Necrolytic migratory erythema associated with glucagonoma treated successfully with cyclosporine

Glucagonoma is a hormonally active rare pancreatic neuroendocrine tumour causing an excess of glucagon. This is a narrative review based on a multidisciplinary approach of the tumour. Typically associated dermatosis is necrolytic migratory erythema (NME) which is most frequently seen at disease onset. Insulin-dependent diabetes mellitus, depression, diarrhoea, deep vein thrombosis are also identified, as parts of so-called 'D' syndrome. Early diagnosis is life saving due to potential aggressive profile and high risk of liver metastasis. NME as paraneoplastic syndrome may be present for months and even years until adequate recognition and therapy; it is remitted after successful pancreatic surgery. Thus the level of practitioners' awareness is essential. If surgery is not curative, debulking techniques may improve the clinical aspects and even the outcome in association with other procedures such as embolization of hepatic metastasis; ablation of radiofrequency type; medical therapy including chemotherapy, targeted therapy with mTOR inhibitors such as everolimus, PRRT (peptide receptor radiotherapy), and somatostatin analogues (including combinations of medical treatments). Increased awareness of the condition involves multidisciplinary practitioners.

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“…Despite its rarity, prompt dermatological diagnosis of NME is the first step of life saving multidisciplinary intervention (34,35).…”

Section: Glucagonoma-associated Skin Lesionsmentioning

confidence: 99%

Glucagonoma: From skin lesions to the neuroendocrine component (Review)

et al. 2020

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“…7,8 In the literature, good therapy response to the NME component of glucagonoma with different treatment modalities was reported. 3,[9][10][11][12] We think that this case is important for demostrating the treatment of NME of glucagonoma with PRRT. Furthermore, regression of these lesions correlated well with the therapeutic outcome as detected by PET/CT.…”

mentioning

confidence: 99%

“…Although not pathognomonic, NME is mostly associated with glucagonoma and considered to be a skin manifestation of nutritional deficiency (zinc, essential fatty acid, and amino acid deficiencies)[1][2][3][4][5][6] Treatment options for glucagonoma include surgery, chemotherapy, somatostatin analogs, PRRT, hepatic artery embolization, radiofrequency ablation, and α interferons 7,8. In the literature, good therapy response to the NME component of glucagonoma with different treatment modalities was reported 3,[9][10][11][12]. We think that this case is important for demostrating the treatment of NME of glucagonoma with PRRT.…”

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confidence: 99%

Treatment of Glucagonoma-Related Necrolytic Migratory Erythema With Peptide Receptor Radionuclide Therapy

Mavi

Tuncel

2021

Clin Nucl Med

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Glucagonomas are rare types of pancreatic neuroendocrine tumors. They may present with a clinical entity called glucagonoma syndrome, which includes necrolytic migratory erythema as a skin component. Here we present a 26-year-old woman experiencing ongoing skin lesions, excessive weight loss, and nausea. She was diagnosed with metastatic glucagonoma. Her 68Ga-DOTATATE PET/CT showed increased uptake at the primary pancreatic lesion and hepatic metastases. She received 2 cycles of peptide receptor radionuclide therapy and had a partial response with a near-complete regression of her skin lesions.

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“…Surgical removal through conventional or laparoscopic means is considered the most definitive treatment for pancreatic glucagonoma and NME. Treatment with the somatostatin analogues and peptide receptor radioligand therapy may also result in clinical resolution, particularly in tumors with somatostatin receptor expression . Chemotherapeutic agents such as doxorubicin, streptozotocin, sunitinib, and everolimus have been used.…”

mentioning

confidence: 99%

“…In addition, systemic corticosteroids or cyclosporine can improve the skin lesions, albeit temporarily. Finally, a few case reports have suggested that administration of intravenous amino acids can replenish deficits in essential amino acids and result in a rapid resolution of NME …”

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confidence: 99%

Necrolytic Migratory Erythema

Liu

Qian

2019

JAMA Dermatol

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A man in his 40s presented with a 7-year history of a pruritic, annular cutaneous eruption and flaccid vesicles. The eruption was episodic in nature with spontaneous exacerbations and remissions. He reported modest weight loss but was otherwise well. Physical examination revealed crusted papules and polycyclic plaques with a map-like margin mainly involving the perioral region, chest, groin, buttocks, and extremities (Figure). His tongue appeared beefy red and his nails were brittle with transverse fissuring. A skin biopsy was performed and showed necrolysis in the upper third of the epidermis, vacuolation of the epidermal keratinocytes, and a superficial perivascular lymphocytic infiltrate. Laboratory analysis found a fasting blood glucose level of 6.5 mmol/L (normal range, 3.6 to 6.1 mmol/L) and a serum glucagon level of 1024 ng/L (normal range, 49 to 114 ng/L). The diagnoses of glucagonoma syndrome and associated necrolytic migratory erythema (NME) were made. A contrastenhanced abdominal computed tomographic scan revealed an enhancing pancreatic tail mass measuring 6.5 cm in maximum diameter. The patient underwent a subtotal pancreatectomy and splenectomy. Pathologic analysis confirmed a pancreatic neuroendocrine carcinoma consistent with a diagnosis of glucagonoma. Two weeks after the surgery, the patient's cutaneous lesions had nearly re-3.

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Necrolytic migratory erythema associated with glucagonoma treated successfully with cyclosporine

Cited by 6 publications

References 5 publications

Glucagonoma: From skin lesions to the neuroendocrine component (Review)

Glucagonoma: From skin lesions to the neuroendocrine component (Review)

Treatment of Glucagonoma-Related Necrolytic Migratory Erythema With Peptide Receptor Radionuclide Therapy

Necrolytic Migratory Erythema

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