Adult Multisystem Langerhans Cell Histiocytosis with Gastrointestinal Presentation: A Case Report

Abstract

Clinical examination and biochemical tests ruled out thyrotoxicosis. Colonoscopy showed mild to moderate pancolitis. Histopathological examination of the colonic mucosa confirmed Langerhans cell histiocytosis. INTRODUCTIONLangerhans cell histiocytosis (LCH) is a rare disorder of unknown cause. The granuloma-like pathologic lesion is crucially characterised by cells sharing many characteristics with normal cutaneous Langerhans cells and variably associated with collections of T-lymphocytes, macrophages, neutrophils and eosinophils [1] . These cells, now known as LCH cells, are CD1a and Langerin-positive. LCH encompasses a spectrum of disorders with diverse clinical presentations, ranging from single organ to multiple organ involvements, which includes bone, skin, lung, gut, liver, bone marrow and central nervous system. LCH can occur at any age, but the peak incidence is in children below two years old [1][2][3] . The LCH patients may present with gastrointestinal involvement, yet symptoms like diarrhoea and failure to thrive, are nonspecific. The reported incidence ranges from 2% to 13%, but the actual figure is unknown. Well-documented colon involvement is very rare, with fewer than 20 convincing published reports worldwide. In these instances, initial presenting symptoms included diarrhoea, sometimes with blood or mucus, malabsorption, failure to thrive and oedema ABSTRACTLangerhans cell histiocytosis is a rare histiocytic disorder, characterised by histiocyte infiltration of the body organ. Although this disease has been diagnosed in all age groups, it is most common in children from one to three years old. The LCH patients may vary in presentation depending upon the sites and extent of involvement. The disease is limited to one organ system in approximately 55% of patients while the remainder presents with multisystem disease. Gastrointestinal involvement of Langerhans cell histiocytosis is even rarer. The majority of gastrointestinal Langerhans cell histiocytosis are from paediatric cases. We present a case of a 69-year old female who had presented with chronic diarrhoea for a three month duration, associated with a significant weight loss.