Background. We report a case of necrolytic migratory erythema in a patient with Waldmann’s disease. Patients and methods. A 55-year-old male patient with a history of Waldmann’s disease was hospitalized for a rash o...Background. We report a case of necrolytic migratory erythema in a patient with Waldmann’s disease. Patients and methods. A 55-year-old male patient with a history of Waldmann’s disease was hospitalized for a rash on the trunk and limbs comprising annular polycyclic lesions with peripheral scaling evocative of necrolytic migratory erythema. High-protein and fatty-acid-supplemented parenteral feeding led to rapid improvement of the patient’s cutaneous lesions. Discussion. Waldmann’s disease is characterized by intestinal lymphatic abnormalities leading to exudative intestinal disease causing protein loss in the bowel lumen and deficient fatty acid absorption. The pathogenesis of necrolytic migratory erythema is not fully understood. Increased serum glucagon does not appear to be the only mechanism involved. The occurrence of necrolytic migratory erythema in a patient with Waldmann’s disease supports the current physiopathological hypothesis of the role of decreased plasma protein and amino acid levels in necrolytic migratory erythema.展开更多
Background: Necrolytic migratory erythema is considered to be a paraneoplastic dermatosis. The classical symptoms are associated with α-cell pancreatic islet cell tumor or ‘glucagonoma’. Generally, extracutaneous h...Background: Necrolytic migratory erythema is considered to be a paraneoplastic dermatosis. The classical symptoms are associated with α-cell pancreatic islet cell tumor or ‘glucagonoma’. Generally, extracutaneous hallmarks of this disease include weight loss, diabetes, anaemia and diarrhoea. Observation: We report a case of a 39-year-old woman with a 3-year history of recalcitrant psoriasiform eruption, who had no other associated symptoms on routine examination. Histologic examinations suggested necrolytic migratory erythema. Abdominal computer tomography was performed, which revealed a tumor in the tail of the pancreas. After distal resection of the pancreas her skin symptoms resolved in a few days time. Histology was consistent with glucagonoma. She is clinically well and symptomless and no signs of metastasis after 4 years. Conclusions: It is infrequent to have only necrolytic migratory erythema, hyperglucagonaemia and islet-cell tumor but no other extracutaneous symptoms in glucagonoma syndrome. To our knowledge, ours is the second such case reported in the literature. Skin symptoms are important, often they are the clue to the diagnosis of glucagonoma syndrome.展开更多
文摘Background. We report a case of necrolytic migratory erythema in a patient with Waldmann’s disease. Patients and methods. A 55-year-old male patient with a history of Waldmann’s disease was hospitalized for a rash on the trunk and limbs comprising annular polycyclic lesions with peripheral scaling evocative of necrolytic migratory erythema. High-protein and fatty-acid-supplemented parenteral feeding led to rapid improvement of the patient’s cutaneous lesions. Discussion. Waldmann’s disease is characterized by intestinal lymphatic abnormalities leading to exudative intestinal disease causing protein loss in the bowel lumen and deficient fatty acid absorption. The pathogenesis of necrolytic migratory erythema is not fully understood. Increased serum glucagon does not appear to be the only mechanism involved. The occurrence of necrolytic migratory erythema in a patient with Waldmann’s disease supports the current physiopathological hypothesis of the role of decreased plasma protein and amino acid levels in necrolytic migratory erythema.
文摘Background: Necrolytic migratory erythema is considered to be a paraneoplastic dermatosis. The classical symptoms are associated with α-cell pancreatic islet cell tumor or ‘glucagonoma’. Generally, extracutaneous hallmarks of this disease include weight loss, diabetes, anaemia and diarrhoea. Observation: We report a case of a 39-year-old woman with a 3-year history of recalcitrant psoriasiform eruption, who had no other associated symptoms on routine examination. Histologic examinations suggested necrolytic migratory erythema. Abdominal computer tomography was performed, which revealed a tumor in the tail of the pancreas. After distal resection of the pancreas her skin symptoms resolved in a few days time. Histology was consistent with glucagonoma. She is clinically well and symptomless and no signs of metastasis after 4 years. Conclusions: It is infrequent to have only necrolytic migratory erythema, hyperglucagonaemia and islet-cell tumor but no other extracutaneous symptoms in glucagonoma syndrome. To our knowledge, ours is the second such case reported in the literature. Skin symptoms are important, often they are the clue to the diagnosis of glucagonoma syndrome.
