An unusual case of adult-onset multi-systemic Langerhans cell histiocytosis with perianal and incident thyroid involvement

Abstract

Langerhans cell histiocytosis (LCH) shows a wide clinical spectrum and prognosis that ranges from benign and self-limiting single-system disease (with single or multifocal lesions) to a potentially lethal multi-system disease with severe organ dysfunction and death in some cases.It has been stated that the diagnosis is often delayed in perianal LCH unless LCH is specifically considered in the etiology, despite the fact that mucosal involvement may precede systemic involvement.Our findings support the statement that most of patients with LCH were PET positive at the time of initial diagnosis, while also emphasize the inclusion of this imaging modality as a part of the diagnostic workflow as well as in the setting of treatment response evaluation among adult LCH patients.