Multifocal eosinophilic granuloma: a clinical pathologic conference.

Eosinophilic granuloma has traditionally been designated as the least severe component of the inter-related triad of diseases, Histiocytosis X (Lichtenstein 1953, 1964). In 1969, Lieberman and his group questioned the use of this time-honored term and proposed instead that eosinophilic granuloma be regarded as a separate and distinct entity which could present either as a singular (unifocal) lesion or as a multifocal process. The term, "multifocal eosinophilic granuloma" was, thus, suggested as a replacement for the designation formerly known as Hand-Schuller-Christian Disease. The remaining component of Histiocytosis X termed Letterer-Siwe Disease was felt to be a totally separate disorder unrelated to eosinophilic granuloma and best classified as a relatively aggressive reticuloendothelial malignancy. In this presentation, we wish to report a case of multifocal eosinophilic granuloma which has run a very interesting course over a total of almost 10 years from the time of initial symptoms to the present. The more recent classification of Lieberman will be employed throughout this presentation. We will also propose the hypothesis that in this case, multifocal eosinophilic granuloma may represent a sequela of acquired immune deficiency perhaps initiated by T-cell viral infection. The possibility that, in a susceptible host, development of eosinophilic granuloma may be triggered by contact with environmental agents will also be discussed.