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Glucagonoma is a neuroendocrine tumour of very low incidence, estimated at 1 case per 20 million people per year. It typically manifests with a clinical syndrome that includes a characteristic dermatosis called necrolytic migratory erythema (NME). We present the case of a 60-year-old male with NME as the initial presentation of a pancreatic tumour and its imaging findings. We emphasize the importance of recognizing the clinical features of NME and the role of different imaging methods for early diagnosis and correct management of these pancreatic tumours.

El glucagonoma es un tumor neuroendocrino de muy baja incidencia, se estima 1 caso cada 20 millones de personas por año. Típicamente, se manifiesta con un síndrome clínico que incluye una dermatosis característica denominada eritema necrolítico migratorio (ENM). Presentamos el caso de un varón de 60 años con ENM como presentación inicial de un tumor pancreático y los hallazgos por imágenes del mismo. Resaltamos la importancia de reconocer las características clínicas del ENM y el rol de los diferentes métodos por imágenes para un diagnóstico precoz y correcto manejo de estos tumores pancreáticos.

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Background: Superficial necrolytic dermatitis (SND), hepatocutaneous syndrome (HCS), metabolic epidermal necrosis (MEN), and necrolytic migratory erythema (NME) are useful terms to describe a disease that likely has a multifactorial etiopathogenesis. SND is a rare and fatal disease characterized by skin lesions and liver disease. Common skin lesions include hyperkeratosis, fissures, erosion, ulceration, crusting, exudation from the paws, face, perianal regions, and pressure points. This case report aimed to report the case of a bitch that developed the rare Superficial Necrolytic Dermatitis disease, emphasizing the clinical signs of the disease, and the importance of complementary exams such as abdominal ultrasound and skin biopsy for the definitive diagnosis. Case: A 9-year-old, mixed-breed, neutered female was referred for clinical examination with 5 months history of hyperkeratosis and ulceration of the paw pads, presenting pain, lameness and weight loss. Abdominal ultrasound revealed a liver with heterogeneous echotexture, mixed echogenicity, irregular and poorly delimited margins with hypoechoic nodules throughout like honeycombs. The gallbladder was visualized with a moderately thick layer. Histological analysis confirmed the diagnosis of SND. Skin biopsies showed an increase in thickness of the epidermis due to irregular hyperplasia and proliferation of keratinocytes in the basal layer of the epidermis, pallor of the spinous layer of the epidermis and important parakeratosis. Due to the progression of the disease, significant worsening of the patient's clinical condition and pain, associated with the impossibility of cure, the animal was submitted to euthanasia. A necropsy was performed to allow assessment of the liver and pancreas. The biopsies showed a severe proliferative chronic hepatitis, steatosis and cholestasis associated with pancreatitis and necrotic multifocal proliferative fibrinopurulent areas in the pancreas. Discussion: Clinical signs such as lethargy, inappetence, weight loss, as well as the dermatological signs presented by this bitch are nonspecific clinical signs and require a deeper clinical, pathological and histopathological diagnostic investigation to reach the diagnosis of this disease. The definitive diagnosis is made on the basis of a characteristic honeycomb pattern in the liver or associated with a neoplastic finding in the pancreas on ultrasound examination and confirmed by histopathological evaluation of skin biopsies. Palliative treatment with corticosteroid anti-inflammatories, improvement in feed quality, with higher nutritional and protein intake and intravenous amino acid supplementation are suggested by some authors as treatment alternatives. However, not all owners can afford a costly lifetime treatment. With the progressive worsening of the condition, many owners opt for euthanasia as a way to shorten the suffering of the animal. This decision is not an easy one to make. Despite the poor prognosis of the disease, treatment options should be tried by veterinarians and owners prior to the option of euthanasia. However, new affordable nutritional and pharmacological strategies to treat or control the disease are needed in order to improve quality of life of SND patients.

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RESUMEN 19- Las dermatosis paraneoplásicas son un grupo heterogéneo de manifestaciones cutáneas que tienen fuerte asociación con patología maligna interna. Su patogenia es poco clara y no se conoce su prevalencia exacta. El tratamiento consiste en el abordaje terapéutico de la enfermedad subyacente. La importancia del conocimiento de las mismas radica en la posibilidad de realizar un diagnóstico temprano de una neoplasia. 20- Se presenta el caso clínico de un paciente con dermatosis paraneoplásica asociada a tumor neuroendócrino de páncreas.

