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. 2022 May 31;12(1):9053.
doi: 10.1038/s41598-022-12882-2.

Necrolytic migratory erythema is an important visual cutaneous clue of glucagonoma

Wei Li #  1 , Xue Yang #  1 , Yuan Deng  2 , Yina Jiang  2 , Guiping Xu  3 , Enxiao Li  4 , Yinying Wu  4 , Juan Ren  5 , Zhenhua Ma  1 , Shunbin Dong  1 , Liang Han  1 , Qingyong Ma  1 , Zheng Wu  6 , Zheng Wang  7
Affiliations

Necrolytic migratory erythema is an important visual cutaneous clue of glucagonoma

Wei Li et al. Sci Rep. .

Abstract

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. 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. 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 first for necrolytic migratory erythema (NME) 7/7 (100%), and 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%), and 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 analog therapy. Symptoms improved significantly in 6 out of 7 patients. Three of seven patients died of this disease by the time of follow-up. 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 analogs are effective for symptom relief and tumor control.

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Conflict of interest statement

The authors declare no competing interests.

Figures

Figure 1
Figure 1
NME of glucagonoma. Skin lesions affecting lower back and gluteal regions. The boundary of the rash is not clear. Erosion, exudation and necrosis can be seen in the center of some erythematous lesions.
Figure 2
Figure 2
Histopathological image of skin biopsy of NME. The H&E image of the skin biopsy of NME showed mild hyperplasia of the epidermis and abundant lymphocyte infiltration (× 40).
Figure 3
Figure 3
Histopathological (H&E) image of glucagonoma. The pathological examination of excised tissue showed a grade 2 pancreatic neuroendocrine tumor, with a mitotic count of 3 per 10 high-power fields (× 100).
Figure 4
Figure 4
Positive immunohistochemical staining for glucagon, Cga, Syn and SSTR2 (× 100).
See this image and copyright information in PMC

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