Vulvar Langerhans cell histiocytosis and thalidomide: an effective treatment option

Abstract

Langerhans cell histiocytosis (LCH) is a disease characterized by clonal proliferation and a pathological infiltration of Langerhans cells in one or more organs, in which almost every tissue may be involved. Its incidence is slightly increased in males.1,2 It is more common in children (only 2% of LCH patients are aged over 70 years), and it may involve multiple systems or may be localized.1 Although it is not a malignancy, LCH may cause the selfdestruction of involved tissues, such as bone, skin, lung, and brain tissue.2,3 Langerhans cell histiocytosis with isolated involvement of the skin or mucous membranes is rare and occurs in only 4–7% of cases.1,2 The occurrence of LCH that is restricted to the vulva is very rare, and fewer than 26 such cases have been published in the literature. We describe a very interesting case in a patient who demonstrated an excellent response to thalidomide.