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Psoriasiform lesions: Uncommon presentation of glucagonoma
Lesiones psoriasiformes: forma infrecuente de presentación de glucagonoma
Álvaro Martínez Manzanoa,
Corresponding author
alvaromm88@gmail.com

Corresponding author.
, María Dolores Balsalobre Salmeróna, María Aránzazu García Lópeza, Sara Soto Garcíab, José Luis Vázquez Rojasa
a Servicio de Cirugía General y del Aparato Digestivo, Hospital General Universitario Santa Lucía, Cartagena, Murcia, Spain
b Servicio de Anatomía Patológica, Hospital General Universitario Santa Lucía, Cartagena, Murcia, Spain
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    "textoCompleto" => "<span class="elsevierStyleSections"><p id="par0005" class="elsevierStylePara elsevierViewall">Glucagonoma is a rare tumour of the alpha cells of the islets of both the body and tail of the pancreas&#46; Approximately 70&#37; are associated with glucagonoma syndrome&#44; characterised by the development of necrolytic migratory erythema &#40;NME&#41;&#44; diabetes mellitus&#44; weight loss&#44; anaemia&#44; diarrhoea&#44; neuropsychiatric disorders and thromboembolic phenomena&#46;<a class="elsevierStyleCrossRef" href="#bib0040"><span class="elsevierStyleSup">1</span></a> NME is a rare skin condition&#44; involving erythematous&#44; pruritic&#44; painful papules in the perineum and intertriginous areas&#46; The papules group together forming plaques around a central blister&#46; In 90&#37; of cases&#44; NME is associated with a glucagonoma&#46;<a class="elsevierStyleCrossRef" href="#bib0045"><span class="elsevierStyleSup">2</span></a> The skin lesions can sometimes adopt a psoriasiform appearance&#44; and the differential diagnosis between psoriasis and NME should be made in the case of widespread psoriasis which does not improve with sustained treatment&#46;</p><p id="par0010" class="elsevierStylePara elsevierViewall">We present the case of a 52-year-old male investigated by dermatology for skin lesions&#46; He was diagnosed with psoriasis vulgaris&#44; but over the course of three years on treatment&#44; without showing any improvement&#44; he developed new lesions consisting of erythematous&#44; erosive-crusted plaques on his lower limbs &#40;particularly below the knees&#41;&#46; A skin biopsy was requested as NME was suspected&#46; The analysis showed psoriasiform dermatitis with compact parakeratosis and morphologically consistent subcorneal pustules and the patient was diagnosed with early-stage NME &#40;<a class="elsevierStyleCrossRefs" href="#fig0005">Figs&#46; 1 and 2</a>&#41;&#46; An abdominal CT scan was performed&#44; finding a 3&#46;2-cm mass in the body of the pancreas with lymphadenopathy in the coeliac trunk of a significant size and multiple liver metastases affecting both lobes&#46; Blood tests showed normal blood glucose&#44; haemoglobin 12&#46;3<span class="elsevierStyleHsp" style=""></span>g&#47;dl &#40;13&#46;5&#8211;17&#46;5&#41;&#44; haematocrit 36&#37; &#40;41&#8211;53&#37;&#41;&#44; lutropin 12&#46;5<span class="elsevierStyleHsp" style=""></span>mIU&#47;ml &#40;1&#46;5&#8211;9&#46;3&#41;&#44; follitropin 5&#46;9<span class="elsevierStyleHsp" style=""></span>mIU&#47;ml &#40;1&#46;5&#8211;12&#46;4&#41;&#44; cortisol 14&#46;3<span class="elsevierStyleHsp" style=""></span>&#956;g&#47;dl &#40;6&#46;2&#8211;19&#46;4&#41;&#44; somatotropin 1&#46;3<span class="elsevierStyleHsp" style=""></span>ng&#47;ml &#40;&#60;10&#46;0&#41;&#44; adrenocorticotropic hormone 87&#46;8<span class="elsevierStyleHsp" style=""></span>pg&#47;ml &#40;&#60;46&#46;0&#41;&#44; enolase 29<span class="elsevierStyleHsp" style=""></span>ng&#47;ml &#40;&#60;16&#46;0&#41;&#44; beta-hCG 69<span class="elsevierStyleHsp" style=""></span>mIU&#47;ml &#40;&#60;3&#46;0&#41; and carcinoembryonic antigen 1&#46;4<span class="elsevierStyleHsp" style=""></span>ng&#47;ml &#40;&#60;5&#46;0&#41;&#46; Investigations were completed with OctreoScan<span class="elsevierStyleSup">&#174;</span> &#40;<a class="elsevierStyleCrossRef" href="#fig0015">Fig&#46; 3</a>&#41;&#44; which showed a large mass in the body of the pancreas measuring approximately 3<span class="elsevierStyleHsp" style=""></span>cm&#44; compatible with a glucagonoma&#44; in addition to numerous areas of abnormal uptake of tracer in both lobes of the liver&#46; Glucagonoma was diagnosed&#44; which we decided to treat surgically&#44; performing distal pancreatectomy with lymphadenectomy&#46; The pathology report concluded that it was a well-differentiated G1 neuroendocrine tumour&#44; compatible with glucagonoma of the body of pancreas&#44; pT3 pN1&#46; After surgery&#44; the patient made good progress and&#44; two weeks after the intervention&#44; the skin lesions had completely disappeared &#40;<a