The clinical and pathologic aspects of 40 cases of eosinophilic granuloma of bone, Hand-Schüller-Christian disease and Letterer-Siwe disease have been reviewed.
These cases were unified by a localized or generalized histiocytic proliferation. However, they could not be subdivided accurately on the basis of either clinical or microscopic findings.
Histologically there was inconstant correlation of foam cells, tissue eosinophilia and fibrosis with clinical course. Nevertheless there was a suggestion that the presence of a homogeneous histiocytic infiltrate, with no eosinophils or foam cells, pointed to a poor prognosis, whereas the presence of large aggregates of eosinophils augured a more favorable course.
With some exceptions, those patients presenting with disease confined to bone had little tendency toward dissemination, whereas those presenting with visceral on soft tissue lesions and generalized maculopapular skin eruptions usually died after a rapid course. Patients with skeletal and visceral or soft tissues lesions at onset had a variable outcome, with marked hepatomegaly and generalized cutaneous involvement being poor prognostic signs.
It is concluded that these conditions probably represent various manifestations of a single disease process and that clinical assessment of rapidity of progression is the best guide to ultimate outcome.
EOSINOPHILIC GRANULOMA OF BONE
