A 2-yr-old with Langerhans cell histiocytosis and diabetes i
A two years old girl was admitted with polyuria and polydipsia. Plasma glucose (random, 86 mg/dl), serum sodium [132 mEq/l (normal range 135-145)], and serum osmolality [284 mOsm/kg (285-295)], were normal. However, urine osmolality was 34 mOsm/kg (normal range 300-900). MRI brain showed normal pituitary stalk thickness and absence of posterior pituitary bright spot.

Based on the symptoms, hypo-osmolar urine and findings of the MRI brain, she was diagnosed to have central diabetes insipidus (DI) and started on desmopressin nasal spray.

One year later, she developed induration over the right gluteal region which later developed into fistula. Biopsy of the wall revealed numerous Langerhans cells (reniform nucleus and abundant pink cytoplasm) with characteristic immunoreactivity for CD1a antigen.

Skeletal survey and positron emission tomography-computed tomography (PET-CT) did not show any other organ involvement. She was diagnosed to have multisystem Langerhans cell histiocytosis (pituitary and skin) and treated with chemotherapy (prednisolone, vinblastine and 6-mercaptopurine) which led to the healing of cutaneous lesion.

The patient has been on follow up for the last three years; though in clinical remission she is continued on desmopressin spray due to the persistence of DI.

Source: https://pxmd.co/NzU8C
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