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AMA Am J Dis Child. 1954;88(6):764-771. doi:10.1001/archpedi.1954.02050100766010.
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LETTERER-SIWE disease, Hand-Schüller-Christian disease, and eosinophilic granuloma are now generally considered as definite, but not always sharply defined, anatomicoclinical entities.1 They present a common histopathological process characterized, essentially, by the proliferation of histiocytic cellular elements. The usual clinical manifestations of these three varieties of histiocytoses are well recognized. Pulmonary involvement, although not uncommon, is less well appreciated. This is particularly true of primary pulmonary involvement associated with cystic changes.

A review of the literature reveals 12 previously reported cases of histiocytoses with cystic, so-called "honeycomb" lungs.* Of these, the Letterer-Siwe variety comprised the smallest group. At the time lung cysts appeared, most patients did not present a problem of diagnosis, inasmuch as the full-blown anatomicoclinical picture was usually present. The present case, one of Letterer-Siwe disease, is reported because of the unusual primary and apparently isolated pulmonary localization dominated by cystic change.

REPORT OF CASE  A 2½-year-old white girl


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