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Dermatologic Manifestations of Glucagonoma Syndrome Treatment & Management

  • Author: Sara Flores, MD; Chief Editor: William D James, MD  more...
Updated: May 12, 2016

Medical Care

Most traditional chemotherapies are not effective on glucagonoma, probably due to the tumor's indolent nature. No cure by medical means has been reported, but some drug and hormone regimens may induce tumor regression or symptomatic remission of necrolytic migratory erythema (NME).

Streptozocin, a drug that causes selective islet cell damage, shows promise in combination with 5-fluorouracil. The combination can achieve a 60-70% response rate. Dacarbazine induced remission from NME by reducing tumor burden and glucagon levels in at least one instance.[13]

Transcatheter hepatic artery embolization (TAE) may induce selective necrosis of hepatic metastases by providing locally high levels of antineoplastic agents, thus sparing systemic adverse effects. TAE, in association with cisplatin-releasing gelatin microspheres, has been reported in one study for metastatic liver tumors.[14]

The somatostatin agonists octreotide and lanreotide are the drugs of choice in the treatment of patients with glucagonoma. These somatostatin analogs can be coupled to radioisotopes for both imaging and treatment applications.[15] Peptide receptor radioligand therapy (PRRT) with radiolabeled somatostatin analogue therapy has been tried with mixed results.[16]

Sunitinib, a small molecule inhibitor of multiple receptor tyrosine kinases critical for tumor growth and angiogenesis, including PDGF receptors, has been shown to be effective in treating numerous pancreatic neuroendocrine tumors but may be particularly efficacious in the treatment of glucagonomas.[17]

In pseudoglucagonoma syndrome, treating the underlying disease often resolves NME. One case report describes the successful use of octreotide for treating NME with pseudoglucagonoma.[18]


Surgical Care

Surgical resection is curative and, thus, is the treatment of choice if the tumor is localized..  Often, the resection can be performed without adjunct therapy because the tumor is slow growing and tends to be encapsulated. The use of laparoscopy has been reported in a few cases.

When the tumor is disseminated, surgical management may be used for palliation while somatostatin or chemotherapeutic agents (see above) are administered. Since NME is the major source of morbidity in glucagonoma syndrome, palliative therapies are aimed at reducing the cutaneous eruption. Tumor debulking has been shown to decrease the intensity of the cutaneous symptoms by reducing the level of serum glucagon.

Multimodality therapy for liver metastases combining surgery, transarterial chemoembolization, percutaneous radiofrequency thermal ablation, and long-acting octreotide has been proven to be effective.[19]



Cases of NME secondary to celiac sprue can resolve with implementation of a gluten-free diet. Deficiencies in amino acids, essential fatty acids, and zinc have been linked to NME by observation that symptoms resolve with supplementation of the deficient nutrient.[20]

Contributor Information and Disclosures

Sara Flores, MD Resident Physician, Department of Dermatology, University of Cincinnati College of Medicine

Sara Flores, MD is a member of the following medical societies: American Academy of Dermatology, American Medical Association, American Society for Dermatologic Surgery, Cincinnati Dermatological Society, Ohio Dermatological Association

Disclosure: Nothing to disclose.


Ali Alikhan, MD Clinical Assistant Professor, Director of Clinical Trials, Residency Program Co-Director, Department of Dermatology, University of Cincinnati College of Medicine

Ali Alikhan, MD is a member of the following medical societies: American Academy of Dermatology, National Psoriasis Foundation, Cincinnati Dermatological Society, National Psoriasis Foundation, Ohio Dermatological Association

Disclosure: Nothing to disclose.

Specialty Editor Board

David F Butler, MD Section Chief of Dermatology, Central Texas Veterans Healthcare System; Professor of Dermatology, Texas A&M University College of Medicine; Founding Chair, Department of Dermatology, Scott and White Clinic

David F Butler, MD is a member of the following medical societies: American Medical Association, Alpha Omega Alpha, Association of Military Dermatologists, American Academy of Dermatology, American Society for Dermatologic Surgery, American Society for MOHS Surgery, Phi Beta Kappa

Disclosure: Nothing to disclose.

Warren R Heymann, MD Head, Division of Dermatology, Professor, Department of Internal Medicine, Rutgers New Jersey Medical School

Warren R Heymann, MD is a member of the following medical societies: American Academy of Dermatology, American Society of Dermatopathology, Society for Investigative Dermatology

Disclosure: Nothing to disclose.

Chief Editor

William D James, MD Paul R Gross Professor of Dermatology, Vice-Chairman, Residency Program Director, Department of Dermatology, University of Pennsylvania School of Medicine

William D James, MD is a member of the following medical societies: American Academy of Dermatology, Society for Investigative Dermatology

Disclosure: Nothing to disclose.

Additional Contributors

David P Fivenson, MD Associate Director, St Joseph Mercy Hospital Dermatology Program, Ann Arbor, Michigan

David P Fivenson, MD is a member of the following medical societies: American Academy of Dermatology, Michigan State Medical Society, Society for Investigative Dermatology, Photomedicine Society, Wound Healing Society, Michigan Dermatological Society, Medical Dermatology Society

Disclosure: Nothing to disclose.

Nathalie C Zeitouni, MDCM, FRCPC Chair of Dermatology, Associate Professor of Dermatology, Roswell Park Cancer Institute

Nathalie C Zeitouni, MDCM, FRCPC is a member of the following medical societies: American Academy of Cosmetic Surgery, American Academy of Dermatology, American College of Mohs Surgery, American Society for Dermatologic Surgery, Women's Dermatologic Society, Royal College of Physicians and Surgeons of Canada

Disclosure: Nothing to disclose.

Nishikant Harvey, MD Clinical Assistant Instructor, Department of Anesthesiology, SUNY at Buffalo Graduate Medical-Dental Consortium

Nishikant Harvey, MD is a member of the following medical societies: American Society of Anesthesiologists

Disclosure: Nothing to disclose.

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Necrolytic migratory erythema in a patient with glucagonoma syndrome. Annular erythematous plaques, vesicles, and erosions involving the whole perineal area are shown.
Necrolytic migratory erythema involving the entire buttock and perineal region in a patient with glucagonoma syndrome.
Close-up view of an annular plaque showing necrolytic migratory erythema in a patient with glucagonoma syndrome.
Acanthosis with upper epidermal necrolysis from a patient with necrolytic migratory erythema and glucagonoma syndrome. Note prominent intracellular edema and loss of granular cell layer (X10).
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