Two sisters and two unrelated patients with an ichthyosiform dermatosis resembling
nonbullous ichthyosiform erythroderma were found to have lipid accumulations in the
granulocytes of the peripheral blood, in the granulocyte precursors in the bone marrow, and
in the liver. In each case, the blood anomaly was readily detected on examining a peripheral
smear. One patient showed many of the features seen in Refsum syndrome. This
association of findings in four patients may constitute another neurocutaneous lipidosis.