Necrolytic migratory erythema

Necrolytic migratory erythema
Classification and external resources
DiseasesDB	8833
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]

• 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

1. ^ ^{a b c d} Thiers BH, Sahn RE, Callen JP (2009). "Cutaneous manifestations of internal malignancy". CA: a Cancer Journal for Clinicians 59 (2): 73–98. doi:10.3322/caac.20005. PMID 19258446. 
2. ^ ^{a b c d e f} 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. http://www3.interscience.wiley.com/resolve/openurl?genre=article&sid=nlm:pubmed&issn=0011-9059&date=2004&volume=43&issue=1&spage=12. 
3. ^ 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. 
4. ^ 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. 
5. ^ Becker WS, Kahn D, Rothman S (1942). "Cutaneous manifestations of internal malignant tumors". Archives of Dermatology and Syphilology 45 (6): 1069–1080. 
6. ^ 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. http://eje-online.org/cgi/pmidlookup?view=long&pmid=15538929. 
7. ^ 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 0-7216-2921-0. 
8. ^ 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. http://linkinghub.elsevier.com/retrieve/pii/0190-9622(95)90345-3. 
9. ^ 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.