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. 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
Crohns 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.
Patients with metastatic EMPD only survive for a median of 1.5 years, and have a 7% 5-year survival rate. Prognosis
Prognosis is generally good, but factors such as depth of invasion and duration of disease need to be considered. In primary EMPD, if invasion of the underlying tissue is non-existent or even minimal, treatment options are more likely to be effective, however, if there are signs that the disease has metastasised, the prognosis is usually poor. Epidemiology
EMPD is most prevalent in Caucasian women and Asian men over the age of 60. The invasive form occurs in 5–25% of all EMPD patients and 17–30% of the cases involve an underlying adenocarcinoma. 10-20% of EMPD is of the secondary form. Approximately 10% of patients develop invasive adenocarcinoma that may progress to metastatic disease.The disease affects regions that are rich in apocrine secretions. 65% EMPD occurs at the vulva, followed by 15% at perianal areas and 14% at the male genitalia. In terms of the vulva, the labia majora is the site that is most often involved, followed by labia minora, clitoris and perineum. EMPD originating at the vulva can spread to the upper vaginal mucosa and cervix. Other areas where EMPD can be found, although very rarely, include: the axillae, eyelids, external auditory canal, umbilical region, trunk and extremities. History
The first case of Pagets disease was described by James Paget in 1874.