Esophagitis dissecans superficialis

2009 Dec;33(12):1789-94. doi: 10.1097/PAS.0b013e3181b7ce21.

Esophagitis dissecans superficialis ("sloughing esophagitis"): a clinicopathologic study of 12 cases.

Carmack SW, Vemulapalli R, Spechler SJ, Genta RM.

Source

Department of Pathology, VA North Texas Health Care System, The University of Texas Southwestern Medical Center, Dallas, USA.

Abstract

Esophagitis dissecans superficialis (EDS) is a term applied to a rare endoscopic finding characterized by sloughing of large fragments of the esophageal squamous mucosa that may be coughed up or vomited. Although EDS has been reported in association with certain medications and esophageal strictures, most cases remain unexplained and the histopathologic features of EDS are inadequately described. We undertook this study to define useful diagnostic criteria based on the examination of a series of well-characterized cases of EDS. To identify patients with EDS, we searched our endoscopy and pathology databases, reviewed the esophageal biopsy specimens from candidate cases, and correlated them with pertinent clinical information. Twelve patients (11 men and 1 woman) had endoscopic and histologic findings of EDS and 9 had the histologic features without the endoscopic correlates. Biopsies from confirmed EDS patients showed sloughing and flaking of superficial squamous epithelium with occasional bullous separation of the layers, parakeratosis, and varying degrees of acute or chronic inflammation. Fungal elements were identified in 3 patients, but were not associated with acute inflammation. None of the EDS patients were on bisphosphonate therapy or had bullous skin disorders. Follow-up endoscopy in 5 patients showed complete resolution of the esophageal abnormalities in 4 and mild esophagitis in one. In spite of its sometimes, dramatic presentation, EDS is a benign condition that resolves without lasting esophageal pathology. Although an association with medications, skin conditions, heavy smoking, and physical trauma has been reported, the pathogenesis of EDS remains unexplained.

Endoscopic view of esophagitis dissecans superficialis. A: With diffuse sloughing mucosa of the lower esophagus in a 76-year-old woman presenting hematemesis, and the cause was idiopathic; B: with longitudinal sloughing mucosa from upper to mid esophagus in a 67-year-old woman with mucocutaneous type pemphigus vulgaris, note fine whitish fragments of sloughed mucosa, and the index value for anti-desmoglein 3 antibody by enzyme-linked immunosorbent assay was over 1280 (normal value < 7).

PANCREATIC REST — A pancreatic rest (also known as ectopic pancreas, aberrant pancreas, and heterotopic pancreas) refers to ectopic pancreatic tissue. These rare submucosal tumors most commonly consist of cystically dilated exocrine cells. Endocrine pancreatic tissue or a combination of exocrine and endocrine cell types may also be seen.

Pancreatic rests are most frequently found in the distal stomach, duodenum, or proximal jejunum, but have also been reported within a Meckel's diverticulum, the gallbladder, bile ducts, and the minor and major papillae. They are typically discovered incidentally during endoscopy, surgery, or autopsy. They are also occasionally found on CT scan. CT findings that may help differentiate pancreatic rests from other submucosal lesions identified in one study included:

A flat-ovoid shape (long diameter to short diameter ratio of greater than 1.4)
Location of the lesion in the antrum, pylorus, or duodenum
An endoluminal growth pattern
An ill-defined border
Prominent enhancement of the overlying mucosa

The study found that the presence of at least two of the above findings had a sensitivity of 100 percent and a specificity of 82.5 percent for diagnosing a pancreatic rest in the upper gastrointestinal tract [52]. The specificity increased to 100 percent if three of the above findings were present.

However, while pancreatic rests may be detected with CT scan, if a submucosal lesion is noted on upper endoscopy, we suggest endoscopic ultrasound with endoscopic mucosal resection for further evaluation, as small lesions may be missed on CT scan. (See 'General principals of tissue sampling' above.)

Complications of pancreatic rests are rare, but may include ulceration, gastric outlet obstruction, and malignancy [50,53].

Endoscopic appearance — A pancreatic rest appears as a submucosal nodule, usually with a central umbilication that corresponds to a draining duct.

Endosonographic findings — Pancreatic rests are hypoechoic or intermediate echogenic heterogeneous lesions with indistinct margins. They most commonly arise from the third or fourth layer, or a combination of the two layers of the GI tract. Anechoic areas within the lesion correlate with ductal structures.

Diagnosis and treatment — The diagnosis can be made histologically from tissue obtained by biopsy forceps or snare excision, although techniques to obtain deeper biopsies (using jumbo biopsy forceps, tunnel biopsy, endoscopic mucosal resection, or EUS-guided FNA) may be required. The management strategy should be guided by symptoms and suspicion for malignancy. Asymptomatic lesions can be followed expectantly. Endoscopic resection can be performed by standard snare, band ligation-assisted, or cap-assisted polypectomy technique. Surgical resection is preferred to endoscopic resection when the muscularis propria is involved.