Article, Gastroenterology

Esophageal dissecans: a rare life-threatening presentation of recurrent pemphigus vulgaris

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American Journal of Emergency Medicine

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Esophageal dissecans: a rare life-threatening presentation of recurrent pemphigus vulgaris?,??

Abstract

Esophagitis dissecans superficialis (EDS) is a Rare condition character- ized by sloughing of the mucosal layer of the esophageal lining, usually triggered by an environmental/immune insult. It is associated with blistering diseases such as pemphigus vulgaris (PV), bullous pemphigoid, and epidermolysis bullosa. Oral mucosa is the most commonly (50%) affected site in PV, but esophageal involvement has only been rarely reported. The most common presentation of EDS includes dysphagia and odynophagia, but overt gastrointestinal bleeding is a distinctly uncommon. We present a unique case of EDS presenting with melena in which diagnostic endoscopy enabled to diagnose and establish link with patient’s remote history of PV. Early suspicion and identification of this treatable condition can reduce morbidity and mortality in these patient populations by decreasing severity and recurrence of bleeding.

Esophagitis dissecans superficialis (EDS) is a rare condition charac- terized by sloughing of the mucosal layer of the esophageal lining, usually triggered by an environmental/immune insult. It is associated with blistering diseases such as pemphigus vulgaris (PV), bullous pemphigoid, and epidermolysis bullosa. Overt gastrointestinal bleeding is a distinctly uncommon manifestation of EDS. We present a unique case of EDS presenting with melena in which diagnostic endoscopy en- abled to diagnose and establish link with patient’s remote history of PV. An 82-year-old man with history of diabetes mellitus, presented to the emergency department with syncope. He denied chest pain, dyspnea, new-onset weakness, Alcohol intake, or use of nonsteroidal anti-inflammatory drugs/anticoagulants. There was a remote history of pemphigus vulgaris treated with systemic steroids. At initial presen- tation, he was hypotensive (90/60), tachycardic, and afebrile. Systemic examination was fairly unremarkable except for melenic stools in rectum. Investigations revealed acute posthemorrhagic anemia with hemoglobin/hematocrit drop to 7.4 and 21 from baseline of 11.8/35, normal platelet count, and normal coagulation profile. Metabolic profile showed prerenal azotemia. He was resuscitated aggressively with intravenous fluids, proton pump inhibitor infusion, and multiple blood transfusions. An urgent endoscopy was then performed, which unexpectedly revealed extensive mucosal sloughing and ulceration with multifocal active ooze from deeper layers in the entire esophagus wall. Using facts from patient history and excluding other causes, these endoscopic findings were attributed to pemphigus, and the patient was given a diagnosis of esophagitis dessicans. In addition to aggressive medical therapy, he was initiated on steroids with gratifying

? Conflict of interest: The authors have no conflict of interest.

?? Funding: No external source of funding was acquired for this manuscript.

response over next few days. The biopsies further confirmed the etiolo- gy as pemphigoid involvement of esophagus.

Pemphigus vulgaris has the most notable association with EDS among the skin disorders. Oral mucosa is the most commonly (50%) affected site, but esophageal involvement has only been rarely reported. Other associ- ations such as celiac disease, alendronate therapy, trauma, and endoscop- ic sclerotherapy need to be ruled out. The pathogenesis of EDS appears to be immune mediated due to immunoglobulin G antibodies against the desmoglein 3 proteins, although rare variants of immunoglobulin A against desmoglein 1 [1] and Intercellular Adhesion Molecule 1 have also been reported.

The most common presentation of EDS include dysphagia (57.1%) and odynophagia (21.4%) [2]. Other manifestations include intractable heartburn, nausea, and vomiting, sometimes bringing out the entire esophageal mucosal cast. Esophagitis dissecans superficialis presenting as upper gastrointestinal bleeding has been reported rarely in small case series only [2]. Classically, Upper gastrointestinal endoscopy in EDS reveals a white plaque or a thin white membrane, which is seen distinctively in the esophagus as a cast [3].

The most common site of involvement is the distal esophagus followed by mid and proximal portions. Pan esophageal involvement like that of our patient is distinctly uncommon [4]. The histology reveals normal squamous epithelium, which is separated from the basal layer or the lamina propria with minimal inflammation and characteristic “tombstoning” of the basal layer [5]. Sometimes, it may also be associated with microabscesses around the esophageal papillary base [4]. Immunofluorescence reveals immuno- globulin G and C3 deposits in the epithelial layer of most of these patients with detached cells and intraepidermal edema.

Few reports classified EDS as a benign self-limiting phenomenon, but in most cases, it needs treatment, generally starting with steroids at 20 to 60 mg daily. Our patient was also started on prednisone treatment and had a favorable response. Other immunosuppressants such as azathio- prine and mycophenolate have been used in steroid-resistant cases [5].

Although rare, PV can involve the esophagus. While evaluating patient with suspected gastrointestinal bleeding with unidentified source, physicians should keep this association in mind. Identification and appropriate treatment of EDS can result in complete resolution.

Rushikesh Shah, MBBS? Viveksandeep Thoguluva, MBBS

Nidhi Bansal, MBBS Divey Manocha, MBBS

SUNY Upstate Medical University, Syracuse, NY 13202

?Corresponding author. Tel.: +1 9723753932

E-mail address: [email protected] http://dx.doi.org/10.1016/j.ajem.2015.04.053

0735-6757/(C) 2015

References

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