Esophagitis dissecans superficialis: a rare cause of upper GI bleeding

Abstract

Background

Esophagitis dissecans superficialis (EDS) is a rare desquamative esophageal condition with a wide range of presentations, from asymptomatic cases to those with severe, life-threatening symptoms. EDS has a strong association with several conditions, such as autoimmune diseases, smoking, and certain medications, although it can also occur without any apparent cause (idiopathic). Diagnosis involves a comprehensive approach, including clinical assessment (history and examination), endoscopic examination, histopathological analysis, and the exclusion of other potential causes. There is still no established standard treatment for EDS, as many cases resolve spontaneously without long-term complications.

Case presentation.

We present a case of a 73-year-old female with an episode of upper gastrointestinal bleeding (UGIB) due to Esophagitis dissecans superficialis, which had a favorable outcome after therapy with a proton pump inhibitor (PPI).

Conclusion

Although EDS is a rare condition, it should be considered a cause of UGIB in patients with risk factors.

Introduction

Formerly known as sloughing esophagitis, Esophagitis dissecans superficialis (EDS) is an exceedingly rare and benign desquamative esophageal ailment of uncertain origin. It is identified by the expulsion of substantial sections of the esophageal squamous mucosa, frequently leading to symptoms such as vomiting or the regurgitation of the esophageal cast [1, 2].

The exact cause of EDS is still not fully understood. While certain instances have no identifiable cause (idiopathic), others are associated with factors like mucosal irritants such as medications, smoking, alcohol, hot beverages, and bullous skin conditions [3, 4]. The symptoms of EDS vary greatly, ranging from minor incidental findings to significant symptomatic issues related to the esophagus [5]. Diagnosing EDS is often missed, with histopathology commonly overlooked due to contamination of samples [6].

To date, only a few cases of Esophagitis dissecans superficialis (EDS) have been reported in the published literature [7]. Here, we present the case of a 73-year-old British female patient who experienced recurrent episodes of hematemesis and melena.

Case presentation

A 73-year-old female patient from the UK presented to the emergency room (ER) with an episode of dark, altered hematemesis and melena. She has hypertension controlled with calcium channel blockers, drinks alcohol (2 g/day), and smokes approximately 20 cigarettes per day. She reported several episodes of melena 10 years ago that were not investigated. The patient was admitted to the hospital, and a full lab workup was performed. She had no diarrhea, dysphagia, odynophagia, chest pain, or dyspnea, and no prior abdominal surgery in recent years.

Initial vital signs were stable. Physical examination revealed only mild dehydration and moderate epigastric tenderness. Her labs were normal, including blood urea, creatinine, complete blood count, liver panel, serum lactate, and coagulation profile. Tests for hepatitis B, C, and HIV were negative. A non-contrast computed tomography (CT) scan of the abdomen and pelvis was unrevealing. The patient’s dehydration resolved with intravenous (IV) fluid rehydration.

Esophagogastroscopy revealed circumferential white exudates with focal areas of erosion in the lower one-third of the esophagus without luminal stenosis or obstruction (Fig. 1). Pathologic findings showed widely ulcerated, focally desquamated esophageal mucosa with neutrophilic exudation and fibrin deposition. No metaplasia, dysplasia, or malignancy was detected (Fig. 2). A diagnosis of Esophagitis dissecans superficialis (EDS) was made, and the patient was started on omeprazole 80 mg/day.

Fig. 1

Esophagogastroscopy revealed circumferential white exudates with focal areas of erosions at the lower one-third of the esophagus

Fig. 2

Photomicrographs showing esophageal mucosal near total ulceration, fibrinous deposits, and neutrophilic exudation (arrows) with desquamated/sloughed epithelial cells (c) (H&E stain original magnification × 40, × 200, × 200, respectively)

Discussion

Desquamative esophagitis (EDS) is a rare, self-limiting, and benign condition characterized by the shedding of esophageal tissue, with its exact cause remaining unknown. Typically affecting adults, especially those aged 50 and above, it has been linked to certain medications like nonsteroidal anti-inflammatory drugs (NSAIDs), smoking, and autoimmune disorders. However, some cases have no discernible cause. While some patients may not show symptoms, others may suffer from severe epigastric pain, dysphagia, abdominal discomfort, nausea, and vomiting [1, 6, 8,9,10,11,12]. There have even been some reported cases of patients coughing out large chunks of sloughed mucosa [13].

The presenting symptoms of EDS vary across different reports and may include dysphagia, heartburn, regurgitation, dyspepsia, upper gastrointestinal bleeding, anemia, and weight loss [2].

EDS is often underestimated and commonly misdiagnosed, as evidenced in our case. Although there are no officially established diagnostic criteria for EDS, recent suggestions by Hart et al. propose the presence of the following features: (1) strips of sloughed esophageal mucosa exceeding 2 cm in length, (2) intact underlying mucosa, and (3) the absence of ulcerations or friability in the surrounding mucosa [2].

EDS is generally regarded as a benign condition that can either resolve on its own or show improvement with treatment using a proton pump inhibitor (PPI) [1, 4]. In a retrospective study analyzing 21,497 esophagogastroduodenoscopy (EGD) cases, the reported incidence of EDS was 0.03% [14, 15]. The infrequency of EDS, along with the common occurrence of biopsy contamination with bacteria and fungi, has resulted in frequent misdiagnosis and underreporting [4, 16, 17].

Currently, there is no universally standardized therapy for EDS, given that many cases resolve spontaneously without leading to long-term complications. However, in cases similar to ours, where modifications targeting underlying EDS risk factors were combined with high-dose PPI therapy, favorable outcomes were noted [6]. Nonetheless, it is worth noting that PPIs are thought to mitigate additional injury rather than addressing the underlying cause of EDS [18].

There is speculation that EDS related to autoimmune factors might exhibit a positive response to steroid treatment, and there have been reports of idiopathic EDS cases showing favorable outcomes with steroid therapy [13]. However, it is important to note that while some authors recommend a follow-up endoscopy after 8 weeks, there is currently insufficient evidence to support its overall benefit [17]. Additionally, smoking and alcohol are considered risk factors for EDS, so our patient was advised to stop smoking and drinking alcohol [13].

Following the administration of high-dose pantoprazole and discontinuation of oral diclofenac, our patient experienced a complete resolution of symptoms and was discharged in good health.

Conclusion

This case highlights a less frequent and more challenging-to-identify cause of gastrointestinal bleeding. EDS continues to be a sporadic diagnosis that can significantly impact the patient’s quality of life. When managing patients with desquamative or sloughing esophagitis, particularly in cases with an unknown etiology and/or no response to antifungal treatment, a high index of suspicion for EDS should be maintained.