Armanni–Ebstein Lesions: A Need for Clarification

Abstract

Armanni-Ebstein lesions were first described by Luciano Armanni, a pathologist at the University of Naples, during autopsy studies undertaken in 1872, as a unique vacuolar nephropathy associated with poorly controlled diabetes that involves selective renal epithelial cell glycogen accumulation. However, within the last two decades, a broader range of vacuolar changes, including lipid deposition, have also been termed Armanni-Ebstein (AE) lesions, creating some confusion on possible etiology. We would suggest that the term AE phenomenon would be best reserved for the original clear cell change associated with glycogen deposition, and that this should be clearly distinguished from subnuclear lipid vacuolization ("basal vacuolization"). Although there is obvious inter-relation between these two types of vacuoles, they appear morphologically and biochemically distinct from each other. More precise classification may assist in clarifying the causal processes and possible diagnostic significance of different types of renal epithelial vacuolization at autopsy.

Septic Ketoacidosis—A Potentially Lethal Entity with Renal Tubular Epithelial Vacuolization

“…These vacuoles contain lipid and appear as a single row beneath the nucleus in contact with the basement membrane, with possible luminal displacement of nuclei. These vacuoles have recently been identified as a separate phenomenon from the Armanni-Ebstein lesion (27), to which they have previously been referred (28). Their presence has been reported in diabetic, alcoholic, and starvation ketoacidosis (20,29,30).…”

Renal Tubular Epithelial Vacuoles—A Marker for Both Hyperlipidemia and Ketoacidosis at Autopsy

“…Renal tubular epithelial cells display varying patterns of vacuolization in response to different metabolic stimuli. Although basal vacuolization has been referred to as “Armanni–Ebstein” lesions due to the association with diabetic ketoacidosis, the two entities should be separated as they most likely have different constituents and etiology . The pathogenesis of Armanni–Ebstein lesions due to hyperglycemia has been clearly established in animal models ; however, the pathogenesis of basal vacuolization remains unclear.…”

“…A variety of metabolic derangements cause pathological changes in renal tubular epithelial cells. Very recently, Armanni–Ebstein phenomenon and basal vacuolization have been distinguished from each other due to different morphologies and probable distinct etiologies . While the pathogenesis of Armanni–Ebstein lesions has been clearly attributed to hyperglycemia , the exact cause of basal vacuolization remains unclear; basal lipid vacuolization has been reported in ketoacidosis due to diabetes, alcoholism, and starvation .…”

Armanni–Ebstein Lesions in Terminal Hyperglycemia

“…Armanni–Ebstein lesions have also been reported in five autopsy cases of severe Fanconi syndrome with glucosuria and death in infancy; however, again the blood glucose levels were not presented . Additionally, many of the more recent forensic studies and case reports of Armanni–Ebstein lesions refer to basal lipid vacuolization of renal tubular epithelial cells , which we now recognize as distinct from the Armanni–Ebstein phenomenon . Therefore, this study was undertaken to investigate the incidence of traditional Armanni–Ebstein lesions in terminal hyperglycemia, its correlation with vitreous glucose levels, and to identify potential factors which may have contributed to its pathogenesis.…”