ABSTRACT 24- Paraneoplastic dermatoses are an heterogeneous group of cutaneous manifestations that have a strong association with internal malignancy. Their pathogenesis is unclear and prevalence is unknown. Treatment consists of a therapeutic approach to the underlying disease. The importance of recognizingthem lies in the possibility of making an early diagnosis oftheneoplasm. 25- We report theclinical case of a patient with paraneoplastic dermatosis associated with a pancreatic neuroendocrine tumor.

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Resumen Presentamos el caso de una paciente que tenía un tumor del páncreas -denominado glucagonoma- y cuyo diagnóstico se sospechó por las manifestaciones cutáneas, las cuales nos condujeron realizar una tomografía axial computarizada (TAC). En ella se halló una masa. La paciente se remitió a cirugía y presentó una buena evolución.

Abstract This is a case report of a patient with a pancreatic tumor, known as glucagonoma, whose diagnosis was suspected because of skin manifestations which led to performing a CT scan, finding the mass. She underwent surgery with satisfactory results.

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Glucagonoma is a rare and slow-growing pancreatic tumor that usually manifests as glucagonoma syndrome. It is mainly characterized by a typical Dermatosis named necrolytic migratory erythema (NME), Diabetes and glucagon oversecretion. Deep vein thrombosis and Depression complete this set. We report the case of an advanced glucagonoma with liver spread, where all these 4D symptoms occurred but a chronic secretory Diarrhea was the most relevant feature. A 65-year-old man was referred to our center to investigate multiple hepatic nodules evidenced by abdominal tomography. He had a recent diagnosis of diabetes and complained of significant weight loss (25 kg), crusted skin lesions and episodes of a large amount of liquid diarrhea during the past 6 months. On admission, there were erythematous plaques and crusted erosions on his face, back and limbs, plus angular cheilitis and atrophic glossitis. The typical skin manifestation promptly led dermatologists to suspect glucagonoma as the source of our patient's symptoms. A contrast-enhanced abdominal computed tomography showed a hypervascularized pancreatic lesion and multiple hepatic nodules also hypervascularized in the arterial phase. Despite initial improvement of diarrhea after subcutaneous octreotide, the patient's impaired nutritional status limited other therapeutic approaches and he died of respiratory failure due to sepsis. His high levels of serum glucagon were not yet available so we performed an autopsy, confirming the diagnosis of metastatic glucagonoma with NME on histology. Chronic diarrhea is not a common feature in glucagonoma syndrome; however, its severity can lead to serious nutritional impairment and set a poor outcome.

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Abstract: Necrolytic acral erythema is a distinct erythema that has been described as an extrahepatic manifestation of hepatitis C virus infection. Most reported cases have been in Africa, especially Egypt. We report the first case (to the best of our knowledge) of necrolytic acral erythema in a Chinese patient with HCV and HBV coinfection. We aim to increase awareness for recognizing this condition in the Chinese population.

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Background: Glucagonomas are very rare neuroendocrine tumors of pancreatic endocrine islets alpha cells and they produced excessive amount of glucagon hormone. Necrolytic migratory erythema (NME) is a rare dermatosis that characterized by erosive, ulcerative and crusted lesions in different sites of the skin and the common cause of this situation related to glucagon secreted tumors. NME can occur commonly in man but some rare and recent reports available the occurrence of this situation in pet animals especially dogs. Both gross and histological findings in both human and animals are similar. This paper reports a NME case with glucagonoma and diabetes mellitus (DM) by clinical, histopathological and immunohistochemical examinations. Case: A 12-year-old, cat presented with complaints of skin lesions in neck region, hyperglycemia, weight loss and history of anorexia during the 2 months. Biochemical analysis results revealed high glucose, aspartate aminotransferase (AST), alanine aminotransferase (ALT), alkaline phosphatase (ALP) and creatinine levels but decreased blood urea nitrogen (BUN), total protein and potassium levels. After 2 weeks of anti-diabetic drug treatment, the blood glucose level became normal and skin lesions ameliorated but anorexia and weight loss continued. The cat exhibited general weakness and pain in abdominal area. Although the clinical sings ameliorated and skin lesions and serum biochemical findings returned the relatively normal levels compared the admission, the cat died after 2 weeks of treatment and necropsy performed. At necropsy, marked cachexia, loss of skin elasticity and decreased skin thickness were observed. During the examination of abdominal cavity of the cat, there was a mass 0.5x0.5 cm in diameter, hard and grayish color was diagnosed at the pancreas...(AU)

