Published online Mar 16, 2017. doi: 10.4253/wjge.v9.i3.139
Peer-review started: May 3, 2016
First decision: May 17, 2016
Revised: December 6, 2016
Accepted: December 27, 2016
Article in press: December 28, 2016
Published online: March 16, 2017
Processing time: 319 Days and 21.8 Hours
Langerhans cell histiocytosis (LCH) is a rare syndrome characterized by unifocal, multifocal unisystem, or disseminated/multi-system disease that commonly involves the bone, skin, lymph nodes, pituitary, or sometimes lung (almost exclusively in smokers) causing a variety of symptoms from rashes and bone lesions to diabetes insipidus or pulmonary infiltrates. We present a previously unreported case of gastrointestinal LCH as well as a novel characteristic lesion affecting the colon of a young woman who presented with signs and symptoms mimicking acute on chronic appendicitis. Immunohistochemical analysis of appendectomy specimen and nodular specimens on colonoscopy demonstrated S-100, CD1a, and langerin reactivity. The patient underwent systemic chemotherapy with cytarabine and demonstrated excellent response to therapy.
Core tip: Langerhans cell histiocytosis (LCH) of the adult gastrointestinal tract can affect the appendix and present as mild inflammatory findings on exploratory laparascopy, or white-yellowish nodularities and polyps on lower endoscopy. Immunohistochemical staining of surgical specimens for CD1a, S-100, and langerin should be considered in gastrointestinal lesions that demonstrate histology concerning for histiocytosis in an attempt to decrease morbidity related to undiagnosed LCH.