Department of Pathology, Molecular and Cell-Based Medicine, Icahn School of Medicine at Mount Sinai, New York, New York, USA.
Department of Dermatopathology, Icahn School of Medicine at Mount Sinai, New York, New York, USA.
J Cutan Pathol. 2020 Nov;47(11):1080-1084. doi: 10.1111/cup.13834. Epub 2020 Sep 9.
Lip edema with non-caseating granulomas or lymphangiectasia pose a clinical and pathological challenge. These findings can be attributed to cheilitis granulomatosa (CG), Melkersson-Rosenthal syndrome (MRS), or Crohn disease (CD) depending on the appropriate clinical context. Lymphangiectasis, in particular, is a common pathological finding in CD due to lymphatic obstruction by granulomas and intralymphatic granulomas. Because oral symptoms can precede gastrointestinal symptoms of CD or be seen in patients with asymptomatic gastrointestinal disease, the identification of lymphangiectasia should raise the possibility of underlying CD. We present a case of a young woman with several years of lip swelling, with notable lymphangiectasia and subtle granulomas on pathological evaluation. The patient was diagnosed with MRS at an outside institution and treated with systemic steroids, without further systemic evaluation. We believe that early recognition of lymphangiectasia and consideration of CD early in the work-up are critical for early diagnosis and appropriate management. Neither clinical nor histopathological findings should be used in isolation to diagnose GC, MRS, or CD as there is significant debate as to the etiology and overlapping findings of these conditions. We highlight the importance of lymphangiectasia in diagnosing underlying CD in the appropriate clinical context.
唇肿胀伴非干酪样肉芽肿或淋巴管扩张构成临床和病理挑战。这些发现可归因于肉芽肿性唇炎(CG)、梅尔基奥尔森-罗森塔尔综合征(MRS)或克罗恩病(CD),具体取决于适当的临床背景。淋巴管扩张,特别是在 CD 中是一种常见的病理发现,因为肉芽肿和淋巴管内肉芽肿导致淋巴管阻塞。由于 CD 的口腔症状可能先于胃肠道症状出现,或在无症状胃肠道疾病的患者中出现,因此淋巴管扩张的识别应提示存在潜在的 CD。我们报告了一例年轻女性,其唇部肿胀数年,病理评估显示明显的淋巴管扩张和细微的肉芽肿。该患者在一家外部机构被诊断为 MRS,并接受了全身类固醇治疗,而未进行进一步的全身评估。我们认为,早期识别淋巴管扩张并在检查早期考虑 CD 对于早期诊断和适当管理至关重要。临床和组织病理学发现都不应单独用于诊断 CG、MRS 或 CD,因为这些疾病的病因和重叠表现存在很大争议。我们强调了在适当的临床背景下,淋巴管扩张在诊断潜在 CD 中的重要性。