Their annual incidence has been estimated at 1 in 20 million. Pseudoglucagonoma has been described in an even smaller group of patients. Data on incidence and prevalence are not currently available for pseudoglucagonoma. The remaining 12 patients were alive after an average follow-up interval of 3.7 years. The mortality rate associated with the pseudoglucagonoma syndrome generally follows that of the underlying pathologic diagnosis.
Some cases resulting from celiac sprue are completely reversible by dietary modification.
Others, such as those resulting from hepatic cirrhosis or other neoplasm, generally have a poor outcome. Males and females appear to be equally affected. These patients usually present with non-specific complaints such as weight loss, diabetes, diarrhea and stomatitis. Unexplained weight loss and the onset of necrolytic migratory erythema, especially with new-onset diabetes mellitus, often hastens the correct diagnosis. Still, because milder, early lesions may exhibit only subtle changes in histology and because inadequate sampling can miss the diagnostic changes, the skin eruption itself is often interpreted as a non-specific dermatitis.