A fine yellow verrucous rash is commonly seen in acute neurologic Niemann-Pick disease, which is also known as sphingomyelin deposition disease, an inborn disorder of glycolipid metabolism. It is characterized by a large number of foam cells containing nerve sphingomyelin throughout mononuclear macrophages and the nervous system. The disease is a disorder of nerve sphingomyelin metabolism caused by a deficiency of nerve sphingomyelinase. This leads to accumulation of the latter in the monocyte0macrophage system, hepatomegaly, splenomegaly, and degenerative changes in the central nervous system. Nerve sphingomyelin is formed by linking N-acyl sphingomyelin with one molecule of phosphorylcholine at C1, site. Nerve sphingomyelin is derived from various cell membranes and erythrocyte matrices, among others. After phagocytosis by macrophages during metabolic cellular senescence. This enzyme has the highest activity in the normal liver, and the liver, kidney, brain and small intestine are also rich in this enzyme. In patients with this disease, the enzyme activity in the liver, spleen and other tissues is reduced to less than 50%. Large lipid-rich foam cells with a diameter of 20 to 90 μm can be found in the reticuloendothelial system of the whole body of the child, mainly in the spleen, bone marrow, liver, lungs and lymph nodes. This kind of foam cell is also known as Niemann-Pick cell, usually only see a small nucleus in the offset, loose chromatin; cytoplasm is full of lipid droplets (cytoplasmic bodies), in the unstained slice of the “mulberry”, Giemsa staining, the cytoplasm is blue or blue-green, with varying shades of blue particles inside. Unlike Gaucher cells, the acid phosphatase stain is weakly positive, and the Schultz reaction (for cholesterol) is positive. The two can also be differentiated by potential phase microscopy or electron microscopy. One case was reported in 1914, and the patient died at 18 months of age.The present neurophosphorus depositional disease was discovered in 1934, and it was not until 1966 that it was recognized to be due to a deficiency of the enzyme neurophospholipase. Deficiency of this enzyme results in disturbances in the systemic metabolism of nerve sheath phospholipids, and neurophospholipids are deposited in the monocyte-macrophage system and in cells of the nervous tissue.