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 The perineum (: perineums or perinea) in placentalia, placental mammals is the space between the anus and the genitals. The human perineum is between the anus and scrotum in the male or between the anus and vulva in the female. The perineum is ...

, and groin. It is strongly associated with glucagonoma, a glucagon-producing tumor of the

pancreas The pancreas (plural pancreases, or pancreata) is an Organ (anatomy), organ of the Digestion, digestive system and endocrine system of vertebrates. In humans, it is located in the abdominal cavity, abdomen behind the stomach and functions as a ...

, but is also seen in a number of other conditions including

liver disease Liver disease, or hepatic disease, is any of many diseases of the liver. If long-lasting it is termed chronic liver disease. Although the diseases differ in detail, liver diseases often have features in common. Liver diseases File:Ground gla ...

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,

, 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 Diabetes mellitus, commonly known as diabetes, is a group of common endocrine diseases characterized by sustained high blood sugar levels. Diabetes is due to either the pancreas not producing enough of the hormone insulin, or the cells of th ...

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 Coeliac disease (British English) or celiac disease (American English) is a long-term autoimmune disorder, primarily affecting the small intestine. Patients develop intolerance to gluten, which is present in foods such as wheat, rye, spel ...

Ulcerative colitis Ulcerative colitis (UC) is one of the two types of inflammatory bowel disease (IBD), with the other type being Crohn's disease. It is a long-term condition that results in inflammation and ulcers of the colon and rectum. The primary sympto ...

Crohn's disease Crohn's disease is a type of inflammatory bowel disease (IBD) that may affect any segment of the gastrointestinal tract. Symptoms often include abdominal pain, diarrhea, fever, abdominal distension, and weight loss. Complications outside of the ...

* Hepatic cirrhosis * Hepatocellular carcinoma *

Lung cancer Lung cancer, also known as lung carcinoma, is a malignant tumor that begins in the lung. Lung cancer is caused by genetic damage to the DNA of cells in the airways, often caused by cigarette smoking or inhaling damaging chemicals. Damaged ...

, including small cell lung cancer * Tumors that secrete

insulin Insulin (, from Latin ''insula'', 'island') is a peptide hormone produced by beta cells of the pancreatic islets encoded in humans by the insulin (''INS)'' gene. It is the main Anabolism, anabolic hormone of the body. It regulates the metabol ...

- 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 In chemistry, in particular in biochemistry, a fatty acid is a carboxylic acid with an aliphatic chain, which is either saturated and unsaturated compounds#Organic chemistry, saturated or unsaturated. Most naturally occurring fatty acids have an ...

deficiency, hypoaminoacidemia, and

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

External links

{{Paraneoplastic syndromes Erythemas Rare diseases