Esophagitis dissecans: a rare cause of odynophagia.

drome, IgA paraproteinemia, and bron− chiectasis presented with a 3−month his− tory of worsening odynophagia and weight loss. Initial endoscopy revealed er− ythema in the mid− to distal esophagus and a small mucosal tear. Barium swallow, esophageal manometry, and 24−hour pH studies were all normal. Her symptoms worsened despite proton−pump inhibitor therapy. Repeat endoscopy showed denuded mucosa with overlying slough in the mid−esophagus (l" Figure 1) and a su− perficial bulla (l" Figure 2). The patient had no mucocutaneous lesions and an empirical trial of oral corticosteroids was ineffective. Esophageal biopsies showed ulcerated and inflamed squamous mucosa (l" Figure 3), but indirect immunofluores− cence studies were negative. Fungal hy− phae were noted but antifungal therapy was unsuccessful. All routine and autoim− mune−screen blood tests were essentially normal. She later developed generalized lymphadenopathy and diffuse large B−cell lymphoma was diagnosed. Treatment with rituximab (anti−CD 20) resulted in a rapid improvement in her dysphagia, and subsequent endoscopy showed normal mucosa with a fibrotic esophageal stric− ture, which was dilated (l" Figure 4). Biopsies showed only mild, nonspecific inflammation and no malignancy. Spontaneous bullous esophagitis is rare and is usually associated with cutaneous diseases, including the bullous dermatos− es (pemphigus, pemphigoid), lichen pla− nus, and Stevens±Johnson syndrome. Chronic esophagitis dissecans was first described by Ponsot et al. [1] and is char− acterized by recurrent dysphagia, due to spontaneous shedding of esophageal mu− cosa, and localized esophageal strictures in the absence of mucocutaneous lesions [2]. Although the presence of lymphoma was unlikely to be coincidental in this pa− tient, we found no histological features to suggest that this was an unusual manifes− tation of paraneoplastic pemphigus. The patient’s medical history suggests an au− toimmune process [3], although there were no other features suggestive of this and corticosteroid treatment was ineffec− tive. Rituximab has been used to treat pemphigus associated with lymphoproli− ferative disease [4,5], and the rapid clini− cal and endoscopic response that this treatment brought about in our patient suggests that it can also be used to treat esophagitis dissecans.