University of Texas Medical Branch, Galveston, TX.
Am J Gastroenterol. 2024 Oct 1;119(10S Suppl):S2185. doi: 10.14309/01.ajg.0001042408.18104.2b.
Lymphocytic esophagitis (LyE) is a rare esophageal disorder of unknown etiology, characterized by dense peripapillary lymphocytes without neutrophils or eosinophils, and spongiosis. Patients typically present with symptoms such as dysphagia or chest pain. Here, we describe a notable case of lymphocytic esophagitis in a patient who presented with food impaction.
CASE DESCRIPTION/METHODS: A 53-year-old woman with a history of pityriasis lichenoideschronica and rheumatoid arthritis reported chronic dysphagia and sought emergency care for food impaction. A subsequent upper endoscopy revealed a long submucosal tear in the upper esophagus during intubation (see Figure 1A), leading to the procedure's cessation. A follow-up upper endoscopy 9 weeks later, employing an ultra-slim scope, identified severe stenosis in the upper esophagus (see Figure 1B). Dilation was performed using a Savary dilator. Examination of the esophageal lining revealed edema, rings, and furrows. Proximal and distal esophageal biopsies were obtained, indicating a marked increase in intraepithelial lymphocytes, with an absence of eosinophils or neutrophils. The squamous epithelium exhibited evidence of spongiosis and necrotic keratinocytes, consistent with a diagnosis of lymphocytic esophagitis. The patient continued omeprazole 40 mg twice daily and started budesonide at 1 mg twice daily. A repeat upper endoscopy with esophageal biopsies demonstrated a significant improvement in lymphocytic infiltration (see Figure 2A-B).
LyE typically manifests in females during their 5th-6th decades, presenting a notable contrast to eosinophilic esophagitis (EOE), which predominantly affects males in their 2nd-3rd decades. However, the endoscopic appearance of LyE often mirrors that of EOE. Studies suggest potential associations between LyE and Crohn's disease in children, as well as primary esophageal dysmotility in adults. Notably, our patient has a history of rheumatoid arthritis, a correlation observed in 2 other cases in the literature. While limited literature exists on pityriasis lichenoideschronica with esophageal involvement, specifically presenting as LyE, this singular case underscores the interconnection of these 2 disease processes. These observations raise the possibility of an autoimmune etiology. There is no consensus on the lymphocyte count threshold for diagnosis, a challenge compounded by the rarity of the disease, leading to a lack of definitive guidelines for optimal management.JOURNAL/ajgast/04.03/00000434-202410001-03261/figure1/v/2024-10-11T031912Z/r/image-tiff.
淋巴细胞性食管炎(LyE)是一种病因不明的罕见食管疾病,其特征为密集的乳头周围淋巴细胞,无中性粒细胞或嗜酸性粒细胞,伴有海绵样变性。患者通常表现为吞咽困难或胸痛等症状。在这里,我们描述了一例以食物嵌塞为表现的淋巴细胞性食管炎患者。
病例描述/方法:一名 53 岁女性,既往有 pityriasis lichenoides chronica 和类风湿性关节炎病史,诉慢性吞咽困难,并因食物嵌塞而寻求紧急医疗。随后的上消化道内镜检查显示在上食管插管过程中有一个长的黏膜下撕裂(见图 1A),导致该过程停止。9 周后,采用超细内镜进行了后续的上消化道内镜检查,发现上食管严重狭窄(见图 1B)。采用 Savary 扩张器进行扩张。食管黏膜检查显示水肿、环和皱襞。获得近端和远端食管活检,提示上皮内淋巴细胞明显增加,无嗜酸性粒细胞或中性粒细胞。鳞状上皮表现出海绵样变性和坏死角朊细胞的证据,符合淋巴细胞性食管炎的诊断。患者继续每天口服奥美拉唑 40mg,每天两次,并开始每天两次口服布地奈德 1mg。再次进行上消化道内镜检查和食管活检显示淋巴细胞浸润明显改善(见图 2A-B)。
LyE 通常在女性 5-6 十年代发病,与嗜酸性食管炎(EOE)形成鲜明对比,EOE 主要影响男性 2-3 十年代。然而,LyE 的内镜表现通常与 EOE 相似。研究表明,LyE 与儿童克罗恩病以及成人原发性食管动力障碍之间存在潜在关联。值得注意的是,我们的患者有类风湿性关节炎病史,这在文献中的另外两例中也有观察到。虽然关于有食管受累的 pityriasis lichenoides chronica 特别是表现为 LyE 的文献有限,但这单一病例强调了这两种疾病过程之间的联系。这些观察结果提示可能存在自身免疫病因。由于疾病罕见,淋巴细胞计数的诊断阈值尚无共识,这使得缺乏明确的最佳管理指南变得更加复杂。