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Letterer‐Siwe disease in the adult

Letterer-Siwe disease was first described by Letterer in 1924. It usually presents in children under 3 years of age and is characterized by the majority of the following features: fever, anaemia, iymphadenopathy, hepatosplenomegaly, otitis media, recurrent bacterial infections, haemorrhage, osteolytic lesions in bones, and a distinctive cutaneous eruption. The pathology shows multisystem infiltration by differentiated histiocytes. However, the disease is not always fatal. In one series 177% of ninety-six patients were well 3 years after the onset ofthe disease (Doede & Rappaport, 1967) and in another seven of eighteen patients with seven or more organs involved survived (Lahey, 1975). Letterer-Siwe disease is now grouped with Hand-SchuUer-Christian Disease and eosinophilic granuloma in the spectrum of histiocytosis X (Table i). Farber (1941) first suggested that all three conditions were part of the same disease spectrum and because of the difficulty