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January 1970

Histiocytosis X (Letterer-Siwe Disease) With Involvement of the Inner Ear

Author Affiliations

Chicago; Philadelphia; Chicago
From the Temporal Bone Laboratory of the Section of Otolaryngology, Department of Surgery, the University of Chicago (Drs. Cohn and Lindsay), and the Jefferson Medical College, Philadelphia (Dr. Sataloff).

Arch Otolaryngol. 1970;91(1):24-29. doi:10.1001/archotol.1970.00770040050007

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.1

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,2 further defined by Siwe3,4 and subsequently termed Letterer-Siwe disease by Abt and Denenholz5 is the aggressive form of histiocytosis X and assumes the most grave prognosis.

Tos6 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

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