Necrolytic migratory erythema explained

Necrolytic migratory erythema
Field:dermatology
Synonyms:NME

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. 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]

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.

Diagnosis

Histology

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

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

Notes and References

  1. Thiers BH, Sahn RE, Callen JP . Cutaneous manifestations of internal malignancy . . 59 . 2 . 73–98 . 2009 . 19258446 . 10.3322/caac.20005. free .
  2. Pujol RM, Wang CY, el-Azhary RA, Su WP, Gibson LE, Schroeter AL . Necrolytic migratory erythema: clinicopathologic study of 13 cases . International Journal of Dermatology . 43 . 1 . 12–8 . January 2004 . 14693015 . 10.1111/j.1365-4632.2004.01844.x. 26012738 .
  3. Becker WS, Kahn D, Rothman S . Cutaneous manifestations of internal malignant tumors . Archives of Dermatology and Syphilology . 45 . 6 . 1069–1080 . 1942 . 10.1001/archderm.1942.01500120037004.
  4. van Beek AP, de Haas ER, van Vloten WA, Lips CJ, Roijers JF, Canninga-van Dijk MR . The glucagonoma syndrome and necrolytic migratory erythema: a clinical review . Eur. J. Endocrinol. . 151 . 5 . 531–7 . November 2004 . 15538929 . 10.1530/eje.0.1510531. free .
  5. Book: Odom, Richard B. . Davidsohn, Israel . James, William D. . Henry, John Bernard . Berger, Timothy G. . Clinical diagnosis by laboratory methods . Dirk M. Elston . Andrews' diseases of the skin: clinical dermatology . limited . Saunders Elsevier . 2006 . 143 . 978-0-7216-2921-6 .
  6. Marinkovich MP, Botella R, Datloff J, Sangueza OP . Necrolytic migratory erythema without glucagonoma in patients with liver disease . Journal of the American Academy of Dermatology . 32 . 4 . 604–9 . April 1995 . 7896950 . 10.1016/0190-9622(95)90345-3.
  7. Mignogna MD, Fortuna G, Satriano AR . Small-cell lung cancer and necrolytic migratory erythema . The New England Journal of Medicine . 359 . 25 . 2731–2 . December 2008 . 19092164 . 10.1056/NEJMc0805992 . free .
  8. Wilkinson DS . Necrolytic migratory erythema with carcinoma of the pancreas . Transactions of the St. John's Hospital Dermatological Society . 59 . 2 . 244–50 . 1973 . 4793623 .
  9. Kheir SM, Omura EF, Grizzle WE, Herrera GA, Lee I . Histologic variation in the skin lesions of the glucagonoma syndrome . The American Journal of Surgical Pathology . 10 . 7 . 445–53 . July 1986 . 3014912 . 10.1097/00000478-198607000-00001. 19879900 .
  10. Compton . Nicholas L. . Chien . Andy J. . A Rare but Revealing Sign: Necrolytic Migratory Erythema . The American Journal of Medicine . May 2013 . 126 . 5 . 387–389 . 10.1016/j.amjmed.2013.01.012. 23477490 . free .

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