Síndrome de Stevens-Johnson y necrólisis epidérmica tóxica en una lactante / Stevens-Johnson Syndrome and toxic epidermic necrolysis in an infant girl

Se describe el caso clínico de una lactante de 6 meses de edad, residente en el municipio de Palma Soriano, de la provincia de Santiago de Cuba, con antecedente de buena salud, atendida por su médico de familia por presentar lesiones en la piel. En esta ocasión se le diagnosticó impétigo contagioso, para lo cual fue tratada con azitromicina; al séptimo día presentó fiebre de 40 °C y nuevas lesiones en la cara, el tronco, así como en los miembros inferiores y superiores, con ampollas y costras, de manera que fue ingresada en el Hospital Pediátrico de su municipio, con el diagnóstico de síndrome mano-boca-pie. Se le indicó tratamiento sintomático y 24 horas después comenzó a presentar toma del estado general, inflamación de las mucosas bucal, conjuntival, vulvar y anal, además de zonas eritematosas y húmedas, que se extendían por casi toda la superficie corporal. El signo de Nikolski resultó positivo y se corroboró que la paciente presentaba síndrome de Stevens Johnson y necrólisis tóxica epidérmica, pero evolucionó favorablemente

The case report of a 6 months infant girl from Palma Soriano municipality, in Santiago de Cuba who had a history of good health is described. She was assisted by her family doctor due to lesions in the skin. In this occasion she was diagnosed impetigo contagiosa, reason why she was treated with azitromicin; at the seventh day she had fever (40 °C) and new lesions in the face, trunk, as well as in the lower and upper members, with blisters and scabs, so that she was admitted in the Pediatric Hospital of her municipality, with the diagnosis of hand-mouth-foot syndrome. She was indicated symptomatic treatment and 24 hours later her general state began to decline, inflammation of the oral, conjuntival, vulvar and anal mucous, besides erithematous and humid areas that extended for almost the whole body surface. Nikolski sign was positive and it was corroborated that the patient presented the Stevens Johnson syndrome and epidermic toxic necrolysis, but she had a favorable clinical course

Eritema necrolítico migratorio y glucagonoma pancreático / Necrolytic migratory erythema and pancreatic glucagonoma

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.

Glucagonoma Syndrome: A case report / Journal of the ASEAN Federation of Endocrine Societies

@#A 58-year-old Malay female with underlying diabetes mellitus, presented with chronic skin lesions, associated with weight loss and anemia. There were erosive, scaling skin lesions over the extremities, gluteal region and perioral area. Skin biopsy histopathological examination revealed Necrolytic Migratory Erythema (NME). A CT scan of the abdomen revealed a pancreatic neck and body tumor with possible liver metastases. She was successfully treated with subcutaneous somatostatin and underwent distal pancreatectomy with wedge resection of liver nodule.

Glucagonoma syndrome associated with necrolytic migratory erythema / Síndrome do glucagonoma associado a eritema necrolítico migratório

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. .

Metastatic glucagonoma presenting with weight loss and necrolytic migratory erythema / Journal of the ASEAN Federation of Endocrine Societies

@#A 42-year-old Filipino female was admitted due to weight loss and recurrent abdominal pain over the past ten years. In 2010, she was diagnosed to have disseminated PTB associated with a liver mass. After 1 year of anti-TB treatment, lung condition was treated but her liver mass has enlarged. She also developed diarrhea, diabetes, and skin lesions with biopsy results showing Necrolytic Migratory Erythema (NME). CT scan showed liver and pancreatic tumors that were biopsied revealing a neuroendocrine tumor. Blood glucagon level was elevated. She was treated as a case of glucagon-secreting tumor with liver metastases with Everolimus and Octreotide. After 3 months of treatment, she gained weight, the skin lesions improved, and the liver mass decreased in size. Many of the initial symptoms of glucagonoma are nonspecific and subtle. As a result, glucagonoma is often diagnosed relatively late in the course of the disease. NME, the characteristic skin lesion of the glucagonoma syndrome, is often the clue that leads to the correct diagnosis.

Prolonged Neutropenia after Sunitinib Treatment in a Patient with a Glucagonoma and Multiple Hepatic Metastases / 대한내과학회지

Pancreatic neuroendocrine tumors (PNET) are rare, with approximately 2.2 in 1,000,000 people affected annually. In the classification of neuroendocrine tumors, glucagonomas are a functional PNET and comprise 1.6% of PNET. Glucagonoma syndrome is a paraneoplastic syndrome that is characterized by necrolytic migratory erythema, weight loss, anemia, and diabetes mellitus. Metastatic disease at presentation is common, but is often limited to the liver and regional lymph nodes. Sunitinib malate improves the progression-free and overall survival of PNET. This report presents a 45-year-old Asian woman with prolonged neutropenia after sunitinib treatment of a glucagonoma with multiple hepatic metastases. The severity of the neutropenia after the sunitinib treatment fluctuated from grade 1 to 4 repeatedly, with a non-febrile pattern. Ultimately, the patient did not recover from the neutropenia, even after stopping the sunitinib.

