NME classically presents with migrating, scaly, annular, and erythematous papules, patches, and plaques with superficial necrosis leading to erosions and bullae classically located in intertriginous areas. Pruritus and burning of the skin have been reported. There is a waxing and waning course. Lesions may improve spontaneously and reappear after some months. The pathophysiology of NME is not yet understood but is likely multifactorial, involving excess glucagon and deficiency of amino acid, zinc, and free fatty acids.
Weight loss, abdominal pain, diarrhea, and fever may accompany the glucagonoma. Clinical improvement or resolution of cutaneous lesions is expected after surgical excision or surgical debulking of the pancreatic lesion. Some patients with NME have normal glucagon levels and/or absence of glucagonoma, and these cases may be associated with conditions such as inflammatory bowel disease, chronic liver disease, pancreatitis, nonpancreatic malignancies, and disorders of malabsorption.
D37.8 – Neoplasm of uncertain behavior of other specified digestive organs
16424000 – Glucagonoma syndrome
Differential Diagnosis & Pitfalls
- Immunobullous diseases such as pemphigus vulgaris, IgA pemphigus, bullous pemphigoid, and paraneoplastic pemphigus should be considered, but in glucagonoma, there are no immunoreactants.
- Subacute cutaneous lupus erythematosus
- Acquired acrodermatitis enteropathica
- Nutritional deficiencies (eg, pellagra, biotin deficiency)
- Drug eruption
- Necrolytic acral erythema
- Figurate erythemas, such as deep gyrate erythema, erythema marginatum, or erythema gyratum repens