Langerhans cell histiocytosis presenting as isolated central diabetes insipidus in a 2-year-old child: a rare manifestation of rare disease

Nirmal S. R., Revathi Krishnakumar, Gayathri G. Nair, Sri Lakshmi Chordia, Arulkumaran Arunagirinathan


Central diabetes insipidus, though uncommon in children, has varied causes, the commonest ones being genetic mutations, infiltrative disorders, infections. Isolated central diabetes insipidus is not one of the often encountered conditions in the pediatric practice. Here we report a case of 2 ½ years old female child who presented to us following history of polyuria for 2 months, who was confirmed to have central diabetes insipidus which was later evaluated to be secondary to Langerhans cell histiocytosis. Magnetic resonance imaging (MRI) and histopathological studies further helped in confirmation of the diagnosis. Langerhans cell histiocytosis is a rare, multifarious, and underdiagnosed hematologic disease in which isolated diabetes insipidus can be the sole presenting feature before other manifestations. Hence, this diagnosis could strongly be considered in the work up of central diabetes insipidus in children.


Children, Central diabetes insipidus, Langerhans cell histiocytosis

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