EC Clinical and Medical Case Reports

Langerhans cell histiocytosis (LCH) is a rare disease characterized by the abnormal proliferation of Langerhans cells, a type of specialized macrophage. It presents a wide range of clinical manifestations, from isolated bone lesions to severe multisystem involvement. We report the case of a 3-year-old child with symptoms of central diabetes insipidus (DIC) revealing, associated with chronic otorrhea, exophthalmos, and splenomegaly. Investigations, including CT scan and bone biopsy, confirmed the diagnosis of HL. Management included administration of desmopressin for DIC, as well as antibiotic treatment for recurrent ENT infections. This case highlights the importance of suspecting HL in the differential diagnosis of pediatric DIC, allowing early identification and management of potential multisystem involvement.

 Keywords: Langerhans Cell Histiocytosis; Desmopressin; Central Diabetes Insipidus; Histology

1. J Donadieu., et al. “Clinical presentation and epidemiology of Langerhans cell histiocytosis in children”. Archives de Pédiatrie5 (2008): 520-522.
2. C Thomas and J Donadieu. “Langerhans cell histiocytosis: therapeutic management”. Archives of Pediatrics5 (2008): 529-553.
3. F Cattin., et al. “Magnetic resonance imaging of diabetes insipidus”. Feuillets de Radiologie6 (2005): 425-434.
4. Thomas E Herman and Marilyn J Siegel. “Langerhans cell histiocytosis: Radiographic images in pediatrics”. Clinical Pediatrics2 (2009): 228-231.
5. N Matoussi., et al. “Central diabetes insipidus: diagnostic difficulties”. Annals of Endocrinology3 (2008): 231-239.
6. Neil Chanchlani., et al. “Langerhans cell histiocytosis in a 5-month-old baby”. Canadian Medical Association Journal 1 (2021): E23.
7. Donadieu J and Héritier S. “Langerhans cell histiocytosis in children”. La Presse Médicale (2017).
8. H Aloulou., et al. “Central diabetes insipidus in children: study of six observations”. Journal of Pediatrics and Child Care 20 (2007): 289-294.
9. Karima Atarraf., et al. “Specific localizations of Langerhans cell histiocytosis in children, scapula and pubis: about two cases”. Pan African Medical Journal 18 (2014): 328.
10. Chalinee Monsereenusorn and Carlos Rodriguez-Galindo. “Clinical characteristics and treatment of langerhans cell histiocytosis”. Hematology/Oncology Clinics of North America 5 (2015): 853-873.
11. Sconocchia T., et al. “Langerhans cell histiocytosis: current advances in molecular pathogenesis”. Frontiers in Immunology 14 (2023): 1275085.
12. Le Louet S., et al. “Childhood Langerhans cell histiocytosis with severe lung involvement: a nationwide cohort study”. Orphanet Journal of Rare Diseases 1 (2020): 241.
13. Carlos Rodriguez-Galindo and Carl E Allen. “Langerhans cell histiocytosis”. Blood16 (2020): 1319-1331.
14. Goyal G., et al. “International expert consensus recommendations for the diagnosis and treatment of Langerhans cell histiocytosis in adults”. Blood17 (2022): 2601-2621.