Case Report

Esophageal Pemphigus Vulgaris: A Rare Etiology of Upper Gastrointestinal Hemorrhage

Table 1

Studies with esophageal involvement of pemphigus vulgaris associated with upper gastrointestinal bleeding.

StudyYearAge (year)/sexPresentationPreexisting PVEndoscopic findingsDIFIIFTreatment

Wood et al. [6]198256/FHematemesisYes, both cutaneous and mucosalMultiple circumferential erythematous and granularity of lower end of the esophagus and area of discrete ulceration above the EGJPositiveNegativeSucralfate and antacid
Venkataram et al. [7]200126/FHematemesis and melenaNoFriable mucosa with erythema and linearmucosal ulcersNANegativePrednisolone, cyclophosphamide, and antacids
Tageja et al. [8]201069/FHematemesis, peptic ulcer diseaseNoMultiple hemorrhagic bullae in the midesophagusNANAPrednisone
Mohan et al. [9]201348/FHematemesisYes, both cutaneous and mucosal lesionsOropharyngeal ulcers and mucosal desquamationNANASteroids and PPI
Chang et al. [2]201441/F
30/F
HematemesisYes, cutaneous PVMucosal edema and erythema on the esophagus and larynx with desquamation. Upper esophageal sphincter to lower esophagus with diffuse exfoliation of mucosa with multiple linear ulcer and erosions.NANAIV hydrocortisone and mycophenolate mofetil
Del castillo et al. (present case)202085/FHematemesisYesMultiple shallow ulcers and grouped blisters throughout the proximal and midesophageal mucosa.NANASystemic steroids and proton pump inhibitor

PV, pemphigus vulgaris; DIF, direct immunofluorescence; IIF, indirect immunofluorescence; NA, not available; PPI, proton pump inhibitors.