skin disorder Cholestasis-lymphedema syndrome OMIM:214900 ICD10:Q82.0 Lymphedema cholestasis syndrome (LCS) is a rare genetic disorder characterized by neonatal intrahepatic cholestasis, often lessening and becoming intermittent with age, and severe chronic lymphedema which mainly affects the lower limbs. Patients often present fat malabsorption leading to failure to thrive, fat soluble vitamin deficiency with bleeding, rickets, neuropathy. In 25% of cases, cirrhosis occurs, during childhood or later in life. orphanet Aagenaes syndrome Rare genetic hepatic disease