A Case of Glucagonoma Syndrome Diagnosed as Necrolytic Migratory Erythema / 대한피부과학회지

Necrolytic migratory erythema (NME) is a typical cutaneous manifestation of glucagonoma syndrome. The entire syndrome consists of NME, glucose intolerance, weight loss, anemia, glossitis, diarrhea, and increased glucagon levels. We herein report a patient with glucagonoma syndrome who was diagnosed as having NME. A 48-year-old male presented with a 2-month history of painful erythematous, desquamative, erosive papules and plaques on both lower extremities. Histological examination revealed an intraepidermal cleft, the presence of vacuolated, pale epidermal cells, and necrosis in the upper epidermis. His glucagon level was 2650 pg/ml, with the upper limit of a normal range being 250 pg/ml. The patient was treated with octreotide, and showed an improvement of the skin eruption with normalization of the glucagon level within 4 weeks.

Malignant Glucagonoma of the Pancreas: A case report / 대한내분비외과학회지

Glucagonomas are rare pancreatic tumors of islet ahpha-2 cells. Less than 430 cases have been reported worldwide and 210 cases are malignant tumors. In generally, the tumors typically present with a characteristic constellation of symptoms including necrolytic migratory erythema of the skin, weight loss, non-insulin-dependent diabetes mellitus, anemia, cheliosis, stomatitis, and an increased thrombotic tendency. Since pancreatic glucagonomas are predominantly located in the tail and findings of radiographic or sonographic examination can remain unspecific, patients often present already metastasis when diagnosis is first established, and can be difficult to differentiate from the other pancreatic tumors. We report the case of a 59-year-old woman with an malignant glucagonoma of the pancreas infiltrating already the spleen and presenting metastatic lesion in perirenal lymph nodes, and that the tumor was not assocated with the characteristic skin rash. The pateint with a past history of a diabetes mellitus and hypertension for 9 years was admitted with cramp-like left lower abdominal pain, watery diarrhea, and nausea. A solid tumor of tail of the pancreas revealed by ultrasonography and abdominal computed tomography and distal pancreatectomy, radical nephrectomy, and splenectomy were performed. Immunohistochemial examination of the tumor did show glucagon-reactive tissue and electron microscopy revealed many secretory granules, 180 to 300 nm in diameter in granulated cells. After pancreatic tumor resection, the patient had normalization of plasma glucagon and blood sugar.

A Case of pseudoglucagonoma Syndrome Treated with Medium-chain Triglyceride / 대한피부과학회지

Necrolytic migratory erythema is a rare, distinctive, necrotizing cutaneous eruption usually associated with glucagonoma, an alpha-cell tumor of the pancreas. Other clinical features include ane- mia, diabetes mellitus and weight loss. However, it also occurrs without the pancreatic glucagonoma. So this rare condition is called pseudoglucagonoma syndrome, and has been described in association with chronic hepatic diseases, malabsorption with villous atrophy, chronic pancreatitis, celiac sprue, hypoproteinemia and odontogenic infection. We report a case of pseudoglucagonoma syndrome induced by malnutrition without glucagonoma. A 28-year-old Korean female was referred to our department complaining of multiple erythematous, desquamative, crusted and erosive patches on the extremities, and diffuse scalp alopecia for 5 years. She also had perianal erosion, angular cheilitis without glossitis, amenorrhea, axillary and pubic hair loss, and weight loss. Six years previously she had had Whipples operation for a pancreatic solid and papillary neoplasm. The laboratory data showed hypoalbuminemia, essential fatty acid deficiency, diabetes mellitus and slightly high levels of zinc and glucagon. A skin biopsy showed irregular acanthosis, epidermal edema and pallor, dyskeratosis, areas of superficial epidermal necrosis and vascular dilatation of the papillary dermis. The patient improved dramatically after intravenous hyperalimentation and oral intake of mediurn chain triglyceride. For 2.5 years she has taken medium-chain triglycerides without any other treatment, and no side effects on recurrence has been observed.

A Case of Necrolytic Migratory erythema Induced by a Pancreatic Insufficiency / 대한피부과학회지

Necrolytic migratory erythema is a cutaneous reaction pattern with specific histopathologic features that is typically associated with a functioning pancreatic islet cell neoplasm such as a glucagonoma. However, cases without. glucagonoma have also been reported, such as, liver cirrhosis, chronic pancreatitis, celiac sprue etc. Other clinical features include anemia, glossitis and weight loss. We report a case of neccrolytic migratory erythema induced by a pancreatic insufficiency without glucagonoma. A 43-year-old male was seen at our department because of rnultiple erythematous, desquamative and erosive patches on the whole body for 1 month. He also had weight loss(25Kg) and stomatitis. Seven years previously he had a Whipple's procedure for a pancreatic head rupture. The laboratory data showed a low protein and zinc level, and high glucagon level. Histopathologic findings of the case showed epidermal edema and pallor, and superficial epiderrnal necrosis. The patient improved progressively after intravenous infusion of amino acids with a pancreatic enzyme supplementation.