RESUMO
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.
Assuntos
Animais , Feminino , Cães , Eritema Migratório Necrolítico/veterinária , Eritema Migratório Necrolítico/diagnóstico por imagem , Dermatopatias/veterinária , Ultrassonografia/veterináriaRESUMO
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
Assuntos
Humanos , Feminino , Lactente , Síndrome de Stevens-Johnson , Eritema Migratório Necrolítico , DermatopatiasRESUMO
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)
Assuntos
Animais , Gatos , Eritema Migratório Necrolítico/veterinária , Glucagonoma/veterinária , Neoplasias Pancreáticas/veterinária , Diabetes MellitusRESUMO
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...
Assuntos
Animais , Gatos , Eritema Migratório Necrolítico/veterinária , Glucagonoma/veterinária , Neoplasias Pancreáticas/veterinária , Diabetes MellitusRESUMO
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.
Assuntos
Glucagonoma/patologia , Hiperglicemia/patologia , Eritema Migratório Necrolítico/patologia , Necrose/fisiopatologia , Neoplasias Pancreáticas/diagnóstico , Pele/patologia , Biópsia , Feminino , Glucagonoma/diagnóstico , Humanos , Eritema Migratório Necrolítico/etiologiaRESUMO
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.
Assuntos
Glucagonoma , Eritema Migratório Necrolítico , Eritema , Neoplasias Pancreáticas , Síndromes Paraneoplásicas , Manifestações CutâneasRESUMO
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.
Assuntos
Glucagonoma/complicações , Eritema Migratório Necrolítico/etiologia , Neoplasias Pancreáticas/complicações , Biópsia , Humanos , Masculino , Pessoa de Meia-Idade , Eritema Migratório Necrolítico/patologia , Pele/patologiaRESUMO
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. .