Acute Disseminated Langerhans Cell Disease – A Rare Case Report

Langerhans cell disease previously known as Histiocytosis X is a non lipidreticuloendotheliosis that presents with single or multiple lesions distributed in the soft tissues, lymph nodes, various organs and bones, especially in areas of marked reticulo endothelial activity.Histiocytosis X encompasses three conditions which include a localized form (Eosinophilic granuloma), a chronic disseminated form (Hand Schuller-Christian disease) and an acute or subacute disseminated form (Letterer-Siwe disease).Eosinophilic granuloma is characterized by solitary or multiple skeletal lesions with no extra skeletal involvement whereas Hand Schuller-Christian disease manifests with widespread skeletal and extra skeletal lesions with a chronic clinical course.Letterer-Siwe disease presents with widespread lesions of both skeletal and extra skeletal tissues with a more acute severe clinical course and a high mortality rate.Most often these patients report initially to the dentist because of the significant oral involvement including gingival swelling and mobility of teeth.Such a rare case of acute disseminated Langerhans cell disease that presented to the dental clinic is discussed in this article.