Discussion
The evolution of PV typically begins with painful mucosal ulceration, especially in the mouth. These ulcers may be persistent or intermittent, but the new lesion will continue to appear. Many patients will develop skin lesions over the following weeks or months with an average of 5 months. Pemphigus vulgaris can affect any site of oral mucosa which gingival papillary tips are common. White patches of hyperkeratosis can be seen after PV heals.4 In fact, esophageal involvement has a relatively high incidence ranging from 46-87%, but EGD was done infrequently enough to detect.5Esophageal involvement in pemphigus vulgaris usually presents with throat pain, dysphagia, odynophagia or even bleeding. However, patient with oropharyngeal and upper esophageal involvement presented with an epigastric pain is very uncommon.
Espana et. Al.2 found that PV patients usually have active lesions in both pharyngeal and laryngeal mucosa leading to throat, pharyngeal or laryngeal symptoms.
Nakamura et al.6found that esophageal involvements of PV include blisters, erosions, erythema, scattered red spots and longitudinal erythematous lines with positive Nikolsky sign, mostly in the upper esophagus. The distribution of lesions can be whole esophagus with more severity in proximal than distal or more rarely isolated lesion.
In our patient, she presented with epigastric and neck pain, which might not be a straightforward manifestation of PV, but can be explained by her oropharyngeal involvement. Her isolate esophageal ulcer found on EGD with benign biopsy initially didn’t lead us to the diagnosis but with retroflexion of EGD, the inflammation of pharynx helped further identify the clues to diagnosis. In addition, the friable ulcer with bleeding as seen here could also be considered as a positive Nikolsky sign. Early diagnosis of the disease becomes very important as the disorder can be life-threatening. Once a diagnosis is made, treatment should be initiated.
Management of PV is considered in two main phases: remission induction (to achieve disease control with no new lesion formation and existing ones to be healed) and remission maintenance (disease-free for at least 2 weeks). First-treatment includes corticosteroid, usually in form of oral prednisolone (0.5-1 mg/kg/day) and start tapering at the end of remission maintenance. It is also thought to lower dosing and duration of steroid therapy in patients which allows for lesser risk of adverse side effects of therapy Second-line treatment indicated if treatment failure occurs and it includes azathioprine, mycophenolate mofetil, cyclophosphamide or methotrexate. Rituximab is recently approved as a third line treatment.4