# First Case of Esophagitis Dissecans Superficialis in an HIV Patient: A Case Report and Literature Review

Authors: Thaninee Prasoppokakorn; Palakorn Panarat
Journal: Case Reports in Infectious Diseases
 (2019)

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## Abstract

Esophagitis dissecans superficialis (EDS), a rare desquamative esophageal disease of uncertain etiology, is characterized by sloughing of fragments of esophageal mucosa. To the best of our knowledge, there has been no reported case of EDS in an HIV-infected patient. We report the first case of EDS in an adult HIV-infected male, who was hospitalized due to dysphagia. Esophagogastroscopy was performed, and the endoscopic findings together with the histopathologic findings of esophageal tissues were consistent with EDS. However, his symptom of dysphagia was not explained by EDS, but was the early symptoms of muscle-specific kinase (MuSK) myasthenia gravis (MG) that we finally diagnosed later by progression of the symptoms and electrophysiologic study. His symptoms had gradually improved after a course of intravenous immunoglobulin treatment. This is the first case of EDS and MuSK myasthenia gravis in an HIV-infected patient. A high index of suspicion of EDS should be made when taking care of the patients with desquamative or sloughing esophagitis especially with unknown etiology.


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## Body

## 1. Introduction

Esophagitis dissecans superficialis (EDS), a very rare benign desquamative esophageal disease of uncertain etiology, is characterized by sloughing of large fragments of the esophageal squamous mucosa, followed by vomiting or regurgitation of the esophageal cast [1, 2]. To date, there have been a few reported cases of EDS in the published literature; hence, accurate characterization of clinical features, etiology, pathologic findings, and outcomes are lacking [2]. We report herein the first human immunodeficiency virus- (HIV-) infected case with EDS and review all EDS case reports in the English literature.


## 2. Case Report

A 50-year-old male was hospitalized at Queen Savang Vadhana Memorial Hospital, Chonburi, Thailand, due to a 3-week course of oropharyngeal dysphagia. He had been diagnosed with HIV infection with a CD4 cell count of 64 cells/mL (8%) 7 years prior, when he presented with a 2-month history of chronic productive cough and significant weight loss. He received a combination treatment of tenofovir, emtricitabine, and efavirenz. Four months prior to the admission, he has achieved viral suppression with a CD4 count of 248 cells/mL (21%). Physical examination revealed neither oral candidiasis nor other intraoral lesions. Esophagogastroscopy was performed and revealed diffuse pseudomembranes in the lower one-third of the esophagus (Figure1). The histopathologic findings of the biopsied esophageal mucosa exhibited mild acute and chronic esophagitis with focal squamous hyperplasia without demonstrated organisms. A presumptive diagnosis of candidal esophagitis was made, and fluconazole of 200 mg/day was prescribed to the patient. No clinical improvement was observed at 3 weeks after the start of antifungal treatment. Fluconazole dose was increased to 400 mg/day. Three weeks after high-dose fluconazole treatment, he was rehospitalized due to worsening condition and development of hypovolemic hyponatremia. Esophagogastroscopy was done once again, which revealed circumferential white exudates with focal areas of erosions at the lower one-third of the esophagus without luminal stenosis or obstruction. The pathologic findings exhibited foci of splitting of the squamous epithelium with few intraepithelial cystic degenerations, no parakeratosis, no basal cell hyperplasia, mild acute and chronic inflammatory infiltrates with focal squamous cell hyperplasia, and normal underlying mucosa, which were similar to the previous findings (Figure 2). A diagnosis of EDS was made, and omeprazole of 80 mg/day was given to the patient. The patient’s symptoms gradually improved after 2 weeks of treatment. During hospitalization, he developed binocular diplopia, proximal muscle weakness, and ventilatory failure requiring ventilator support. Myasthenia gravis was suspected. The diagnosis was confirmed by an electrophysiologic study and positive result of muscle-specific tyrosine kinase (muscle-specific kinase, MuSK). The patient gradually improved after a course of intravenous immunoglobulin treatment.Figure 1
Esophagogastroscopy revealed diffuse pseudomembranes in the lower one-third esophagus.Figure 2
Pathologic findings of the lower esophagus revealed squamous cell hyperplasia (a), mild acute and chronic inflammatory infiltrations (b), a few small foci of splitting of the squamous epithelium (c), and a few intraepithelial cystic degenerations (d).
(a)
(b)
(c)
(d)


