Necrolytic migratory erythema

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Necrolytic migratory erythema
Other namesNME
Necrolytic migratory erythema.tif
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.

Contents

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]

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]

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

Related Research Articles

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References

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  2. 1 2 3 4 5 6 Pujol RM, Wang CY, el-Azhary RA, Su WP, Gibson LE, Schroeter AL (January 2004). "Necrolytic migratory erythema: clinicopathologic study of 13 cases". International Journal of Dermatology. 43 (1): 12–8. doi:10.1111/j.1365-4632.2004.01844.x. PMID   14693015.
  3. Becker WS, Kahn D, Rothman S (1942). "Cutaneous manifestations of internal malignant tumors". Archives of Dermatology and Syphilology. 45 (6): 1069–1080. doi:10.1001/archderm.1942.01500120037004.
  4. van Beek AP, de Haas ER, van Vloten WA, Lips CJ, Roijers JF, Canninga-van Dijk MR (November 2004). "The glucagonoma syndrome and necrolytic migratory erythema: a clinical review". Eur. J. Endocrinol. 151 (5): 531–7. doi: 10.1530/eje.0.1510531 . PMID   15538929.
  5. Odom, Richard B.; Davidsohn, Israel; James, William D.; Henry, John Bernard; Berger, Timothy G.; Clinical diagnosis by laboratory methods; Dirk M. Elston (2006). Andrews' diseases of the skin: clinical dermatology . Saunders Elsevier. pp.  143. ISBN   978-0-7216-2921-6.
  6. Marinkovich MP, Botella R, Datloff J, Sangueza OP (April 1995). "Necrolytic migratory erythema without glucagonoma in patients with liver disease". Journal of the American Academy of Dermatology. 32 (4): 604–9. doi:10.1016/0190-9622(95)90345-3. PMID   7896950.
  7. Mignogna MD, Fortuna G, Satriano AR (December 2008). "Small-cell lung cancer and necrolytic migratory erythema". The New England Journal of Medicine. 359 (25): 2731–2. doi:10.1056/NEJMc0805992. PMID   19092164.
  8. Wilkinson DS (1973). "Necrolytic migratory erythema with carcinoma of the pancreas". Transactions of the St. John's Hospital Dermatological Society. 59 (2): 244–50. PMID   4793623.
  9. Kheir SM, Omura EF, Grizzle WE, Herrera GA, Lee I (July 1986). "Histologic variation in the skin lesions of the glucagonoma syndrome". The American Journal of Surgical Pathology. 10 (7): 445–53. doi:10.1097/00000478-198607000-00001. PMID   3014912. S2CID   19879900.
  10. Compton, Nicholas L.; Chien, Andy J. (May 2013). "A Rare but Revealing Sign: Necrolytic Migratory Erythema". The American Journal of Medicine. 126 (5): 387–389. doi:10.1016/j.amjmed.2013.01.012. PMID   23477490.