Letterer-Siwe disease. Presentation as an otologic problem.

Letterer-Siwe disease is one of the three clinical syndromes that are collectively known as histiocytosis X. This is the most aggressive form of histiocytosis and is frequently fatal. Recently, however, early treatment with chemotherapeutic agents and steroids has prolonged survival and has even resulted in remission of a number of cases. This paper draws attention to a persistent external otitis as an early symptom of Letterer-Siwe disease and identifies, histologically, involvement of the external auditory canal.