## 3. Discussion

To the best of our knowledge, this is the first case of EDS in an HIV-infected patient. EDS is a rare self-limited desquamative esophagitis of unknown etiology, usually affects adults after the age of 50 [1]. It is likely underestimated and frequently misdiagnosed as in our case. The diagnostic criteria of EDS have not been established yet; however, the endoscopic and pathologic criteria were recently proposed by Hart et al. including the presence of all of the following features: (1) the strip(s) of sloughed esophageal mucosa of >2 cm in length, (2) the normal underlying mucosa, and (3) the lack of ulcerations or friability of immediately adjacent mucosa [2].The etiology of EDS is unknown. There have been many reports demonstrating the association of EDS with (1) drugs including nonsteroidal anti-inflammatory drugs [1, 3, 4], bisphosphonates [5], potassium chloride [6], and clindamycin [7]; (2) celiac disease; and (3) autoimmune bullous dermatoses [6]. To date, there has been no report about the association of EDS and HIV infection, as in our case. Interestingly, due to lack of similar cases, the association between antiretroviral drugs and EDS required further studies. Similarly, the association between EDS and other immunodeficiency conditions has not been described. As a result, it is uncertain whether the HIV virus itself plays a role in the development of EDS. Furthermore, the association between EDS and MG also has never been reported. The presenting symptoms vary in different reports including dysphagia, heartburn, regurgitation, dyspepsia, upper gastrointestinal bleeding, anemia, and weight loss [2]. EDS is a benign condition that can resolve spontaneously or with the treatment with a proton-pump inhibitor (PPI) [1, 4]. Our patient had protracted course of dysphagia for 6 weeks before clinical improvement with PPI treatment.In addition, we review all 30 adult patients (including our case) with EDS [1, 4, 5, 7–11] (Table 1). There were 15 males and 15 females with the mean age of 65.8 ± 14.9 (range: 42–88) years. Our case is the first case with HIV infection. Regarding the race, there were 1 Asian (our case) and 28 Caucasians. The presenting symptoms included dysphagia (12 patients, 40.0%), upper gastrointestinal bleeding (4, 13.3%), epigastric pain (4, 13.3%), heartburn (3, 10.0%), weight loss (3, 10.0%), anemia (2, 6.7%), regurgitation (1, 3.3%), and atypical chest pain (1, 3.3%). Of 30 patients undergoing endoscopy, 21 (70.0%) patients had the characteristic features of EDS (the distinct features of denuded mucosal lining or peeling mucosa of the distal esophagus). Of 30 patients, the histology of the esophageal biopsy exhibited the sloughing of superficial layer of epithelium (30 patients, 100.0%), the presence of eosinophils on the surface (4, 13.3%), parakeratosis (17, 56.7%), and normal underlying mucosa (2, 6.7%). Of 30 patients with available data, all with (21 patients, 70.0%) and without PPI treatment (9, 30.0%) had good outcome with both clinical and endoscopic improvement.Table 1
A summary of all 30 cases with esophagitis dissecans superficialis (EDS).
Patient
Gender/age (year)
Race
Presentation
Diagnosis and associated condition
Endoscopic finding
Histology
Treatment
Outcome
1 [8]
F/81
Canadian
Chronic intermittent dysphagia
No significance
A thin white membrane lifting from mucosa
Mild candidal esophagitis; splitting of squamous mucosa at suprabasal and superficial layers; focal lymphocytic infiltration at deepest surfaces and diffuse parakeratosis
Double dose of PPI
Improvement
2 [9]
M/51
NA
Intermittent heartburn, anorexia, and epigastric pain without dysphagia
NSAID use
A thin white membrane lifting from mucosa with necrotic zones located on the lower esophagus
Detached superficial strips of squamous epithelium; prominent parakeratosis; acute inflammation and spongiosis in deep layers of squamous epithelium
Double dose of PPI
Endoscopic resolution 2 weeks after treatment
3 [10]
F/76
Belgian
Weight loss and nausea
COPD, CABG, hypertension
Fibrotic and sclerotic aspect of distal third of the esophagus
Sloughing of superficial layer of esophageal epithelium; presence of eosinophils on surface
No