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Background: Glucagonomas are very rare neuroendocrine tumors of pancreatic endocrine islets alpha cells and they produced excessive amount of glucagon hormone. Necrolytic migratory erythema (NME) is a rare dermatosis that characterized by erosive, ulcerative and crusted lesions in different sites of the skin and the common cause of this situation related to glucagon secreted tumors. NME can occur commonly in man but some rare and recent reports available the occurrence of this situation in pet animals especially dogs. Both gross and histological findings in both human and animals are similar. This paper reports a NME case with glucagonoma and diabetes mellitus (DM) by clinical, histopathological and immunohistochemical examinations. Case: A 12-year-old, cat presented with complaints of skin lesions in neck region, hyperglycemia, weight loss and history of anorexia during the 2 months. Biochemical analysis results revealed high glucose, aspartate aminotransferase (AST), alanine aminotransferase (ALT), alkaline phosphatase (ALP) and creatinine levels but decreased blood urea nitrogen (BUN), total protein and potassium levels. After 2 weeks of anti-diabetic drug treatment, the blood glucose level became normal and skin lesions ameliorated but anorexia and weight loss continued. The cat exhibited general weakness and pain in abdominal area. Although the clinical sings ameliorated and skin lesions and serum biochemical findings returned the relatively normal levels compared the admission, the cat died after 2 weeks of treatment and necropsy performed. At necropsy, marked cachexia, loss of skin elasticity and decreased skin thickness were observed. During the examination of abdominal cavity of the cat, there was a mass 0.5x0.5 cm in diameter, hard and grayish color was diagnosed at the pancreas...

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Abstract: Necrolytic acral erythema is a rare skin disease associated with hepatitis C virus infection. We report a case of a 31-year-old woman with hepatitis C virus infection and decreased zinc serum level. Physical examination revealed scaly, lichenified plaques, well-demarcated with an erythematous peripheral rim located on the lower limbs. After blood transfusion and oral zinc supplementation the patient presented an improvement of lesions.

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Necrolytic migratory erythema is a rare paraneoplastic dermatosis that may be the first clinical manifestation of the glucagonoma syndrome, a disorder characterized by mucocutaneous rash, glucose intolerance, hypoaminoacidemia, hyperglucagonaemia and pancreatic glucagonoma. The clinical case of a 45-year-old woman is presented. She had been experiencing weight loss, polydipsia, polyphagia, postprandial emesis, excessive hair loss and abdominal pain for two months. Erythematous, scaly and migratory plaques with 20 days of evolution were found on her trunk, perineum, elbows, hands, feet, inframammary and antecubital folds. The skin biopsy revealed noticeable vacuolar changes in high epidermal cells, extensive necrosis and thin orthokeratotic cornified layer. These findings pointed to a diagnosis of necrolytic migratory erythema. A suggestion was made to investigate a pancreatic glucagonoma. Laboratory tests showed moderate anemia, hyperglycemia and marked hyperglucagonaemia. Abdominal ultrasound revealed a mass in the tail of the pancreas measuring 6 x 5 x 5 cm which was resected. The histopathological findings were compatible with a diagnosis of glucagonoma, as confirmed by immunohistochemistry. Skin symptoms disappeared 10 days after the tumor resection. We can conclude that the histological changes defined may be clues that can lead the search for a distant skin disease and allow for its diagnosis. The histological pattern of vacuolation and epidermal necrosis should arouse suspicion of pancreatic glucagonoma.

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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.

