Abstract

SIMIELE NARVARTE, A.; NOVOA SANJURJO, F.; GOMEZ RODRIGUEZ, N.  and  ANTON BADIOLA, I.. Erdheim-Chester disease and Langerhans histiocytosis: A fortuitous association?. An. Med. Interna (Madrid) [online]. 2004, vol.21, n.12, pp.27-30. ISSN 0212-7199.

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.

Keywords : Erdheim-Chester disease; Langerhans histiocytosis; Insipidus diabetes; Cerebellar syndrome.

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