Esophageal Involvement as a First Clinical Presentation in a Patient with Newly Diagnosed Pemphigus Vulgaris and a Review of Literature

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Purpose: Sir William Osler once famously said that the mouth is the window to the body. Fittingly enough, many systemic diseases have oropharyngeal manifestations, and pemphigus vulgaris is no exception. However, esophageal involvement in pemphigus is a rare manifestation, and to date, this has only been described in small case series and case reports. However, it is an important clinical presentation that physicians have to be aware of, as timely intervention and appropriate therapy can alter clinical outcome in such patients. Here, we report a 57-year-old woman of Malay descent, who presented with odynophagia and few oral lesions with no cutane-ous or pharyngeal involvement, and was found to have newly-diagnosed pemphigus vulgaris after undergoing a diagnostic esophagogastroduodenoscopy (OGD). She was initially treated for presumed oropharyngeal candidiasis, and failed to improve with a one-week course of oral fluconazole. Diagnostic OGD showed the presence of characteristic bullous blisters in the esophagus, commonly seen in pemphigus vulgaris. She then underwent a biopsy of her lip ulcers, which confirmed the diagnosis. The patient was started on a course of oral prednisolone with marked improvement of her symptoms. To date, she remains well. By highlighting this unusual presentation, we hope to draw attention to the rarer presentations of pemphigus so that these manifestations can be recognized early and uneccessary investigations, treatment and costs can be avoided.