Perianal Langerhans-Cell Histiocytosis: Review of the Literature

Langerhans-cell histiocytosis (LCH) is a rare disorder characterized by the proliferation of Langerhans cells, which originate in the bone marrow. It presents in two main clinical forms: single system LCH (SS-LCH), when the disease involves a single organ or system, and multisystem LCH (MS-LCH), which follows an acute, progressive, and disseminated course, characterized by the proliferation of Langerhans cells which originate from multiple organs or systems, including the lungs, bones, liver, spleen, bonemarrow, skin, central nervous and mucous membranes. Mortality is often associated with complications such as chronic obstructive pulmonary disease, secondary primary neoplasms, sepsis, hemorrhagic syndromes, and bone marrow failure. Although rare in adults, the initial presentation of MS-LCH may involve the perianal region. We herein report the case of a 38-year-old man with initial perianal involvement of MS-LCH, refractory to treatment with vinblastine, prednisone, cytarabine, and cladribine, who ultimately died from sepsis due to bone marrow failure. Additionally, we conducted a literature review using the keywords perianal histiocytosis, anal histiocytosis, and perineal histiocytosis on the PubMed, SciELO, and LILACS databases.