a 10-year history of cutaneous PV, currently in remission, who developed dysphagia and odynophagia and was subsequently found to have oesophageal PV involvement with multiple flaccid bullae which were positive for anti-DSG3 antibodies on in-direct immunofluorescence. She had her treatment switched from azathioprine to mycophenolate and prednisolone, leading to resolution of her symptoms.

Esophageal Nikolsky’s Sign: A Rare Finding in a Patient with Bullous Pemphigoid

“…Patients with BP have increased risk of mortality compared to the general population [15]. The involvement of the esophagus in BP is very rare, with a few cases reported in the literature [6, 7, 8, 9, 10, 11, 12, 13]. The esophageal manifestations of BP are often nonspecific, and the clinical presentation may be dysphagia, hematemesis, melena, or hemodynamic instability due to brisk upper gastrointestinal bleeding.…”

“…Antigens identified in MMP include BP180, BP230, Laminin 5/6, Type VII collagen, and Integrin 4 subunit [2, 3, 5], whereas only BP180 and BP230 are involved in BP [2]. Esophageal involvement in BP is quite rare and generally manifests as hemorrhagic bullae [1, 6, 7, 8, 9, 10, 11, 12, 13]. We report a case of acute-onset esophageal bullae identified in a patient with active skin BP seen only upon withdrawal of the upper endoscope, not present on insertion.…”

Novel and rare forms of oesophagitis

“…88,112,113 Endoscopic examination classically reveals multiple flaccid bullae, which may or may not contain haemorrhagic contents. 91 Endoscopic findings may also be consistent with ODS and include separation or sloughing of the mucosa with inflammation. 112,113 Biopsy of the oesophageal lesion may be inadequate for diagnosis if there is significant separation of the epithelium, leaving only superficial detached fragments for evaluation.…”

“…90 Pemphigus vulgaris involving the oesophagus may also show desquamation and is distinguished from ODS by the presence of inflammation and suprabasal acantholysis with a clefting 'tombstone' appearance. 91,92 A band-like lymphocytic infiltrate along the basal layer of the mucosa with parakeratosis and apoptotic keratinocytes is highly suggestive of lichen planus oesophagitis. 66 Candidal oesophagitis may also present with desquamated epithelium but is typically associated with abundant neutrophils and lymphocytes, along with fungal forms.…”

“…91,112 The most common presenting symptoms are dysphagia and odynophagia. 91,92 Patients may also present with upper gastrointestinal tract haemorrhage 112,113 and ODS. 88,112,113 Endoscopic examination classically reveals multiple flaccid bullae, which may or may not contain haemorrhagic contents.…”