It particularly affects the skin around the mouth and distal extremities; but may also be found on the lower abdomen, buttocks, perineum, and groin.

It is strongly associated with glucagonoma, a glucagon-producing tumor of the pancreas, but is also seen in a number of other conditions including liver disease and intestinal malabsorption.

NME features a characteristic skin eruption of red patches with irregular borders, intact and ruptured vesicles, and crust formation.

[4] NME is considered part of the glucagonoma syndrome,[5] which is associated with hyperglucagonemia, diabetes mellitus, and hypoaminoacidemia.

[citation needed] The histopathologic features of NME are nonspecific[8] and include:[9] The vacuolated, pale, swollen epidermal cells and necrosis of the superficial epidermis are most characteristic.