| dc.description.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. | en_US |