class="elsevierStyleCrossRef" href="#fig0020">Fig&#46; 4</a>&#41;&#46; The patient is currently on treatment with lanreotide and is awaiting assessment for liver transplantation&#46;</p><elsevierMultimedia ident="fig0005"></elsevierMultimedia><elsevierMultimedia ident="fig0010"></elsevierMultimedia><elsevierMultimedia ident="fig0015"></elsevierMultimedia><elsevierMultimedia ident="fig0020"></elsevierMultimedia><p id="par0015" class="elsevierStylePara elsevierViewall">Pancreatic neuroendocrine tumours&#44; of which glucagonoma represents 4&#37;&#44; account for 2&#37; of all gastrointestinal tumours&#46;<a class="elsevierStyleCrossRef" href="#bib0050"><span class="elsevierStyleSup">3</span></a> Glucagonoma is more common in females aged over 45&#46; Approximately 50&#37; of the patients diagnosed with these tumours develop clinical signs and symptoms related to the biological activity of the hormones secreted by the cancer&#46; In patients with glucagonoma&#44; 70&#8211;80&#37; have the following triad&#58; diabetes&#44; NME and anaemia&#46; Like other neuroendocrine tumours&#44; glucagonomas express somatostatin receptors in more than 80&#37; of cases&#46;</p><p id="par0020" class="elsevierStylePara elsevierViewall">Glucagonomas can be detected by CT&#44; MRI or ultrasound&#46;<a class="elsevierStyleCrossRef" href="#bib0055"><span class="elsevierStyleSup">4</span></a> As they express somatostatin receptors&#44; scintigraphy with somatostatin analogues is used to demonstrate the presence of the cancer and the extent to which it may have spread&#46;</p><p id="par0025" class="elsevierStylePara elsevierViewall">These tumours are slow-growing&#44; with a high 10-year survival rate of over 50&#37; in patients with metastasis&#44; and around 65&#37; in those without metastases&#46; Mean survival is from three to seven years after diagnosis&#46;<a class="elsevierStyleCrossRef" href="#bib0060"><span class="elsevierStyleSup">5</span></a> The most effective treatment for NME is based on returning the glucagon levels to normal through surgical removal of the pancreatic tumour&#44; as occurred with our patient&#46; Surgical treatment is indicated in patients with disease limited to the pancreas&#44; with or without lymphadenopathy&#44; and for patients with liver or other potentially resectable metastases&#46;<a class="elsevierStyleCrossRef" href="#bib0065"><span class="elsevierStyleSup">6</span></a></p><p id="par0030" class="elsevierStylePara elsevierViewall">Over 50&#37; of patients with clinical symptoms have liver metastases at the time of diagnosis&#46; The surgical treatment of single liver metastasis has a 10-year survival rate of 60&#37;&#46; When the patient has liver metastases in both lobes with no extrahepatic disease and the tumour is resectable&#44; liver transplantation with mean five-year survival of 40&#8211;81&#37; may be indicated&#46; If surgical treatment of metastases is contraindicated&#44; chemoembolisation or radiofrequency ablation may be performed&#46; Palliative treatment with somatostatin or its analogue &#40;octreotide&#41; achieves good results&#46; These drugs reduce the conversion of proglucagon to glucagon&#44; causing their levels to fall&#44; achieving clinical improvement in many cases&#46; Medical treatment with somatostatin analogues is considered the treatment of choice in cases of unresectable tumour&#46; It also has benefits in the states of hormone overproduction &#40;should be used in cases where the symptoms persist&#41;&#46; The choice of treatment has to be made on an individual basis&#46;<a class="elsevierStyleCrossRef" href="#bib0070"><span class="elsevierStyleSup">7</span></a></p><p id="par0035" class="elsevierStylePara elsevierViewall">In conclusion&#44; the spread of the tumour and the presence of metastases will determine the curative strategy to follow&#44; with surgery being the technique of choice when the primary disease is under control and liver metastases &#40;if any&#41; are potentially resectable&#46; Aggressive treatment of liver metastases seems to obtain better results in terms of the individual&#39;s survival&#46; We believe it is important to stress that in the case of a psoriasiform lesion which does not respond to usual treatment&#44; we should suspect a possible NME secondary to glucagonoma&#46;</p></span>"
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        "nota" => "<p class="elsevierStyleNotepara" id="npar0005">Please cite this article as&#58; Mart&#237;nez Manzano &#193;&#44; Balsalobre Salmer&#243;n MD&#44; Garc&#237;a L&#243;pez MA&#44; Soto Garc&#237;a S&#44; V&#225;zquez Rojas JL&#46; Lesiones psoriasiformes&#58; forma infrecuente de presentaci&#243;n de glucagonoma&#46; Gastroenterol Hepatol&#46; 2018&#59;41&#58;500&#8211;502&#46;</p>"
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