Necrolytic migratory erythema

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Necrolytic migratory erythema - Wikipedia

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Necrolytic migratory erythema
Other names	NME

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]

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. S2CID 26012738.
^ 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. pp. 143. ISBN 978-0-7216-2921-6.
^ 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.
^ 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.
^ 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. S2CID 19879900.
^ 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

Urticaria and erythema

Urticaria
(acute/chronic)

Allergic urticaria

Urticarial allergic eruption

Physical urticaria

Cold urticaria Familial Primary cold contact urticaria Secondary cold contact urticaria Reflex cold urticaria
Heat urticaria Localized heat contact urticaria Solar urticaria
Dermatographic urticaria Vibratory angioedema Pressure urticaria Delayed pressure urticaria Cholinergic urticaria Aquagenic urticaria

Other urticaria

Angioedema

Erythema

Erythema multiforme/ drug eruption	Erythema multiforme minor Erythema multiforme major Stevens–Johnson syndrome Toxic epidermal necrolysis panniculitis (Erythema nodosum) Acute generalized exanthematous pustulosis
Figurate erythema	Erythema annulare centrifugum Erythema marginatum Erythema migrans Erythema gyratum repens Annular erythema of infancy
Other erythema	Necrolytic migratory erythema Erythema toxicum Erythroderma Palmar erythema Generalized erythema Necrolytic acral erythema

Paraneoplastic syndromes

Reactive erythema	Erythema gyratum repens Necrolytic migratory erythema
Papulosquamous	Acanthosis nigricans Ichthyosis acquisita Acrokeratosis paraneoplastica of Bazex Extramammary Paget's disease Florid cutaneous papillomatosis Leser-Trélat sign Pityriasis rotunda Tripe palms
Other	Febrile neutrophilic dermatosis Pyoderma gangrenosum Paraneoplastic pemphigus

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Classification	D MeSH: D058568 DiseasesDB: 8833 SNOMED CT: 15576007
External resources	eMedicine: derm/168