Primary atrophy of the pancreas is an unusual pancreatic disease characterized pathologically by almost complete disappearance of the acinar cells and by disappearance of the islets of Langerhans to a lesser extent. Fatty replacement varying from mild fatty infiltration to massive replacement of the organ by adipose tissue may accompany this atrophy. No specific causative factors have been demonstrated. The possible relationship to other systemic diseases, to malnutrition and dietary deficiencies, or to an unusual form of chronic pancreatitis has been considered.

The characteristic clinical state is one of a deficiency syndrome associated with steatorrhea, loss of weight, normal or increased appetite, and occasionally edema, anasarca, and diabetes mellitus. Although the disease may possibly develop in childhood, it appears to be more frequent after the fifth decade of life. Surgical exploration of the pancreas may be necessary to establish the diagnosis.

Recognition of this syndrome is important since it represents a benign pancreatic disease that responds to adequate replacement therapy.