Glucagonoma syndrome

Necrolytic migratory erythema
Classification and external resources
DiseasesDB eMedicine derm/168

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]

See also

References

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