Langerhans cell histiocytosis (LCH) is a neoplasm of the monocyte-macrophage lineage, characterized by clonal proliferation and dissemination of cells that express CD1a+ and CD207. It is a disorder that predominates in childhood. Although the skin is the second most frequently affected organ (30-60%), isolated cutaneous involvement is rare; its frequency does not exceed 4 to 12 percent of cases. Single system-LCH usually has a good prognosis. We describe a case of LCH with isolated cutaneous involvement that presented in an adult patient and was refractory to polychemotherapy.
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