Langerhans cell histiocytosis: a multisystem disorder

Abstract

Langerhans cell histiocytosis can involve single or multiple organ/tissue systems and may go undiagnosed for years until it enters the clinician's differential diagnosis framework. We report on a young patient who initially presented with diabetes insipidus and subsequently with pyrexia of unknown origin. She progressed from single system Langerhans cell histiocytosis to multisystem involvement and remains in long-term remission following chemotherapy.

Multifocal, Multisystem Presentation of Adult-Onset Langerhans Cell Histiocytosis on 18F-Fluorodeoxyglucose Positron-Emission Tomography–Computed Tomography

“…The Histiocyte Society classifies LCH based on the number of organ systems involved and whether or not disease activity is unifocal or multifocal in each organ system [ Table 1 ]. [ 5 ] They also classify disease based on the involvement of high-risk organs.…”

Hand–Schüler–Christian disease presenting with recurrent, bilateral, symmetrical mandibular lesions in an 8‐year‐old boy: report of a case

“…Bone involvement may be asymptomatic or accompanied by swelling and pain of the affected area, pathologic fracture or optic atrophy and otitis media as a result of adjacent bone involvement . Systemic and central nervous system involvement, delay in sexual maturity and bone development may result due to the involvement of the pituitary gland and hypothalamus . Approximately 1/3 of the patients with HSC disease present with the classical triad of exophtalmos, calvarial lytic lesions, and diabetes insipidus due to extension from the calvarial bone to the posterior pituitary stalk of the hypothalamus .…”