Clinical Conundrums in Diagnosis and Management of Multisystem Langerhans Cell Histiocytosis

Abstract

Langerhans Cell Histiocytosis (LCH) is a rare disorder with an incident of 1.8 cases per 1,000,000. It involves granulomatous deposits in multiple organs, leading to a wide variety of manifestations such as bone lesions, pulmonary nodules, pituitary lesions, and skin lesions. With mucocutaneous manifestation, it is often diagnosed in childhood, typically making LCH a childhood disease. However, due to the involvement of multiple organ systems and variable clinical courses, if not diagnosed during childhood, the patients with LCH often suffer from delayed diagnosis and treatments as adults. choroid glioma, and metastatic lesion. Given the concerning changes involving the pituitary as shown on radiographic findings, serum levels of the following pituitary hormones were obtained: FSH, LH, ACTH stimulation test, TSH, prolactin, IGF-1. The results came back with abnormally low FSH at 0.4 mIU/mL (reference range for male is 0.9 11.9 mIU/mL), LH < 0.1 mIU/mL (1.5-9.3 mIU/mL for male), ACTH stimulation test with a peak cortisol level at 14.8 ug/dL (normal response consists of at least 7 mcg/dL from baseline and a peak of *Corresponding author: Jun Park, St. Vincent Hospital Indianapolis, Indiana, USA CaSe RePoRt