Necrolytic migratory erythema
Other names	NME
Necrolytic migratory erythema in the gluteal area
Specialty	Dermatology

Necrolytic migratory erythema is a red, blistering rash that spreads across the skin. It particularly affects the skin around the mouth and distal extremities; but may also be found on the lower abdomen, buttocks, perineum, and groin. It is strongly associated with glucagonoma, a glucagon-producing tumor of the pancreas, but is also seen in a number of other conditions including liver disease and intestinal malabsorption.

Signs and symptoms

Clinical features

NME features a characteristic skin eruption of red patches with irregular borders, intact and ruptured vesicles, and crust formation.^[1] It commonly affects the limbs and skin surrounding the lips, although less commonly the abdomen, perineum, thighs, buttocks, and groin may be affected.^[1] Frequently these areas may be left dry or fissured as a result.^[1] All stages of lesion development may be observed synchronously.^[2] The initial eruption may be exacerbated by pressure or trauma to the affected areas.^[1]

Associated conditions

William Becker first described an association between NME and glucagonoma in 1942^[2][3] and since then, NME has been described in as many as 70% of persons with a glucagonoma.^[4] NME is considered part of the glucagonoma syndrome,^[5] which is associated with hyperglucagonemia, diabetes mellitus, and hypoaminoacidemia.^[2] When NME is identified in the absence of a glucagonoma, it may be considered "pseudoglucagonoma syndrome".^[6] Less common than NME with glucagonoma, pseudoglucagonoma syndrome may occur in a number of systemic disorders:^[7]

• Celiac disease
• Ulcerative colitis
• Crohn's disease
• Hepatic cirrhosis
• Hepatocellular carcinoma
• Lung cancer, including small cell lung cancer
• Tumors that secrete insulin- or insulin-like growth factor 2
• Duodenal cancer

Cause

The cause of NME is unknown, although various mechanisms have been suggested. These include hyperglucagonemia, zinc deficiency, fatty acid deficiency, hypoaminoacidemia, and liver disease.^[2]

Mechanism

The pathogenesis is also unknown.^{[citation needed]}

Diagnosis

Histology

The histopathologic features of NME are nonspecific^[8] and include:^[9]

• epidermal necrosis
• subcorneal pustules
• confluent parakeratosis, epidermal hyperplasia, and marked papillary dermal hyperplasia in a psoriasiform pattern
• angioplasia of papillary dermis
• suppurative folliculitis

The vacuolated, pale, swollen epidermal cells and necrosis of the superficial epidermis are most characteristic.^[2] Immunofluorescence is usually negative.^[2]

Management

Managing the original condition, glucagonoma, by octreotide or surgery. After resection, the rash typically resolves within days.^[10]

See also

• List of cutaneous conditions

References

External links