Clinical Manifestations

NME is the hallmark finding in glucagonoma syndrome. It is characterized by a polymorphous eruption that most commonly presents as scaly, erythematous papules and plaques with superficial erosions. The lesions typically are in a perioral distribution on the face (Fig. 9), but also involve the perineum, lower abdomen, thighs, buttocks, and less commonly the distal extremities. NME has been reported to be the presenting complaint in approximately two-thirds of patients with this tumor, but there are only rare cases of glucagonoma without NME ever occurring. Stomatitis, glossitis, dystrophic nails, and alopecia can also be seen. The eruption is frequently misdiagnosed as seborrheic dermatitis or intertrigo and can also resemble pemphigus foliaceus, acrodermatitis enteropathica, chronic mucocutaneous candidiasis, or psoriasis.

Weight loss has been reported to be the most common initial symptom of glucagonoma (71%), followed by NME (67%), and diabetes (38%) (19).

FIGURE 9 Perioral erythema and scale with superficial erosions and crusting characteristic of necrolytic migratory erythema. Courtesy of Dr. Kristina Callis, University of Utah.

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