| Necrolytic migratory erythema |
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| DiseasesDB |
8833 |
| eMedicine |
derm/168 |
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Necrolytic migratory erythema (NME) 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
- 1 Appearance
- 1.1 Clinical features
- 1.2 Histology
- 2 Cause
- 3 Associated conditions
- 4 See also
- 5 References
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 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]
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] 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
- ^ 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.
- ^ 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.
- ^ 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.
- ^ 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.
- ^ 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.
- ^ 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.
- ^ 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. p. 143. ISBN 0-7216-2921-0.
- ^ 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.
- ^ 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.
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Urticaria and erythema (L50–L54, 695, 708)
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Urticaria
(acute/chronic) |
| Allergic urticaria |
- Urticarial allergic eruption
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| Physical urticaria |
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- Urticaria-like follicular mucinosis
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| Erythema |
Erythema multiforme/
drug eruption |
- Erythema multiforme minor
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- panniculitis (Erythema nodosum)
- Acute generalized exanthematous pustulosis
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| Figurate erythema |
- Erythema annulare centrifugum
- Erythema marginatum
- Erythema migrans
- Erythema gyratum repens
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| Other erythema |
- Necrolytic migratory erythema
- Erythema toxicum
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- Generalized erythema
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Paraneoplastic syndromes
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- Hypercalcaemia
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| Musculoskeletal |
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| Mucocutaneous |
| reactive erythema |
- Erythema gyratum repens
- Necrolytic migratory erythema
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| papulosquamous |
- Acanthosis nigricans
- Ichthyosis acquisita
- Acrokeratosis paraneoplastica of Bazex
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- Florid cutaneous papillomatosis
- Leser-Trélat sign
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| Other |
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