Neutral lipid storage disease (also known as Chanarin–Dorfman syndrome) is a congenital autosomal recessive disorder characterized by accumulation of triglycerides in the cytoplasm of leukocytes[1], (Jordan’s Anomaly) muscle, liver, fibroblasts, and other tissues. It commonly occurs as one of two subtypes, cardiomyopathic neutral lipid storage disease (NLSD-M), or ichthyotic neutral lipid storage disease (NLSD-I) which is also known as Chanarin–Dorfman syndrome), which are characterized primarily by myopathy and ichthyosis, respectively. Normally, the ichthyosis that is present is typically non-bullous congenital ichthyosiform erythroderma which appears as white scaling. It has been associated genetically with mutations in the CGI58 gene, (for NLSD-I), or the ATGL gene (for NLSD-M.)

Cause
Neutral lipid storage disease is caused by the abnormal and excessive accumulation of lipids in certain bodily tissues, including the liver, the heart, and muscle. Normally, these lipids are stored as lipid droplets and are normally used for metabolism, cell signaling and trafficking of vesicles. Neutral lipid storage disease is a disease that is diagnosed with the simultaneous occurrence of myopathy and/or  ichthyosis. Myopathy is defined as a disease of the muscle tissue. Ichthyosis is a skin related disease in which the skin becomes very scaly, thick, and dry. Genetics
Neutral lipid storage disease (NLSD) occurs in one of two genetic and clinical subtypes.
Treatment for the ichthyosis is limited; moisturizers are commonly used to help manage dry, flaky, itchy skin. Epidemiology
The rarity and likely under-diagnosis of neutral lipid storage disease prevents an accurate epidemiological estimate of its frequency in human populations. Fewer than 100 cases of NLSD have been reported  since the first case of two women with nonbullous ichthyosiform erythroderma was reported in 1974 by Maurice Dorfman. Many (but not all) of the cases reported since 1974 were in individuals of Middle Eastern.descent. A possible reason for the elevated rates of occurrence may be the higher frequency of consanguineous marriages in these populations, as opposed to an elevated frequency of carriers. Males and females are equally likely to be diagnosed with the disease. Genetic testing in families with a history of this disorder may be recommended. History
Lipid inclusions in the leukocytes of blood smears were first articulated by G.H. Jordans in 1953.This is a feature of the disease, but he wasn’t necessarily finding the first case of the NLSD disease  Now, having lipid deposits in the white blood cells of the individual is known and recognized as Jordan’s anomaly, due to the medical professional who discovered it. The first case of Neutral Lipid Storage Disease was reported by Maurice Dorfman when he treated two sisters with non bullous ichthyosiform erythroderma in 1974. His observations were then able to be confirmed with the emergence of other cases. Since then, less than 100 cases as of have been reported. See also
List of cutaneous conditions

References
External links
NIH
GARD