The glucagonoma syndrome and necrolytic migratory erythema: a clinical review

Beek, André P van; Haas, E; Vloten, W. A. van; Lips, C. J. M.; Roijers, Janine F. M.; Dijk, Marijke R. Canninga‐van

doi:10.1530/eje.0.1510531

Cited by 177 publications

(104 citation statements)

References 37 publications

(17 reference statements)

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“…El déficit de cinc, que semeja la acrodermatitis enteropática, presenta hallazgos histopatológicos similares al eritema necrolítico migratorio, como vacuolización de los queratocitos superficiales, necrosis de queratocitos y paraqueratosis (6,8,14).…”

Section: Discussionunclassified

“…Las manifestaciones clínicas del síndrome se atribuyen a los efectos metabólicos por exceso de glucagón, es decir, el aumento de la glucogenólisis, la lipólisis y la gluconeogénesis a partir de aminoácidos, y la inhibición de la glucólisis (2,8,19). Se cree que al disminuirse los niveles de aminoácidos séricos se aumentan los niveles cutáneos de ácido araquidónico, lo que incrementa la producción de mediadores inflamatorios como prostaglandinas y leucotrienos (2,12).…”

Section: Discussionunclassified

“…En los pacientes con tumores metastásicos o que no se pueden resecar, suelen emplearse análogos de la somatostina (2,6) y suplementos nutricionales (2). La normalización de los niveles séricos de glucagón con tratamiento quirúrgico o médico, generalmente produce la desaparición de las lesiones cutáneas (8,11). El diagnóstico precoz del eritema necrolítico migratorio puede disminuir las consecuencias catabólicas y el riesgo de metástasis (4,14).…”

Section: Discussionunclassified

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Eritema necrolítico migratorio y glucagonoma pancreático

et al. 2016

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El eritema necrolítico migratorio es una dermatosis paraneoplásica infrecuente que puede ser la primera manifestación clínica del glucagonoma, el cual se caracteriza por erupción mucocutánea, intolerancia a la glucosa, hipoaminoacidemia, hiperglucagonemia y glucagonoma pancreático. Se presenta el caso clínico de una mujer de 45 años que presentó pérdida de peso, polidipsia, polifagia, vómito posprandial, caída abundante del cabello y dolor abdominal de dos meses de evolución. Tenía, además, placas eritematosas, descamativas y migratorias en tronco, periné, codos, manos, pies, pliegues submamarios y antecubitales de 20 días de evolución. En la biopsia de piel se observaron células epidérmicas altas con cambio vacuolar notorio, extensa necrosis y delgada capa córnea ortoqueratósica, hallazgos interpretados como eritema necrolítico migratorio. Se sugirió investigar un glucagonoma pancreático. En los exámenes de laboratorio se encontró anemia moderada, hiperglucemia e importante hiperglucagonemia. La ecografía abdominal reveló una masa de 6 x 5 x 5 cm en la cola pancreática, la cual fue resecada. El diagnóstico histopatológico fue de glucagonoma confirmado por inmunohistoquímica. Los síntomas cutáneos desaparecieron a los 10 días de la resección tumoral. Se concluye que los cambios histológicos observados pueden ser claves en la búsqueda de una enfermedad distante de la piel y permiten hacer su diagnóstico. El patrón histológico de vacuolización y necrosis epidérmica subcórnea debe llevar a sospechar la presencia de un glucagonoma pancreático.

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Eritema necrolítico migratorio y glucagonoma pancreático

et al. 2016

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“…Glucagon also exerts a catabolic role by attenuating protein synthesis (9). The elevated glucagon secreted by glucagonoma results in amino acid catabolism and serum glucose elevation, which are considered to be responsible for skin lesions and DM (10). Glucagonoma syndrome consists of a triad comprising glucagon-secreting tumors, DM and NME (11).…”

