Necrolytic migratory erythema

Necrolytic migratory erythema (NME) is a red, blistering rash that spreads across the skin, particularly 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.

Appearance

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]

Histology

The histopathologic features of NME are nonspecific^[3] and include:^[4]

• epidermal necrosis
• subcorneal pustules
• confluent parakeratosis, epidermal hyperlpasia, 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]

Cause

The etiology of NME is unknown, although various mechanisms have been suggested. These include hyperglucagonemia, zinc deficiency, fatty acid deficiency, hypoaminoacidemia, and liver disease.^[2] In addition to the etiology being unknown, the pathogenesis is also unknown.

Associated conditions

William Becker first described an association between NME and glucagonoma in 1942^[2][5] and since then, NME has been described in as many as 70% of individuals with a glucagonoma.^[6] NME is considered part of the glucagonoma syndrome,^[7] 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".^[8] Less common than NME with glucagonoma, pseudoglucagonoma syndrome may occur in a number of systemic disorders:^[9]

• 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

See also

• List of cutaneous conditions

References