Necrolytic migratory erythema is a rare paraneoplastic dermatosis that may be the first clinical manifestation of the glucagonoma syndrome, a disorder characterized by mucocutaneous rash, glucose intolerance, hypoaminoacidemia, hyperglucagonaemia and pancreatic glucagonoma. The clinical case of a 45-year-old woman is presented. She had been experiencing weight loss, polydipsia, polyphagia, postprandial emesis, excessive hair loss and abdominal pain for two months. Erythematous, scaly and migratory plaques with 20 days of evolution were found on her trunk, perineum, elbows, hands, feet, inframammary and antecubital folds. The skin biopsy revealed noticeable vacuolar changes in high epidermal cells, extensive necrosis and thin orthokeratotic cornified layer. These findings pointed to a diagnosis of necrolytic migratory erythema. A suggestion was made to investigate a pancreatic glucagonoma. Laboratory tests showed moderate anemia, hyperglycemia and marked hyperglucagonaemia. Abdominal ultrasound revealed a mass in the tail of the pancreas measuring 6 x 5 x 5 cm which was resected. The histopathological findings were compatible with a diagnosis of glucagonoma, as confirmed by immunohistochemistry. Skin symptoms disappeared 10 days after the tumor resection. We can conclude that the histological changes defined may be clues that can lead the search for a distant skin disease and allow for its diagnosis. The histological pattern of vacuolation and epidermal necrosis should arouse suspicion of pancreatic glucagonoma.

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Summary Introduction: glucagonoma is a pancreatic neuroendocrine tumor derived from alpha-cells of the islets of Langerhans. It is marked by tumoral autonomous production of glucagon and characterized, among other symptoms, by necrolytic migratory erythema, an erythematous circinate lesion with areas of necrosis and sloughing. This is a rare disease with worldwide incidence estimated at 1 case per 20 million people. Case report: we report a case of glucagonoma associated necrolytic migratory erythema in a male patient, 56 years, with signs of skin lesions mainly on his legs and groin, hyperglycemia and weight loss. Biopsies of the skin lesions were performed and imaging of the abdomen showed a mass of 10 x 9 cm, at the pancreatic region. The patient was subjected to body-caudal pancreatectomy and splenectomy with autotransplant of the spleen in the greater omentum. The histopathologic report indicated a tumor in the pancreatic alpha cells. Immunohistochemistry showed expression of glucagon and chromogranin A in most tumor cells, consistent with the diagnosis of glucagonoma. The patient presented 3 years of outpatient follow-up with no complications. Conclusion: the necrolytic migratory erythema is important for the clinical recognition of glucagonoma, and its early diagnosis is essential for a successful curative therapy. .

Resumo Introdução: Introdução: o glucagonoma é um tumor neuroendócrino do pâncreas derivado das células alfa das ilhotas de Langerhans. É marcado pela produção tumoral autônoma de glucagon e caracterizado, dentre outros sintomas, por eritema necrolítico migratório (ENM), uma lesão eritematosa circinada com áreas de necrose e descamação. Trata-se de uma doença rara com incidência mundial estimada em 1 caso para cada 20 milhões pessoas. Relato de caso: apresentamos um caso de glucagonoma associado a ENM em um paciente de sexo masculino, 56 anos de idade, com quadro de lesões cutâneas, principalmente em membros inferiores e região inguinal, hiperglicemia e perda ponderal. Biópsias das lesões cutâneas foram realizadas e exames de imagem do abdome evidenciaram uma massa de 10 x 9 cm em região pancreática. O paciente foi submetido à pancreatectomia corpocaudal e esplenectomia total com autoimplante do baço em omento maior. O laudo histopatológico foi de tumor de células alfa pancreáticas. Imuno-histoquímica evidenciou expressão de glucagon e cromogranina A na maioria das células tumorais, compatível com diagnóstico de glucagonoma. O paciente apresentou seguimento de 3 anos em ambulatório sem intercorrências clínicas. Conclusão: o ENM é importante para o reconhecimento clínico do glucagonoma, sendo seu diagnóstico precoce fundamental para uma terapia curativa de sucesso. .

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O eritema necrolítico migratório é uma síndrome cutânea rara em humanos e cães muitas vezes associadaà presença de tumores pancreáticos produtores de glucagon. Pacientes com esta síndrome apresentamfrequentemente, além das alterações dermatológicas associadas à necrose epidérmica, a perda de peso e o diabetes mellitus, como consequência da hiperglucagonemia. Apenas um caso de síndrome do glucagonoma canino foi relatado até o momento na literatura nacional e aproximadamente dez casosexistem na literatura mundial. Relatamos um caso de eritema necrolítico migratório em uma cadeladiabética da raça Cocker Spaniel com oito anos de idade e quadro clínico compatível com a síndromedo glucagonoma. (AU)

