What is the relationship between necrolytic migratory erythema (NME) and glucagonoma, a rare tumor of the pancreas that produces excess glucagon (Glucagon)?

From the Guidelines

Necrolytic migratory erythema (NME) from glucagonoma is best managed with surgical resection of the underlying tumor when possible, and symptomatic treatment with zinc supplementation, topical corticosteroids, and somatostatin analogs for metastatic disease. The skin manifestations of NME result from hyperglucagonemia causing metabolic derangements, particularly affecting protein metabolism, leading to hypoaminoacidemia and subsequent epidermal protein breakdown 1. For patients with recent-onset diabetes, cachexia, and/or a necrolytic erythematous skin rash, a blood test for glucagon and blood glucose, multiphase contrast-enhanced CT or MRI, and Octreoscan are recommended as appropriate diagnostic tests 2.

Some key points to consider in the management of NME from glucagonoma include:

• Surgical resection is the optimal treatment for locoregional pancreatic endocrine tumors, including glucagonoma 1
• Symptomatic management of the skin manifestations with zinc supplementation (220 mg oral zinc sulfate daily) is recommended as zinc deficiency often contributes to the skin findings 3
• Topical treatments including emollients, medium to high-potency corticosteroids (such as triamcinolone 0.1% or clobetasol 0.05% applied twice daily), and wound care for erosions can provide symptomatic relief
• Essential amino acid supplementation may also help improve the skin condition
• For patients with metastatic disease where surgery isn't an option, somatostatin analogs like octreotide (100-500 mcg subcutaneously 2-3 times daily or long-acting formulations) can dramatically improve skin symptoms by inhibiting glucagon secretion 3

It's also important to note that preoperative management of patients with glucagonoma should include treatment of hyperglycemia and diabetes, including the use of intravenous fluids, and consideration of perioperative anticoagulation due to the increased risk of pulmonary emboli 4.

From the Research

Definition and Characteristics of Necrolytic Migratory Erythema

• Necrolytic migratory erythema (NME) is a rare skin disorder that is a cutaneous manifestation of the glucagonoma syndrome 5, 6, 7.
• It presents with annular eruptions of migrating erythematous papules and plaques with superficial epidermal necrosis, central flaccid bullae, and crusted erosions located primarily in the intertriginous areas 7.
• NME is usually a specific and often initial finding of glucagonoma syndrome, but it may occur in other settings unassociated with an alpha-cell pancreatic tumour (pseudoglucagonoma syndrome) 5.

Diagnosis and Treatment

• Prompt recognition of NME and subsequent workup for a glucagonoma can allow for an earlier diagnosis and enhance the chances of a favourable outcome 5, 6.
• Surgical resection is the definitive treatment for glucagonoma syndrome, although chemotherapeutic agents, somatostatin analogues and radionuclide therapy are also employed 5, 6.
• Treatment with the long-acting somatostatin analog Octreotide is a potential therapy to help ameliorate skin symptoms 7, 8.
• Administration of corticoids, aminoacids, zinc or essential fatty acids can be helpful in addition to glucagonoma treatments 9.

Clinical Presentation and Prognosis

• NME is often the first clinical finding of an occult neuroendocrine pancreatic neoplasia 6.
• The clinical and microscopical diagnosis is complex, and NME waxes and wanes by successive relapses and remissions 9.
• Early recognition of glucagonoma syndrome before liver metastases can be life-saving, as metastases occur late 5, 6.