Regurgitation and prolapse of oesophageal mucosa: a dramatic presentation of the oesophagitis dissecans superficialis

Abstract

Oesophagitis dissecans superficialis is a rare benign entity that is usually self-limited, characterised by sloughing of the oesophageal mucosa. We preset a 38-year-old woman, known case of epidermolysis bullosa acquisita who presented to us with regurgitation and prolapse of the oesophageal mucosa from the mouth. Upper gastrointestinal endoscopy showed sloughing of the mucosa. The patient was managed conservatively and discharged.

Background

Oesophagitis dissecans superficialis (ODS) is a rare self-limiting benign disorder of the oesophagus characterised by sloughing of the oesophageal mucosa.^{1 2} It can occur following trauma, drug intake or autoimmune disorders. Patients usually present with coughing and vomiting of the oesophageal mucosa. Upper gastrointestinal endoscopy (UGIE) is usually diagnostic. Patients are usually managed conservatively. We present a 38-year-old woman who presented to us with prolapse and regurgitation of the oesophageal mucosa, an infrequent presentation of the ODS.

Case presentation

A woman in her 30s presented with multiple episodes of non-projectile vomiting associated with a minimal amount of blood, followed by prolapse of a long string-like tissue from the mouth. The patient was a known case of epidermolysis bullosa acquisita (EBA). On examination, a string of tissue of approximately 20 cm was seen hanging from the mouth (figure 1). Oral examination showed the presence of mild erythema and erosions over the lower lip. A string of tissue was originating behind the throat; however, the exact origin could not be made. There were multiple tiny bullous lesions and crusted lesions present all over the body suggestive of EBA (figure 2). Systemic examination was unremarkable.

Figure 1.

Sloughed oesophageal mucosa prolapsing from the mouth (arrow) originating from behind the tongue.

Figure 2.

Multiple tiny bullous and crusted lesions in the upper (A) and lower (B) limbs.

Blood investigations were within normal limits except for the mild anaemia. Indirect laryngoscopy showed a string of tissue was coming from the oesophagus; however, the exact origin could not be seen. We divided the tissue behind the tongue, and histopathological examination showed stratified squamous epithelium without any other specific features.

UGIE showed multiple translucent stripped-off oesophageal mucosa along with a few areas of oozing of blood. Multiple sloughed-off oesophageal mucosal strips were present, diagnostic of ODS. A string of the oesophageal mucosa, which had prolapsed out of the mouth, was found to be attached to the lower third oesophagus (figure 3). The patient was managed conservatively with proton-pump inhibitors (PPI) and analgesics. She was continued on oral dapsone and steroids for EBA.

Figure 3.

Upper gastrointestinal endoscopy showing (A) string of the sloughed mucosa (black arrow) originating from the oesophagus behind the epiglottis (yellow arrow), (B) sloughed-off oesophageal mucosa attached to the lower oesophagus (arrow), (C) sloughed-off strip of the oesophageal mucosa (yellow arrow).

Outcome and Follow-up

The patient was followed for the next 6 months. There was no recurrence of such episodes, and the patient was completely asymptomatic.

Discussion

ODS is a rare benign condition of the oesophagus characterised by the sloughing and desquamation of the superficial oesophageal mucosal lining.¹ It usually has a benign course and resolves without any significant problems. Previously, it was also called chronic oesophagitis dissecans or oesophagitis dissecans.²

ODS is commonly seen in middle-aged women. There are various aetiologies described for ODS. It can occur following oesophageal trauma, heavy smoking, medications causing oesophageal irritation such as bisphosphonates, doxycycline and ferrous sulfate.³ It is also known to occur in elderly patients with multiple comorbidities. There is an association of the ODS with autoimmune bullous skin disorders. However, exact pathogenesis is not known. These disorders include pemphigus vulgaris, pemphigoid, bullous systemic lupus erythematosus and epidermolysis bullosa. In the case described, the patient had skin lesions all over the body, diagnosed with EBA.^{1 4}

Most of the patients have associated skin disorders or autoimmune disorders. Clinical presentation varies from incidental endoscopic findings to significant disability. The patient may have dysphagia, odynophagia, vomiting and regurgitation. Few patients present with vomiting of strips of mucosa or coughing out sloughed mucosa. Our patient had a prolapse of the sloughed-off oesophageal mucosa from the mouth, causing significant discomfort.

UGIE may show single or multiple stripped-off translucent whitish oesophageal mucosa involving part or the whole oesophagus. Vertical strips of sloughing squamous mucosa may be seen giving an appearance of ‘gift wrap ribbons’.⁵ Patients with associated skin disorders may have erythema, erythematous longitudinal lines, blisters or ulcers.⁶ Other features include stripped mucosa with bleeding, mucosal breaks, exudative oesophagitis, peeling off the whitish mucosa.¹ Although prolapse of the sloughed-off oesophageal mucosa has never been reported in the literature, such a phenomenon was noticed in the present case.

Histopathology shows necrosis of the superficial layers of the oesophageal mucosa with separation of the underlying layers. Parakeratosis, basal cell hyperplasia, focal inflammation may also be seen.³ The presence of spongiosis is an infrequent finding. Few other findings described in ODS include bullae formation, a ‘tombstone appearance’, intraepithelial cysts, bacterial and fungal colonies, separation of the mucosa from the submucosa, microabscesses, pyknotic nuclei, detached strips of squamous epithelium and cell necrosis.⁷ Histopathology of the prolapsed tissue in the present case showed stratified squamous epithelium without any other specific features consistent with oesophageal mucosa.

There are a few conditions reported in the literature that may have similar endoscopic findings to ODS. These include drug-induced oesophagitis, fungal oesophagitis, mucosal trauma. Whitish exudates can be seen in fungal oesophagitis, which may be confused with sloughing oesophageal mucosa of ODS. Drug-induced oesophagitis may have erosions or ulcers and may mimic ODS.⁸ Hence, all the physicians and surgeons involved in endoscopic procedures should be aware of such rare presentation of the ODS.

Although it may have a drastic presentation, ODS usually has a self-limited course and good prognosis. PPIs are commonly used to reduce further injury from the acid to damaged mucosa. In patients who have autoimmune disorders, immunosuppressive drugs play a significant role in the management.³ We treated our patient with PPI and analgesics for ODS and dapsone and oral steroids for EBA.

Patient’s perspective.

In an emergency, I had come to the hospital with prolapse of regurgitation of the tissue from the mouth. I was initially told by treating doctors that the layer of my food pipe had regurgitated. I underwent scopy the next day, and I was informed by my doctors that it was a benign condition. I was evaluated by the dermatologist and was told to have some skin disorder for which medications were started. I thank my treating doctors for their kind help.

Learning points.

• This case highlights the drastic presentation of oesophagitis dissecans superficialis (ODS) as prolapse of the sloughed-off oesophageal mucosa. Such a presentation is rare, and all surgeons, endoscopists and physicians should be aware of it. All should have sound knowledge of the different conditions, like ODS.

• ODS is usually associated with an autoimmune skin disorder, and a detailed dermatological evaluation must be done.

• A detailed history of drug intake is also essential as ODS can occur due to drug intake.

• Endoscopy is diagnostic of the disease, and histopathology further strengthens the diagnosis.

Ethics statements

Patient consent for publication

Consent obtained directly from patient(s).