Improvement
4 [10]
F/70
Belgian
Epigastric pain
Gastric bypass
White pseudomembranes of mid and distal third of the esophagus
Sloughing of superficial layer of esophageal epithelium; presence of PMN on surface separating it from basal layer
No
NA
5 [10]
F/71
Belgian
Epigastric pain
Arthrodesis L5-S1
Elevated lesion of distal third of the esophagus
Sloughing of superficial layer of esophageal epithelium; presence of eosinophils on surface
No
Improvement
6 [10]
F/47
Belgian
Dysphagia
Gastrectomy with eso-jejunal anastomosis, hypothyroidism, autoimmune pancreatitis
Sloughed esophageal mucosa in the esophagus
Sloughing of superficial layer of esophageal epithelium; presence of a few bacterial colonies on surface
No
Improvement
7 [10]
F/77
Belgian
Epigastric pain
Hypertension, hypercholesterolemia, hepatitis C, OSA
Sloughing of esophageal mucosa in the distal two-third esophagus
Sloughing of superficial layer of esophageal epithelium separated from normal basal layer
No
Improvement
8 [10]
M/86
Belgian
Atypical chest pain
Hypertension, hyperuricemia
Grade A reflux esophagitis
Sloughing of superficial layer of esophageal epithelium separated from normal basal layer
No
NA
9 [10]
F/83
Belgian
Dysphagia
Hypertension hypothyroidism, Alzheimer disease, DVT
Suspicion of an esophageal tumor from 16 to 24 cm of dental arch, circumferential from 18 to 20 cm
Sloughing of superficial layer of esophageal epithelium, presence of PMN on surface separating it from basal layer
No
Improvement
10 [7]
F/42
Portuguese
Dysphagia
Type 1 diabetes mellitus, clindamycin treatment for diabetic foot
Sloughing reddish membranes in the distal esophagus, adjacent to intact easily removed mucosa
Squamous epithelium detached from underlying basal layer; preservation of remaining layers; mild parakeratosis; severe acute inflammation with some eosinophils
Double dose of PPI
Improvement
11 [5]
F/59
USA
Dysphagia and odynophagia
Hypersensitivity pneumonitis, bisphosphonate
Sloughing whitish membranes that were easily removed, adjacent to intact healthy mucosa
Parakeratosis and desquamation of epithelial layer
Bisphosphonate discontinuation
Improvement
12 [4]
M/46
Caucasian
Nausea, vomiting, regurgitation
Hypertension, NSAID use
Distinct features of denuded mucosal lining, stripped off mucosa, long mucosal breaks, linear furrows, and vertical fissures
Fragments of superficial epithelium, parakeratosis; coagulative necrosis with epithelial sloughing
Double dose of PPI; NSAID discontinuation
Improvement
13 [4]
F/42
Caucasian
Dysphagia, regurgitation
GERD
Distinct features of denuded mucosal lining, stripped off mucosa, long mucosal breaks, linear furrows, and vertical fissures
Fragments of superficial epithelium, parakeratosis; coagulative necrosis with epithelial sloughing
Single dose of PPI
Improvement
14 [4]
F/79
African American
Dysphagia, nausea, vomiting
GERD, bullous pemphigoid, diabetes
Distinct features of denuded mucosal lining, stripped off mucosa, long mucosal breaks, linear furrows, and vertical fissures
Fragments of superficial epithelium, parakeratosis; coagulative necrosis with epithelial sloughing
Single dose of PPI
Improvement
15 [4]
F/46
African American
Dysphagia, dyspepsia
GERD, hypertension, diabetes
Distinct features of denuded mucosal lining, stripped off mucosa, long mucosal breaks, linear furrows, vertical fissures
Fragments of superficial epithelium, parakeratosis; coagulative necrosis with epithelial sloughing
Double dose of PPI
Improvement
16 [4]
F/55
Hispanic
Heartburn
GERD, hypertension, NSAID use
Distinct features of denuded mucosal lining, stripped off mucosa, long mucosal breaks, linear furrows, and vertical fissures
Fragments of superficial epithelium, parakeratosis; coagulative necrosis with epithelial sloughing
Single dose of PPI; NSAID discontinuation
Improvement
17 [1]
M/61
White
Dysphagia
NA
Diffuse erythema, patches of peeling mucosa in the mid to distal esophagus
Parakeratosis; fragments of necrotic epithelium with minimal inflammation; intraepithelial splitting at varying degrees above basal layer
Double dose of PPI
Improvement
18 [1]
M/81
African American
UGIB
NA
Multiple patches of peeling mucosa
