Title: Histiocytosis X (Letterer-Siwe Disease) With Involvement of the Inner Ear
Abstract: THE term histiocytosis X was introduced by Lichtenstein to group together the syndromes of eosinophilic granuloma of bone, Hand-Schüller-Christian disease, and Letterer-Siwe disease into a common disease entity expressing different manifestations of reticuloendotheliosis, or histiocytic proliferation of uncertain etiology.<sup>1</sup> Eosinophilic granuloma is the localized variety of histiocytosis X manifested by single or multiple isolated bony lesions, and carries a favorable prognosis. The Hand-Schüller-Christian variety is the more chronic protean manifestation with multiple bony and soft tissue involvement, and carries a prognosis less favorable than eosinophilic granuloma. Acute progressive histiocytosis, first described by Letterer,<sup>2</sup>further defined by Siwe<sup>3,4</sup>and subsequently termed Letterer-Siwe disease by Abt and Denenholz<sup>5</sup>is the aggressive form of histiocytosis X and assumes the most grave prognosis. Tos<sup>6</sup>has recently reviewed the otolaryngological manifestations in Letterer-Siwe disease, essentially as follows. This variety of histiocytosis X occurs in young children generally under 4 years
Publication Year: 1970
Publication Date: 1970-01-01
Language: en
Type: article
Indexed In: ['crossref']
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Cited By Count: 33
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