Necrolytic migratory erythema

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(Redirected from
Glucagonoma syndrome
)

Necrolytic migratory erythema
Other namesNME
Necrolytic migratory erythema in the gluteal area
SpecialtyDermatology

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

intestinal malabsorption
.

Signs and symptoms

Clinical features

NME features a characteristic skin eruption of red

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

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]

Cause

The cause of NME is unknown, although various mechanisms have been suggested. These include hyperglucagonemia,

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

  1. ^
    PMID 19258446
    .
  2. ^
    S2CID 26012738
    .
  3. doi:10.1001/archderm.1942.01500120037004
    .
  4. PMID 15538929
    .
  5. ISBN 978-0-7216-2921-6
    .
  6. PMID 7896950
    .
  7. PMID 19092164
    .
  8. PMID 4793623
    .
  9. S2CID 19879900
    .
  10. PMID 23477490
    .

External links

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