Medicine:Necrolytic migratory erythema
| Necrolytic migratory erythema | |
|---|---|
| Other names | NME |
| File: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.
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]
- 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
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]
- 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
- ↑ 1.0 1.1 1.2 1.3 "Cutaneous manifestations of internal malignancy". CA – A Cancer Journal for Clinicians 59 (2): 73–98. 2009. doi:10.3322/caac.20005. PMID 19258446.
- ↑ 2.0 2.1 2.2 2.3 2.4 2.5 "Necrolytic migratory erythema: clinicopathologic study of 13 cases". International Journal of Dermatology 43 (1): 12–8. January 2004. doi:10.1111/j.1365-4632.2004.01844.x. PMID 14693015.
- ↑ "Cutaneous manifestations of internal malignant tumors". Archives of Dermatology and Syphilology 45 (6): 1069–1080. 1942. doi:10.1001/archderm.1942.01500120037004.
- ↑ "The glucagonoma syndrome and necrolytic migratory erythema: a clinical review". Eur. J. Endocrinol. 151 (5): 531–7. November 2004. doi:10.1530/eje.0.1510531. PMID 15538929. http://eje-online.org/cgi/pmidlookup?view=long&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. pp. 143. ISBN 978-0-7216-2921-6. https://archive.org/details/andrewsdiseasess00mdwi_659.
- ↑ "Necrolytic migratory erythema without glucagonoma in patients with liver disease". Journal of the American Academy of Dermatology 32 (4): 604–9. April 1995. doi:10.1016/0190-9622(95)90345-3. PMID 7896950.
- ↑ "Small-cell lung cancer and necrolytic migratory erythema". The New England Journal of Medicine 359 (25): 2731–2. December 2008. doi:10.1056/NEJMc0805992. PMID 19092164.
- ↑ 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.
- ↑ "Histologic variation in the skin lesions of the glucagonoma syndrome". The American Journal of Surgical Pathology 10 (7): 445–53. July 1986. doi:10.1097/00000478-198607000-00001. PMID 3014912.
- ↑ 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.
External links
| Classification | |
|---|---|
| External resources |
 |  |