Section: Discussionmentioning

confidence: 99%

Glucagonoma syndrome: A case report

Wei

Lin

et al. 2015

Oncology Letters

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Abstract. Necrolytic migratory erythema (NME), diabetes mellitus and glucagon-secreting tumors form the hallmarks of glucagonoma syndrome, and represent the major clinical manifestations of glucagonoma. NME is usually presented as the initial complaint of patients. Due to the rare incidence of glucagonoma, its diagnosis is often delayed, which leads to its progression. Here, we report a case of NME with a typical skin rash, which was misdiagnosed and treated with corticosteroids for two years. Removal of the tumor in the pancreatic body led to the rapid relief of the symptoms. The aim of the present study is to demonstrate the typical characteristics of glucagonoma syndrome to clinicians in order to improve its diagnosis and treatment. IntroductionGlucagonoma is an uncommon neoplasm of the pancreatic neuroendocrine islet α-cells, whereby the islet cells secrete abundant glucagon (1). The estimated annual incidence of glucagonoma is ~1 case per 20,000,000 individuals (2). Due to the rarity of the disease, mortality rates remain unclear. In a study of 21 glucagonoma patients, Wermers et al (3) reported that nine patients succumbed to the diease after a mean duration of 4.91 years following diagnosis (3). Complete tumor resection may cure glucagonoma, with a 10-year survival rate of 64.3% following surgery, however, liver and lymph node metastasis are major risk factors that contribute to tumor-associated mortality (4). For patients with unresectable advanced disease, tumor debulking, chemotherapy or somatostatin may also be considered to reduce the tumor-associated symptoms, however, the survival benefit of such treatments is limited (2,3). Glucagonoma induces various manifestations, which are characterized by necrolytic migratory erythema (NME), diabetes mellitus (DM), weight loss, anemia and neuropsychiatric disturbances (1). NME represents the most specific manifestation of glucagonoma syndrome and has therefore provided the most valuable indications for diagnosis in the majority of previous cases (5). Early recognition of NME is likely to lead to a more rapid diagnosis of glucagonoma, thereby allowing surgical resection and achieving a promising therapeutic outcome. This study presents the case of a glucagonoma patient who recovered following spleen-preserving distal pancreatectomy. Case reportIn November 2010, a 50-year-old female presented to The Pancreas Center of Nanjing Medical University (Nanjing, China) with a 2-year history of a pruritic and ulcerating skin rash, which initially appeared at the waist and slowly progressed to the entire body within three months (Fig. 1A). Phacoscotasmus, cheilitis and glossitis were also present (Fig. 1B). The patient also complained of blurred vision and weight loss of 5 kg. The patient was initially diagnosed with pemphigus and treated with prednisone without relief of the skin rash prior to her admission to our clinic. Physical examination revealed that the skin lesions were erythematous macules with erosions, serous exudate and crusting (Fig. 1C). A skin biopsy reve...

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“…58,59 The characteristic cutaneous eruption, necrolytic migratory erythema, often affects the perioral region and the distal extremities, but may also occur on the abdomen, perineum, thighs, and buttocks, as well as in the groin. Patches of intense erythema with irregular outlines expand and coalesce to result in circinate or polycyclic configurations (Fig.…”

Section: Glucagonoma Syndromementioning

confidence: 99%

Cutaneous Manifestations of Internal Malignancy

Thiers¹,

Sahn²,

Callen³

2009

CA: A Cancer Journal for Clinicians

125

107

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The skin often mirrors changes in the organism it envelops. Many neoplastic diseases that affect internal organs display cutaneous manifestations, which may be the presenting signs and symptoms of the underlying malignancy. These may reflect direct involvement of the skin by the tumor (ie, tumor metastasis) or indirect involvement, in which changes in the skin occur in the absence of malignant cells. This review focuses on the latter conditions, which are often referred to as paraneoplastic dermatoses. Included in the discussion are the cutaneous manifestations of inherited syndromes that are associated with an increased risk of internal malignancy, cutaneous changes in patients with hormone-secreting tumors, and the wide spectrum of proliferative and inflammatory dermatoses that have been associated with internal cancer. CA Cancer J Clin 2009;59:73-98.

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The glucagonoma syndrome and necrolytic migratory erythema: a clinical review

Cited by 177 publications

References 37 publications

Eritema necrolítico migratorio y glucagonoma pancreático

Eritema necrolítico migratorio y glucagonoma pancreático

Glucagonoma syndrome: A case report

Cutaneous Manifestations of Internal Malignancy

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