Necrolytic migratory erythema is an important visual cutaneous clue of glucagonoma

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.

Total pancreatectomy in a patient with a dermadrome caused by intraductal papillary mucinous neoplasm: A case report

“…The term “dermadrome,” coined by Wiener, originally referred to skin symptoms reflecting visceral lesions, encompassing all skin symptoms associated with systemic diseases [ 2 ]. Various dermadromes are caused by visceral tumors, and previous reports have suggested improvements in skin lesions after tumor excision [ 3 , 4 ].…”

“…However, in Europe and the United States, the systematic classification and description of systemic diseases and skin symptoms based on etiology became the prevailing approach, leading to the disuse of the term dermadrome [ 4 ]. Meanwhile, in Japan, according to Mitsuhashi and Oyama, the term “dermadrome” has been used in special cases, i.e., those with “usefulness,” “unexpectedness,” and “specificity,” rather than encompassing all skin symptoms associated with systemic diseases [ 5 , 6 ].…”

“…Necrolytic migratory erythema is conventionally associated with 70 %–90 % of glucagonomas [ 4 ]. In a report of six patients with necrolytic migratory erythema due to glucagonomas who underwent primary pancreatic lesion resections, skin rash in all patients tended to improve within 1 week postoperatively, and no recurrence was observed thereafter [ 4 ]. Similarly, in the present case, the rash gradually improved after surgery, allowing for steroid dose tapering and eventual treatment termination [ 4 ].…”

“…In a report of six patients with necrolytic migratory erythema due to glucagonomas who underwent primary pancreatic lesion resections, skin rash in all patients tended to improve within 1 week postoperatively, and no recurrence was observed thereafter [ 4 ]. Similarly, in the present case, the rash gradually improved after surgery, allowing for steroid dose tapering and eventual treatment termination [ 4 ].…”

An Overview of Clinical Manifestations of Dermatological Disorders in Intensive Care Units: What Should Intensivists Be Aware of?

“…The sacrum, perineum, buttocks, intertriginous and periorificial areas, distal extremities, and face are all affected by a radially distributed, extremely erythematous rash with scaly necrotic plaques. It is commonly associated with diabetes symptoms, diarrhea, and weight loss [ 121 , 122 ].…”