Central Diabetes Insipidus as a Manifestation of Langerhans Cell Histiocytosis: A Narrative Review

Abstract

INTRODUCTION: Langerhans Cell Histiocytosis (LCH) is a hematological disorder caused by abnormal proliferation and infiltration of dendritic cells. Due to its potential to affect multiple systems, LCH symptoms can be heterogeneous and nonspecific. When the Central Nervous System (CNS) is involved, it may manifest as Central Diabetes Insipidus (CDI).  OBJECTIVE: Analyze the relationship between LCH and CDI in children and its clinical progression.  METHODS: A literature review was conducted using the PubMed database, with the following health descriptors: Children, Central Diabetes Insipidus, and Langerhans Cell Histiocytosis. Articles not relevant to the topic were excluded, resulting in a final sample of *n=12*.  RESULTS: LCH is a multisystemic disease and should be considered a differential diagnosis in pediatric patients. It primarily affects the CNS and endocrine system, with CDI present in up to 30% of cases. Diagnostic workup should include laboratory tests, neuroimaging, and lesion biopsy. Brain MRI typically reveals pituitary stalk thickening (<6.5 mm) or hypothalamic masses. Prognosis depends on disease type, with worse outcomes in multisystem or high-risk organ involvement. Due to the broad spectrum of affected systems, there is no standard treatment. Generally, chemotherapy and corticosteroids are used. For CDI secondary to LCH, desmopressin provides symptomatic relief.  CONCLUSION: As demonstrated, LCH can present with diverse clinical syndromes, complicating early diagnosis and increasing the risk of potentially fatal complications. Therefore, LCH must be considered in the differential diagnosis of patients with mucocutaneous, skeletal, and endocrine manifestations, especially in children within the prevalent age group.