Histiocytosis X (Eosinophilic Granuloma of Bone, Letterer-Siwe Disease, and Schüller-Christian Disease)

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The literature on histiocytosis X has been reviewed from 1952 to date. Clinical and pathological observations during this period tended to substantiate the integrated concept of histiocytosis X as a nosological entity embracing eosinophilic granuloma of bone, Letterer-Siwe disease (when this is non-neoplastic), and Schuller-Christian disease. The impression has been reinforced that the lesion of eosinophilic granuloma is the pathological expression of an early, rather rapidly developing reaction to an etiological agent. This appears to be especially true in the chronic disseminated form of the malady (Schuller-Christian disease). Recent recorded experience has added new sites of involvement—the liver, kidneys, female genitalia, and, possibly, the cranial nerves and eye, as well. Altogether, the manifestations of the disease may be quite protean. Especially noteworthy are the numerous reports on pulmonary eosinophilic granuloma, occurring with or without demonstrable lesions elsewhere. Stress was placed on the practical importance of making a clear distinction between acute (or subacute) histiocytosis X and neoplastic reticuloendotheliosis, both of which require different therapy. From existing evidence it would appear that cytotoxic agents, radioactive isotopes, and the like should be avoided in acute disseminated histiocytosis X since they may seriously injure an already damaged bone marrow. In these circumstances, as in many severe cases of chronic disseminated histiocytosis X (Schuller-Christian disease), the experience of recent years points up the value of steroids in tiding patients over critical situations. Collaterally, the value of selected antibiotics in combating secondary infections and of roentgen therapy, especially for mucocutaneous and skeletal lesions, has been further documented. Among the features brought out by our own material are: (1) specific involvement of the liver in chronic disseminated histiocytosis X, leading to serious intrahepatic obstructive jaundice; (2) extension of eosinophilic granuloma in bone to regional lymph nodes (reinforcing the concept of infection); and (3) reappearance of the lesion of eosinophilic granuloma as the pathological expression of recrudescence in chronic disseminated histiocytosis X (Schuller-Christian disease) of long standing.