Langerhans cell histiocytosis (LCH) is a disease of unknown etiology, characterized by proliferation of pathological Langerhans cells within different organs. It mainly affects children, but adult cases also occur, with an incidence rate of one to two per million. The head and neck are affected in almost 90% of cases. Diagnosis is made by means of histopathological analysis, and imaging studies are necessary in order to determine extent of the disease. There are no controlled trials proposing an optimal treatment protocol for LCH. Prognosis in adults is generally good due to the slow evolution of the disease and its favourable response to treatment. In our report, we present three cases of LCH in patients aged 16, 24, and 28 years respectively, with primary manifestation in the maxillofacial area. A literature review was also conducted.
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Cutaneous Intertriginous Langerhans Cell Histiocytosis in Adults: A Case Report and Systematic Review.
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Saint Louis University School of Medicine, St. Louis, MO.
Objective: Authors characterized all published adult cases of cutaneous, intertriginous Langerhans cell histiocytosis (LCH) to bring this clinical presentation to the attention of clinicians. We emphasize the morphology, histopathology, immunohistochemical profiles, and genetic mutations associated with these cases.
Materials And Methods: A systematic review of the National Center for Biotechnology Information's PubMed was conducted, utilizing the following specific key words to identify all adult LCH patients with cutaneous intertriginous involvement: "Intertriginous Langerhans," "Vulvar Langerhans," "Genital Langerhans," "Perineal Langerhans," "Perianal Langerhans," "Intergluteal Langerhans," "Inguinal Langerhans," "Axillary Langerhans," and "Inframammary Langerhans.
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Department of Nuclear Medicine, West China Hospital of Sichuan University, No. 37, Guoxue Alley, Chengdu, Sichuan, 610041, People's Republic of China.
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