Necrolytic Migratory Erythema is the Commonest Cutaneous Manifestation of Glucagonoma
Necrolytic migratory erythema (NME) is the characteristic and most common cutaneous manifestation of glucagonoma syndrome, serving as a critical diagnostic clue for this rare pancreatic neuroendocrine tumor. 1, 2, 3
Clinical Presentation of NME
NME presents with distinctive features:
- Annular eruptions of migrating erythematous papules and plaques
- Superficial epidermal necrosis
- Central flaccid bullae and crusted erosions
- Predominantly affects intertriginous areas, extremities, and face 3, 4
- Often pruritic with active borders, central scaling, and postinflammatory hyperpigmentation 4
- May also present with erosions, pustules, and crusted lesions 4
Diagnostic Significance
NME is particularly important in the diagnostic pathway for glucagonoma for several reasons:
- It is often the initial and specific finding of glucagonoma syndrome 2
- The rash frequently precedes diagnosis by months to years, with an average time from symptom onset to diagnosis of 14 months 2, 5
- Early recognition of NME can lead to earlier diagnosis before liver metastases develop, significantly improving outcomes 2
Pathophysiology and Histopathology
Histopathologically, NME shows:
- Dyskeratosis in all layers of the epidermis as an early feature
- Long-standing lesions typically display psoriasiform hyperplasia
- Pallor and necrosis of upper epidermal layers 4
Glucagonoma Syndrome
NME occurs as part of the classic triad of glucagonoma syndrome:
- Necrolytic migratory erythema
- Recent-onset diabetes mellitus (occurs in 75-80% of patients)
- Cachexia/weight loss 2
Management Considerations
When NME is identified:
- Immediate measurement of fasting glucagon levels (typically >500 pg/mL; normal range: 50-150 pg/mL) 2
- Multiphase contrast-enhanced CT or MRI for tumor localization 2
- Somatostatin receptor scintigraphy (Octreoscan) to determine receptor status and guide therapy 2
- Treatment with octreotide/lanreotide can help ameliorate skin symptoms 1, 3
- Surgical resection is the definitive treatment for localized disease 2
Clinical Pearls
- NME may have a relapsing and remitting course, even when the underlying pancreatic tumor is stable and the patient is undergoing treatment 3
- The rash can dramatically improve with octreotide treatment 3
- Peptide receptor radionuclide therapy has shown promise in treating glucagonoma-related NME in patients with metastatic disease 6
- Awareness of this characteristic rash among physicians and dermatologists is crucial for early diagnosis 5
The recognition of NME as the hallmark cutaneous manifestation of glucagonoma is essential for timely diagnosis and treatment of this rare but potentially curable pancreatic neuroendocrine tumor when identified early.