Putting the pieces together: necrolytic migratory erythema and the glucagonoma syndrome

Abstract

Glucagonomas are slow-growing, rare pancreatic neuroendocrine tumors. They may present with paraneoplastic phenomena known together as the "glucagonoma syndrome." A hallmark sign of this syndrome is a rash known as necrolytic migratory erythema (NME). In this paper, the authors describe a patient with NME and other features of the glucagonoma syndrome. The diagnosis of this rare tumor requires an elevated serum glucagon level and imaging confirming a pancreatic tumor. Surgical and medical treatment options are reviewed. When detected early, a glucagonoma is surgically curable. It is therefore imperative that clinicians recognize the glucagonoma syndrome in order to make an accurate diagnosis and refer for treatment.

Figure 1.

Photo from dermatology clinic showing friable, erosive, erythematous plaques with crusting and scale on the lower back and gluteal region.

Figure 2.

Photo from dermatology clinic showing papules along the corners of the mouth.

Figure 3.

Photo on admission to the hospital showing acute left lower extremity cellulitis, as well as erythematous thin plaques with scale present on the right lower extremity and scattered hyperpigmented patches at the site of prior disease activity.

Figure 4.

Photo on post-operative day 3 showing resolution of cellulitis, as well as evidence of healing of the NME (for example lateral aspect of the right shin and dorsum of right foot).