The necrolytic migratory erythema is a rare skin syndrome in humans and dogs often associated with the presence of glucagon-producing pancreatic tumors. Patients with this syndrome develop epidermal necrosis generally associated with weight loss and diabetes mellitus as a consequence of hyperglucagonemia.Only one case of canine glucagonoma syndrome has been reported so far in the nacional literature and there are about ten cases all over the world. We report a case of necrolytic migratory erythema in an eight year-old, female, diabetic Cocker Spaniel dog with a clinical history compatible withthe glucagonoma syndrome. (AU)

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O eritema necrolítico migratório é uma síndrome cutânea rara em humanos e cães muitas vezes associadaà presença de tumores pancreáticos produtores de glucagon. Pacientes com esta síndrome apresentamfrequentemente, além das alterações dermatológicas associadas à necrose epidérmica, a perda de peso e o diabetes mellitus, como consequência da hiperglucagonemia. Apenas um caso de síndrome do glucagonoma canino foi relatado até o momento na literatura nacional e aproximadamente dez casosexistem na literatura mundial. Relatamos um caso de eritema necrolítico migratório em uma cadeladiabética da raça Cocker Spaniel com oito anos de idade e quadro clínico compatível com a síndromedo glucagonoma.

The necrolytic migratory erythema is a rare skin syndrome in humans and dogs often associated with the presence of glucagon-producing pancreatic tumors. Patients with this syndrome develop epidermal necrosis generally associated with weight loss and diabetes mellitus as a consequence of hyperglucagonemia.Only one case of canine glucagonoma syndrome has been reported so far in the nacional literature and there are about ten cases all over the world. We report a case of necrolytic migratory erythema in an eight year-old, female, diabetic Cocker Spaniel dog with a clinical history compatible withthe glucagonoma syndrome.

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Presentamos el caso de una paciente de 62 años con un cuadro de tres años de evolución, caracterizado por placas y pápulas eritematosas arciformes que comenzaron en cara, extendiéndose luego al resto del cuerpo, asociado a baja de peso y depresión. Después de un completo estudio y dos biopsias de piel se diagnóstica eritema necrolítico migratorio (ENM). El ENM junto con estomatitis/glositis, baja de peso, diarrea, diabetes y anemia forman el síndrome paraneoplásico asociado al tumor de células a pancreáticas, llamado síndrome del glucagonoma, El ENM corresponde a lesiones maculopapulares, coalescentes, de borde serpiginoso, acompañadas de una bula central que se erosiona y forma costras. La biopsia cutánea muestra hiperplasia psoriasiforme y espongiótica, paraqueratosis y separación de las capas superficiales de la epidermis. La resección del tumor conduce a la resolución del ENM. Presentamos este caso y revisión del tema por la baja frecuencia de esta enfermedad y para reforzar lo importante de su sospecha temprana.

We report the case of o 62 year old woman with a 3 year history of erythematous arciform plaques and papules that began in the face and spread to the rest of the body. These lesions were associated with depression and weight loss. After a comprehensive study and two skin biopsies, necrolytic migratory erythema (NME) was diagnosed together with glossitis/stomatitis, weight loss, diarrhea, diabetes and anemia, NME is part of the paraneoplastic syndrome associated with a cell pancreatic tumor, known as glucagonoma syndrome NME lesions are characterized by a coalescent maculopapular rash with a serpiginous edge and a central bulla that erodes and become crusted. Histological studies show a psoriasiform and spongiotic hyperplasia, porakeratosis, and detachment of the superficial layers of the epidermis. ENM usually resolves after tumor resection. We present this case and a review of the literature because of the low frequency of this disease and to reinforce the importance of its early suspicion.

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A necrose epidérmica metabólica é uma rara enfermidade endócrino-metabólica, com importantes manifestações cutâneas, possuindo paralelo clinicopatológico com o eritema necrolítico migratório em humanos. O presente estudo tem por objetivo relatar três casos clínicos de NEM em cães acometidos por dermatite eritematosa, crostosa e ulcerativa associada à hepatopatia. O diagnóstico definitivo foi alcançado a partir do exame dermatopatológico. O prognóstico é desfavorável e os três animais morreram, apesar do tratamento.

Metabolic epidermal necrosis is a rare endocrine metabolic disease, characterized by dermatological signs which has clinical and pathological similarity with necrolytic migratory erythema in humans. The present report describes three cases of MEN in dogs affected with erythematous ulcerative crusting dermatitis associated with hepatopathy. The diagnosis was based on cutaneous histopathology. The prognosis is poor and the three dogs died despite the treatment.