P045 A Case of Esophagitis Dissecans Superficiales in a Pediatric Patient With Crohn’s Disease During Treatment With Infliximab
Authors: Keeley Melissa , Cloney Deborah , Kammeraad Colin 
Published: December 2019

CASE:

Esophagitis Dissecans Superficiales (EDS) is a rarely reported condition in pediatrics and has never been reported in patients on anti-TNFα therapy before or in patients with Crohn’s disease. Additionally, there are limited documented cases of EDS occurring in the pediatric population. It is characterized by separation of the superficial epithelium from the underlying mucosa in the middle or distal esophagus. Patients are often asymptomatic, but may present with dysphagia, odynophagia, nausea, reflux, and/or regurgitation of esophageal casts. Diagnosis is made during esophageal endoscopy by the presence of sloughed esophageal mucosa greater than 2 cm in length, normal underlying esophageal mucosa, and lack of ulcerations or friability of immediately adjacent esophageal mucosa. Biopsied tissue typically reveals intraepithelial splitting above the basal layer, non-specific findings of parakeratosis, basal cell hyperplasia, occasional bullae, and minimal to no inflammatory changes. Bacterial or fungal colonization on necrotic tissue has also been documented. Little is known regarding the pathogenesis of this condition, however current literature including case reports and case series suggest a strong link with immunocompromised patients, general debility, use of five or more medications, use of certain medications (NSAIDs, bisphosphonates, SSRIs/SNRIs), and autoimmune disorders. This benign condition is often self-limited and most symptomatic cases resolve with use of high dose-PPIs or oral fluticasone. The following case further expands the list of potential causative factors for EDS and adds to the depth of reported pediatric cases. We present the case of a 17-year-old female with history of duodenal and ileocolonic Crohn’s disease on infliximab therapy who developed Esophagitis Dissecans Superficiales. Her Crohn’s symptoms were initially well-controlled with nutritional therapy, however, after an exacerbation she began treatment with 6-mercaptopurine and infliximab infusions. She again had well-controlled symptoms for approximately a year and a half. At that time, routine esophagogastroduodenoscopy and colonoscopy were performed to determine if there was adequate remission of her Crohn’s to discontinue 6-MP. There was no reported dysphagia, odynophagia, or reflux. EGD revealed sloughing esophageal mucosa. Biopsy from the distal esophagus showed non-keratinizing squamous epithelium with basal cell hyperplasia and increased intraepithelial lymphocytes. Biopsies from the proximal esophagus showed mild to moderate acute and chronic esophagitis, basal cell hyperplasia, necrosis of the surface squamous epithelium with colonies of actinomyces, and a small fragment of exudate suggestive of ulceration. These findings suggest a diagnosis of esophagitis dissecans superficiales in the distal esophagus. Colonoscopy was remarkable for only mild proctitis. The patient was started on a PPI and 6-MP was discontinued. Per the recommendation of our infectious disease colleagues, the patient began a course of Augmentin to treat the actinomyces colonization. The patient did not develop any esophageal symptoms following initiation of the PPI or discontinuation of 6-MP. A repeat EGD was not immediately performed as the patient was asymptomatic and she declined follow up EGD. However, EGD and colonscopy were repeated 2 years later due to abdominal pain and diarrhea. The esophagus was grossly and microscopically unremarkable with no further evidence of esophagitis dissecans superficiales.