Parakeratosis; fragments of necrotic epithelium with minimal inflammation; intraepithelial splitting at varying degrees above basal layer
Double dose of PPI
Improvement
19 [1]
M/77
African American
Weight loss
NA
Distal 2 cm patch of peeling mucosa
Parakeratosis; fragments of necrotic epithelium with minimal inflammation; intraepithelial splitting at varying degrees above basal layer
Double dose of PPI
NA
20 [1]
M/65
White
Dysphagia
NA
Grade 4 esophagitis
Parakeratosis; fragments of necrotic epithelium with minimal inflammation; intraepithelial splitting at varying degrees above basal layer
Double dose of PPI
Improvement
21 [1]
M/60
African American
UGIB
NA
Grade 3 esophagitis from the mid to distal esophagus
Parakeratosis; fragments of necrotic epithelium with minimal inflammation; intraepithelial splitting at varying degrees above basal layer
Double dose of PPI
Improvement
22 [1]
M/57
African American
Epigastric pain
NA
Distal multiple patches of peeling mucosa
Parakeratosis; fragments of necrotic epithelium with minimal inflammation; intraepithelial splitting at varying degrees above basal layer
Double dose of PPI
NA
23 [1]
F/49
White
Heartburn
NA
Mid to distal grade 3 esophagitis, achalasia
Parakeratosis; fragments of necrotic epithelium with minimal inflammation; intraepithelial splitting at varying degrees above basal layer
Double dose of PPI
Improvement
24 [1]
M/65
White
Anemia
NA
Mid to distal grade 4 esophagitis
Parakeratosis; fragments of necrotic epithelium with minimal inflammation; intraepithelial splitting at varying degrees above basal layer
Double dose of PPI
NA
25 [1]
M/80
White
Iron deficiency anemia
NA
Mid to distal grade 4 esophagitis
Parakeratosis; fragments of necrotic epithelium with minimal inflammation; intraepithelial splitting at varying degrees above basal layer
Double dose of PPI
NA
26 [1]
M/84
White
UGIB
NA
Sloughing of entire esophagus
Parakeratosis; fragments of necrotic epithelium with minimal inflammation; intraepithelial splitting at varying degrees above basal layer
Double dose of PPI
NA
27 [1]
M/63
White
Dysphagia
NA
Multiple patches of peeling mucosa in the mid to distal esophagus
Parakeratosis; fragments of necrotic epithelium with minimal inflammation; intraepithelial splitting at varying degrees above basal layer
Double dose of PPI
NA
28 [1]
M/84
White
Weight loss
NA
Distal adherent yellow patches of peeling mucosa
Parakeratosis; fragments of necrotic epithelium with minimal inflammation; intraepithelial splitting at varying degrees above basal layer
Double dose of PPI
NA
29 [11]
F/88
USA
UGIB
Atrial fibrillation, mitral regurgitation, post-cardiac arrest syndrome
White, linear, desquamating plaques in the distal esophagus
Sloughing of superficial layer of esophageal epithelium; presence of eosinophils on surface; parakeratosis
No
Improvement
30 (our case)
M/50
Thai
Dysphagia
HIV infection, myasthenia gravis
Circumferential white exudates with focal areas of erosions at the lower one-third esophagus without luminal stenosis or obstruction
Foci of splitting of the squamous epithelium with few intraepithelial cystic degeneration; no parakeratosis; no basal cell hyperplasia; mild acute and chronic inflammatory infiltrates with focal squamous cell hyperplasia; and normal underlying mucosa
Double dose of PPI
Improvement
COPD: chronic obstructive pulmonary disease; CABG: coronary artery bypass graft; OSA: obstructive sleep apnea; DVT: deep vein thrombosis; UGIB: upper gastrointestinal bleeding; PMN: polymorphonuclear cell; PPI: proton-pump inhibitor; NSAID: nonsteroidal anti-inflammatory drug.


## 4. Conclusion

To the best of our knowledge, this is the first case of EDS in an HIV-infected patient. Due to lack of similar cases, the association between immunodeficiency conditions including antiretroviral drugs and EDS required further studies. EDS is a rare self-limited desquamative esophagitis of unknown etiology, which is diagnosed by excluding all other possible causes of oropharyngeal dysphagia. A high index of suspicion of EDS should be made when taking care of patients with desquamative or sloughing esophagitis especially with unknown etiology and/or no response to antifungal treatment.



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