Necrolytic Migratory Erythema With Recalcitrant Dermatitis as the Only Presenting Symptom

Diagnosis of glucagonomas often is delayed due to their rarity and lack of classical signs and symptoms. The distribution of the lesions seen in necrolytic migratory erythema (NME) usually involves the inguinal crease, perineum, lower extremities, buttocks, and other intertriginous areas.¹ Our patient had involvement in the typical distribution but also had involvement of the scalp, face, and upper body. The typical histology for NME is crusted psoriasiform dermatitis with a tendency for the upper epidermis to have necrosis and a vacuolated pale epidermis.² Our patient’s histologic findings were less specific showing epidermal spongiosis with a scant lymphocytic infiltrate and at times acanthosis. The lack of classical skin findings and histology delayed diagnosis. In more than 50% of patients, metastasis has already occurred by the time the patient is diagnosed.³ Treatment is aimed at complete removal of the pancreatic tumor, which typically leads to a rapid improvement in symptoms. For patients unable to undergo surgery, chemotherapy agents and octreotide are used; unfortunately, symptoms may persist.⁴ The response to azathioprine in our patient suggests it is a possible alternate therapy for those with persistent NME.

This patient highlights the difficulty of diagnosing a glucagonoma when the only clinical manifestation may be NME. Moreover, skin biopsies that can sometimes be diagnostic may be nonspecific. This patient also shows a potential benefit of azathioprine in the treatment of NME.