Esophageal Involvement of Pemphigus Vulgaris

Pemphigus is an autoimmune and life-threatening disease. Esophageal involvement of pemphigus vulgaris is rare. The most common presenting symptoms are odynophagia and dysphagia. Here, I present one cases of pemphigus vulgaris presenting with dysphagia because of esophageal involvement of the disease. In case, a 27-year-old female patient with a prior diagnosis of pemphigus vulgaris presented with dysphagia. Esophagogastroduodenoscopy showed diffuse mucosal exfoliation and oozing bleeding of the esophagus. The patient recovered after the administration of corticosteroids, pantoprazole and azathioprurine. © 2020 Vedat Goral. Hosting by Science Repository. All rights reserved


Introduction
Pemphigus is an autoimmune and life-threatening disease characterized by acantholysis (loss of keratinocyte-keratinocyte adhesion) caused by acantholysis (loss of keratinocyte-keratinocyte adhesion) caused by the mucous membrane and skin, directed against keratinocyte cell surface [1]. The main subgroups of Pemphigus are Pemphigus Vulgaris (PV), Pemphigus Foliaseus and Paraneoplastic Pemphigus. The most common subtype of pemphigus is PV [2][3][4][5]. PV is seen equally in both sexes. Although it differs geographically, its incidence varies between 0.1 and 0.5 per 100.000 per year [6][7][8][9][10]. In almost all cases during the course of the disease, the multi-layer flat epithelial mucosa, especially the oral mucosa, is involved. Pharyngolaryngeal, conjunctival, genital, anorectal mucosa are less often involved.
Odynophagia and dysphagia are the most common symptoms of esophageal involvement. However, patients with esophageal involvement can sometimes be asymptomatic. It is thought that many esophageal involvement cannot be detected because endoscopy is not performed mostly in patients without esophageal complaints. Some immunohistopathological studies show that esophageal involvement is higher than previous reports [2][3][4].
Here, I present one cases of pemphigus vulgaris presenting with dysphagia because of esophageal involvement of the disease. In case , a 27-year-old female patient with a prior diagnosis of pemphigus vulgaris presented with dysphagia. Esophagogastroduodenoscopy showed diffuse mucosal exfoliation and oozing bleeding of the esophagus. The patient recovered after the administration of corticosteroids, pantoprazole and azathioprurine.

Case Report
A 32-year-old woman complained about difficulty swallowing and pain in her chest about 3 months ago. The patient applied to our gastroenterology outpatient clinic with his current complaints and an upper gastrointestinal system endoscopy was performed. In the esophageal mucosa, an appearance that started around 20 cm, was circular, widespread, erosional, hemorrhagic and slightly narrowing in the lower region. Two biopsies were taken from here. The esophageal lumen was naturally observed. The Z line was crossed at 40.cm from the front incisors. At the pathology report, although no specific findings are seen in the DIF examination, it is rare with focal C3 accumulation and immunohistochemical examination. C4d positivity suggests an immunological mechanism. Intraepidermal suprabasal dissociation supports the diagnosis of pemphigus vulgaris.   A biopsy was taken by monitoring vesicle-nodular lesions in the midesophagus (Figure 1a & 1b). Starting from the biopsy site, it was observed that the esophageal mucosa was separated from the underlying tissue enveloping the esophageal lumen all around (Figure 2). Pathology report: Although no specific findings are seen in the DIF examination, it is seen rare with focal C3 accumulation and immunohistochemical examination. C4d positivity suggests an immunological mechanism. Intraepidermal suprabasal dissociation evaluated in favor of pemhigus vulgaris.

Discussion
The incidence of PV varies from 0.5 to 3.2 cases per 100,000, with an approximately equal male-to-female ratio. The patient was started on proton pump inhibitor and sucralfate therapy. Steroid and azathiopurine treatment was started with the recommendation of the dermatology clinic. The patient's complaints of swallowing difficulties disappeared, the patient is under pemphigus and his treatment is still ongoing. The patient recovered after the administration of corticosteroids, pantoprazole and azathioprurine.
As conclusion; pemphigus is an autoimmune and life-threatening disease. Esophageal involvement of pemphigus vulgaris is rare. The most common presenting symptoms are odynophagia and dysphagia. If the patient with pemphigus has dysphagia, endoscopy should be done.