SYSTEMIC RETICULOENDOTHELIOSIS (LETTERER-SIWE DISEASE) IN THE ADULT MALE

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Abstract
The diverse manifestations of reticulo-endotheliosis are now thought to represent different phases of one disease depending upon the rapidity of the pathologic process and the location of the lesions. The form of the disease usually evidences itself as either xanthoma disseminata of the skin, eosinophilic granuloma of bone, the Schuller-Christian syndrome, or as one of the variants. The acute form of the disease, which was chiefly described in children, is referred to as Letterer-Siwe disease. Transition from one clinical form to another was observed. The pathologic picture may be divided into 3 phases (1) prolific (2) lipoid (3) fibrous. Two cases of systemic reticulo-endotheliosis (Letterer-Siwe disease) in men are described. Clinically one case resembled multiple myeloma, complicated by nephrocalcinosis and secondary hyperparathyroidism. The 2nd case was similar to those reported in the literature, in that fever, splenomegaly, adenopathy, anemia and purpura were major findings. The results of treatment with various recommended therapeutic agents were disappointing.