Abstract A 55-year-old man presented with an 11-year history of necrolytic migratory erythema and glossitis. [ncbi.nlm.nih.gov]

• Pain

  Sometimes, the various clinical presentations can be misleading, but the presence of a painful scaly erythematous rash (Figure 1) is one of the first signs of onset. [omicsonline.org]

  She had been experiencing weight loss, polydipsia, polyphagia, postprandial emesis, excessive hair loss and abdominal pain for two months. [ncbi.nlm.nih.gov]

• Inflammation

  The early lesions revealed superficial perivascular inflammation in the dermis, minor epidermal spongiosis, and scattered dyskeratotic cells in the upper epidermis. [ncbi.nlm.nih.gov]

• Overeating

  Because of apparent responsiveness to these medications, the diagnosis of necrolytic migratory erythema was not considered, and the diagnosis of pancreatic carcinoma was delayed for over one year. [ncbi.nlm.nih.gov]

• Abdominal Pain

  She had been experiencing weight loss, polydipsia, polyphagia, postprandial emesis, excessive hair loss and abdominal pain for two months. [ncbi.nlm.nih.gov]

• Polydipsia

  She had been experiencing weight loss, polydipsia, polyphagia, postprandial emesis, excessive hair loss and abdominal pain for two months. [ncbi.nlm.nih.gov]

• Eruptions

  Abstract Necrolytic migratory erythema is the distinctive cutaneous eruption seen with glucagon-producing tumours of the pancreas. Recognition of this eruption is important because it may lead to the early diagnosis of a glucagonoma. [ncbi.nlm.nih.gov]

  Simple pruritus and prurigo-like eruptions with hyperpigmentation have been observed frequently in cases of internal malignant tumor. [doi.org]

• Ulcer

  We present a case of a patient with a 1-year history of a pancreatic glucagonoma that developed an ulcerated, plaque-like, weeping rash over multiple areas of their body despite current treatment with Octreotide and stable pancreatic tumor staging. [ncbi.nlm.nih.gov]

  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 [en.wikipedia.org]

• Skin Rash

  Abstract Necrolytic migratory erythema is the distinctive skin rash of the glucagonoma syndrome. Its presence is virtually pathognomonic of a glucagon-producing pancreatic islet cell neoplasm. [ncbi.nlm.nih.gov]

  Typical symptoms include fever, headache, fatigue, and as illustrated here, the characteristic skin rash called erythema migrans. If left untreated, infection can spread to joints, the heart, and the nervous system. [farmacosalud.com]

• Mucocutaneous Rash

  Abstract Necrolytic migratory erythema is a rare paraneoplastic dermatosis that may be the first clinical manifestation of the glucagonoma syndrome, a disorder characterized by mucocutaneous rash, glucose intolerance, hypoaminoacidemia, hyperglucagonaemia [ncbi.nlm.nih.gov]

• Colitis

  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 [en.wikipedia.org]

Treatment with the long-acting somatostatin analog Octreotide is a potential therapy to help ameliorate skin symptoms. [ncbi.nlm.nih.gov]

Early recognition of NME can lead to possible cure, while delayed identification of the disease is associated with metastatic disease and a poor prognosis for the patient. [ncbi.nlm.nih.gov]

Saint-Denis - Bobigny Rapporté par Régis Cohen (AP-HP, Avicenne) d’après les communications : S15- Progression et prognosis RT. Jensen S16- Surgical treatment options in the managements of MEN 1 pancreatic zed duodenal neoplastic disease. N. [mediscoop.net]

At least 50% of these tumors are metastatic at the time of diagnosis and, therefore, generally carry a poor prognosis. [emedicine.com]

METHODS: We performed a PubMed review of the literature on the etiology and pathogenesis of NME to identify case reports and reviews published in both the internal medicine and dermatology literature. [ncbi.nlm.nih.gov]

There exist several etiological hypotheses for NME. These are based on modifications of pancreatic enzyme activities and on variations of aminoacids, fatty acids, zinc or glucagon concentrations. Author, co-author : Dandrifosse, J. F. [orbi.uliege.be]

Etiology Hyperglucagonemia can be found as part of a polyfunctional endocrine tumor or an exclusively glucagon-producing tumor. The tumor may be part of a clinical syndrome (eg, Zollinger-Ellison syndrome ), or it may be asymptomatic. [emedicine.com]

Epidemiology Frequency Glucagonomas are quite rare; since 1942, more than 300 cases have been reported worldwide. Their annual incidence has been estimated at 1 in 20 million. [emedicine.com]

However, publications have reported that neither glucagonoma nor hyperglucagonemia is necessary for NME. [4] Pseudoglucagonoma syndrome refers to NME in the absence of a glucagon-secreting tumor. [5, 6] Pathophysiology Although the pathogenesis of NME [emedicine.com]

Early recognition and treatment may prevent metastatic disease and ensure its cure with resolution of the cutaneous and catabolic manifestations. [ncbi.nlm.nih.gov]