Enfermedad de Erdheim-Chester e histiocitosis de células de Langerhans. ¿Una asociación fortuita?

Abstract

Erdheim-Chester disease is an idiopathic systemic histiocytosis hystologically different to Langerhans histiocytosis which is characterized by symmetric sclerotic bone lesions, predominantly affecting the diaphyseal and metaphyseal areas of large bones. It is not classified within malignant histicytosis, but the clinical course tends to be agressive with a poor reponse to different schedules of treatment. In rare cases the association of both entities has been reported: Erdheim-Chester disease and Langerhans histiocytosis. We present a new case with histological data of both histiocytosis whose clinical course included bone and muscle pain, insipidus diabetes, exophthalmos, bilateral symmetrical sclerosing bone lesions and a cerebellar syndrome.