Necrolytic migratory erythema

Signs and symptoms

Clinical features

NME features a characteristic skin eruption of red patches with irregular borders, intact and ruptured vesicles, and crust formation. It commonly affects the limbs and skin surrounding the lips, although less commonly the abdomen, perineum, thighs, buttocks, and groin may be affected. Frequently these areas may be left dry or fissured as a result. All stages of lesion development may be observed synchronously. The initial eruption may be exacerbated by pressure or trauma to the affected areas.

Associated conditions

William Becker first described an association between NME and glucagonoma in 1942 and since then, NME has been described in as many as 70% of persons with a glucagonoma. NME is considered part of the glucagonoma syndrome, which is associated with hyperglucagonemia, diabetes mellitus, and hypoaminoacidemia. When NME is identified in the absence of a glucagonoma, it may be considered "pseudoglucagonoma syndrome". Less common than NME with glucagonoma, pseudoglucagonoma syndrome may occur in a number of systemic disorders:

• 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.

Mechanism

The pathogenesis is also unknown.

Diagnosis

Histology

The histopathologic features of NME are nonspecific and include:

• 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. Immunofluorescence is usually negative.

Management

Managing the original condition, glucagonoma, by octreotide or surgery. After resection, the rash typically resolves within days.

References

References

1. (2009). "Cutaneous manifestations of internal malignancy". [[CA – A Cancer Journal for Clinicians]].
2. (January 2004). "Necrolytic migratory erythema: clinicopathologic study of 13 cases". International Journal of Dermatology.
3. (1942). "Cutaneous manifestations of internal malignant tumors". Archives of Dermatology and Syphilology.
4. (November 2004). "The glucagonoma syndrome and necrolytic migratory erythema: a clinical review". Eur. J. Endocrinol..
5. Odom, Richard B.. (2006). "Andrews' diseases of the skin: clinical dermatology". Saunders Elsevier.
6. (April 1995). "Necrolytic migratory erythema without glucagonoma in patients with liver disease". Journal of the American Academy of Dermatology.
7. (December 2008). "Small-cell lung cancer and necrolytic migratory erythema". The New England Journal of Medicine.
8. Wilkinson DS. (1973). "Necrolytic migratory erythema with carcinoma of the pancreas". Transactions of the St. John's Hospital Dermatological Society.
9. (July 1986). "Histologic variation in the skin lesions of the glucagonoma syndrome". The American Journal of Surgical Pathology.
10. (May 2013). "A Rare but Revealing Sign: Necrolytic Migratory Erythema". The American Journal of Medicine.