NIEMANN-PICK'S DISEASE WITH CHERRY-RED SPOTS IN THE MACULA

Abstract

Niemann,¹ in 1914, described a peculiar disease in a female infant, aged 18 months, which resembled Gaucher's disease in some respects, but which was unlike it in others. Several years later, Pick² showed that similar cases subsequently reported were not cases of Gaucher's disease, but constituted a clinical and pathologic entity for which he proposed the term lipoid splenohepatomegaly. The specific anatomic feature was the presence of large numbers of vacuolated or "foam" cells in many organs and in most tissues of the body. As the great mass of foam cells are derived from the various kinds of histiocytes, and as the foamy appearance is due to lipoid droplets in the histiocytes, Bloom³ suggested the term lipoid histiocytosis as a more accurate description of the condition than lipoid splenohepatomegaly, the latter indicating that the process is limited to the liver and spleen, which is not the case.