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