Diabetes Insipedes in a child disclosing localized Langerhans’ Cell Histiocytosis

摘要

Langerhans cell histiocytosis (LCH) is monoclonal neoplastic condition of aberrant bone marrow histiocytes. The latter are part of the innate immune system and certain exogenous/endogenous stimuli may trigger its expansion. Hence LCH can present with limited or multiple organ involvement that may include; bones, lung, endocrine, skin, lymph nodes, spleen and bone marrow. In this case report, we describe a 3-year-old boy who presented with severe polyuria and polydipsia. Laboratory investigations were consistent with diabetes insipidus (DI). MRI of the brain; confirmed absence of the bright spot in his pituitary gland did not show evidence of tumor or enlargement by inflammation. Moreover, MRI revealed 2 skull lesions and their subsequent biopsy confirmed LCH. Systemic examination and tests including PET scan did not show additional lesions. Since his disease was localized, he received only Desmopressin acetate 120 ug twice daily for his DI without surgery, radiotherapy or chemotherapy. One year later, his disease remained limited to DI and the 2 bonny lesions.