Glucagonoma syndrome with atypical necrolytic migratory erythema

He, Shujuan; Zeng, Weihui; Geng, Shuang; Jia, Jinjing

doi:10.4103/ijdvl.ijdvl_588_18

Cited by 10 publications

(11 citation statements)

References 9 publications

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“…There are many theories regarding the pathogenesis of ENM, including direct action of glucagon in inducing skin necrolysis, hypoaminoacidemia-induced epidermal protein deficiency and necrolysis, nutritional or metabolic deficiency of zinc or essential fatty acids, enteroglucagon induction of inflammatory mediators, and generalized malabsorption [7,9,10]. The occurrence of necrolytic erythema migrans in celiac disease reinforces current pathophysiological hypotheses suggesting a role for hypoprotidemia and plasma amino acid deficiency in necrolytic erythema migrans.…”

Section: Discussionmentioning

confidence: 93%

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Necrolytic erythema migrans in celiac disease

Dassouli¹,

Baybay²,

Choukri³

et al. 2022

Our Dermatol Online

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Erythema migrans necrolytica is a red, blistering rash that spreads over the skin. It particularly affects the skin around the mouth and distal extremities, but can 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 such as celiac disease. We present a case of a patient with a history of poorly followed celiac disease presenting with a clinical picture of ENM. This rare case adds to our understanding of the clinical presentation of NME, as well as highlights the importance of acting in a timely manner to avoid the most redoubtful complications.

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Section: Discussionmentioning

confidence: 93%

“…Angular cheilitis, glossitis, or alopecia may also be present. After healing of the lesions, residual areas of hyperpigmentation and a peripheral collar of scales may remain [8,9].…”

Section: Discussionmentioning

confidence: 99%

Necrolytic erythema migrans in celiac disease

Dassouli¹,

Baybay²,

Choukri³

et al. 2022

Our Dermatol Online

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“…Hyperglucagonemia might lead an important role, as surgical removal of glucagonomas or stabilizing glucagon levels using somatostatin analogue results in rash controlling [7,16]. Other theories including hypoaminoacidaemia-induced epidermal protein and micro-nutrient depletion, de ciency of essential fatty acids and zinc should also be considered because nutritional support therapy and topical zinc therapy has been used to ameliorate NME [17][18]. Surgical removal is considered the only de nitive and curative treatment for pancreatic glucagonoma and NME [7].…”

Section: Discussionmentioning

confidence: 99%

Necrolytic migratory erythema: an important visual cutaneous clue of glucagonoma

Xue

Deng

et al. 2022

Preprint

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Objective: Glucagonoma is an extremely rare neuroendocrine tumor that arises from pancreatic islet alpha cells. Although glucagonoma is usually accompanied by a variety of characteristic clinical symptoms, early diagnosis is still difficult due to the scarcity of the disease. Methods: In this study, we present the cumulative experiences, clinical characteristics and treatments of seven patients diagnosed with glucagonoma during the past 10 years at the First Affiliated Hospital of Xi’an Jiaotong University. Results: The seven patients in our cohort consisted of six females and one male with an average diagnosis age of 40.1 years (range 23-51). The average time from onset of symptoms to diagnosis of glucagonoma was 14 months (range 2-36 months). All the patients visited dermatology firstly for necrolytic migratory erythema (NME) 7/7 (100%), other presenting symptoms included: diabetes mellitus (DM) 4/7 (57%), stomatitis 2/7 (28%), weight loss 4/7 (57%), anemia 4/7 (57%), diarrhea 1/7 (14%), DVT1/7 (14%). Plasma glucagon levels were increased in all patients (range 216.92–3155 pg/mL), and declined after surgery. Imaging studies revealed that four of seven patients had liver metastasis. Six of seven patients received surgical resection, and all of them received somatostatin analogue therapy. Symptoms improved significantly in 6 out of 7 patients. Three of seven patients died of this disease by the time of follow-up. Conclusion: Our data suggest that if persistent NME is associated with DM and high glucagon levels, timely abdominal imaging should be performed to confirm glucagonoma. Once diagnosed, surgery and somatostatin analogues are effective for symptom relief and tumor control.

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“…[11] However, the diagnostic process is not without di culties. [12][13] In our experience, a combination of clinical and laboratory ndings offers the best means of establishing a diagnosis.…”

Section: Discussionmentioning

confidence: 99%

Diagnosis and treatment of pancreatic glucagonoma presenting with multiple metastases and recurring postoperatively: Report of two patients

Zhang

et al. 2022

Preprint

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Background: Pancreatic glucagonoma is a rare neuroendocrine neoplasm with malignant potential. Its etiology is unclear, and its course is protracted, often fraught by recurrences and greatly diminished quality of life. Case presentation: Herein, we report two patients with such tumors, one a 60-year-old woman hospitalized for blood glucose elevation and recurrent necrolytic migratory erythema (NME) of the trunk and limbs. Imaging studies showed an area (~3 cm) of reduced enhancement at head of pancreas. Examination of the subsequent pancreaticoduodenectomy specimen confirmed a pancreatic neuroendocrine tumor (G2). The patient’s blood glucose normalized postoperatively, and her skin erythema cleared. During follow-up, however, intramuscular injections (30 mg) of long-acting release (LAR) octreotide acetate microspheres were required at 28-day intervals for recurrent NME; and there was apparent hepatic and retroperitoneal nodal involvement after 36 months. The second patient was a 32-year-old woman hospitalized for recurrent glossitis, perioral dermatitis, and NME of both lower limbs. Imaging studies revealed a low-density nodule (~2 cm) in tail of pancreas, as well as multiple space-occupying lesions of liver. Distal pancreatectomy was undertaken, with splenectomy and palliative resection of hepatic nodules, confirmingmetastasis of a primary pancreatic neuroendocrine tumor (G2) to liver.The NME resolved postoperatively, but this patient also received intramuscular octreotide LAR injections (30 mg) at 28-day intervals. After 6 months, percutaneous radiofrequency ablation of residual liver lesions took place. Conclusions: The present accounts detail our diagnostic and therapeutic experiences with this rare type of tumor, especially the management of multiple metastases upfront and postoperative recurrences.

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Glucagonoma syndrome with atypical necrolytic migratory erythema

Cited by 10 publications

References 9 publications

Necrolytic erythema migrans in celiac disease

Necrolytic erythema migrans in celiac disease

Necrolytic migratory erythema: an important visual cutaneous clue of glucagonoma

Diagnosis and treatment of pancreatic glucagonoma presenting with multiple metastases and recurring postoperatively: Report of two patients

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