Hpi 73 f with dm, hypertension, atrial fibrillation on warfarin who presented with vomiting, diarrhea, hyperglycemia ems took blood sugar which was in the 490s. Weakly associated with internal malignancy are acanthosis nigricans, acquired ichthyosis, and multiple seborrheic keratoses. Psychiatric symptoms, weight loss, necrolytic migratory erythema, diarrhea, and diabetes mellitus developed gradually after diagnosis of the tumor. Darier in 1916 was the first to use the term erythema annulare centrifugum 1 eac, although similar lesions had been described previously under other names. Case presentationcase presentation evaluated by patientevaluated by patient ss gynecologist gynecologist. Necrolytic migratory erythema associated with a glucagonproducing primary hepatic neuroendocrine carcinoma in a cat. The function of hypothalamic glucagon is incompletely understood and currently no clinical disorders of hypothalamic glucagon function. Erythema gyratum repens dramatic erythematous concentric scaly rings woodgrain appearance. A patient who survived for 21 years since initial discovery of glucagonoma with concurrent liver metastases is described. Necrolytic migratory erythema nme is a rare skin disorder which was first described by becker et al. Necrolytic migratory erythema painful glossitis, angular stomatitis.
In this paper, the authors describe a patient with. Glucagonoma with necrolytic migratory erythema exhibiting responsiveness to subcutaneous octreotide injections. Glucagonoma is a rare, functioning type of pancreatic neuroendocrine tumor pnet. What links here related changes upload file special pages permanent link page information. We report a case of necrolytic migratory erythema nmelike skin lesions developing during treatment with gefitinib. I read with interest the report by goodenberger et al1 of two cases of necrolytic migratory erythema without glucagonoma. Necrolytic acral erythema bears microscopic and clinical resemblance to other necrolytic erythemas, including necrolytic migratory erythema nme and several nutrientdeficient syndromes. Glucagonoma syndrome is a rare paraneoplastic phenomenon, with an estimated incidence of one in 20 million, characterized by necrolytic migratory erythema, hyperglucagonemia, diabetes mellitus, anemia, weight loss, glossitis, cheilitis, steatorrhea, diarrhea, venous thrombosis and neuropsychiatric disturbances in the setting of a glucagonproducing alphacell tumor of the pancreas. The skin lesions can arbitrarily be classified into either early or late associations table 1 where early. Necrolytic migratory erythema glucagonoma like skin lesions induced by. Check this box if you wish to receive a copy of your message. Media in category erythemas the following 42 files are in this category, out of 42 total. A hallmark sign of this syndrome is a rash known as necrolytic migratory erythema nme.
The classical symptoms are associated with alphacell pancreatic islet cell tumor or glucagonoma. An unusual cause for weight loss and diabetes hkmj. Glucagonoma and the glucagonoma syndrome cumulative. Necrolytic migratory erythema may affect any site but it most often affects the genital and anal region, the buttocks, groin and lower legs. Background pancreatic masses are rare in the pediatric population pancreatic tumors only account for 0. Also within 14 days, amend all pending supplemental applications that include. Excessive glucagon action produces a catabolic state resulting in weight loss. Elevated chromogranin a cga and neuronspecific enolase nse levels were also manifested, whereas carbohydrate antigen 199 ca199 and carcinoembryonic antigen cea were unremarkable. Our search in the english literature of such a kind of association did not reveal any case reported. Hypotension has been reported up to 2 hours after administration in patients receiving glucagen as premedication for upper gi.
Ct scan dil t d ti d t ith b t t ffdilated pancreatic duct with abrupt cutoff. A 44yearold woman was diagnosed with type ii diabetes in 1998 and 1 year later she developed necrolytic migratory erythema, which is a specific skin lesion of glucagonoma. The clinical mucocutaneous manifestations of glucagonoma syndrome are recognized easily when they occur in the classic pattern of acral or periorificial lesions evolving in recurrent crops, with an annular and migratory distribution, in a patient. His skin presented with necrolytic migratory erythema nme, anemia and other morphologies. Pdf necrolytic migratory erythema associated with glucagonoma. Glucagon cell hyperplasia and neoplasia with and without. Information on submitting spl files using elist may be found in the guidance for industry titled.
Necrolytic migratory erythma nme as a rare skin disorder that can affected perineum, distal extremities, lower abdomen and face are the most. Erythema nodosum erythema nodosum2 erythema nodosum3 fixed drug reaction gianotticrosti syndrome glucagonoma syndromenecrolytic migratory erythema flame figures gout gout2 graft versus host disease granuloma annulare. Initially there is a ringshaped red area that blisters, erodes and crusts over. Table 431 lists the figurate erythemas and the differential diagnoses to consider. Necrolytic migratory erythema is the distinctive skin eruption of the glucagonoma syndrome, and this eruption led to the diagnosis in many of the cases reported.
Clinical pearls in dermatology american college of. Necrolytic migratory erythema swiss medical weekly. Figurate erythema, erythema annulare centrifugum, erythema gyratum repens, erythema migrans, necrolytic migratory erythema. Glucagonoma syndrome is a rare disease that is usually associated with an underlying neuroendocrine tumor. Patient 3 which metabolic abnormality is most likely in. Pdf the glucagonoma syndrome and necrolytic migratory.
Acrodermatitis enteropathica belongs to the family of necrolytic erythemas, which include necrolytic migratory erythema, necrolytic acral erythema, pellagra and essential fatty acid deficiencies. In the know with rnao assessment and management of. Necrolytic migratory erythema is considered to be a paraneoplastic dermatosis. Second most common pancreatic tumor found in men 1. Glucagonoma genetic and rare diseases information center. Glucagonoma with necrolytic migratory erythema exhibiting. All structured data from the file and property namespaces is available under the creative commons cc0 license. Necrolytic migratory erythema erythema toxicum erythroderma palmar erythema generalized erythema. Vulvar ulcers vulvar ulcersdiagnostic algorithm clinical pattern and biopsies 9 bullous dermatoses a autoimmune bmm pemphigoid p. Retroperitoneal liposarcoma associated with small plaque. An introduction to dermatology 1905 erythema bullosum.
Glucagon is a singlechain polypeptide of 29 amino acids that is derived from a larger precursor peptide big plasma glucagon, which is cleaved upon secretion. Srj is a eritroddermia metric based on the idea that not all citations are the same. It is distinct, however, in its predominantly acral distribution and strong association with hcv infection. Dailymed glucagen hypokit glucagon hydrochloride kit. The main sites of glucagon production are the hypothalamus and pancreatic alphaislet cells. Pdf the glucagonoma syndrome is a rare disease in which a typical skin disorder, necrolytic migratory erythema, is often one of the first presenting. Necrolytic migratory erythema as the first manifestation of.
Papulosquamous hyperkeratotic cutaneous conditions medical mnemonics. Nme is the most characteristic clinical manifestation of glucagonoma and also the core diagnostic criteria in most cases. They are stored as adobe pdf files and you will need a reader to access these. Necrolytic migratory erythma nme is an obligatory paraneoplastic syndrome. Post marketing reports of a skin rash known as necrolytic migratory erythema nme has been reported following continuous glucagon infusion and resolved upon discontinuation secondary hypoglycemia may occur when glucagon is administered to patients with glucagonoma drug interactions. Necrolytic migratory erythema is a red, blistering rash that spreads across the skin. Although other granules may be present, in most cases beta and delta granules are not seen. Here we describe a woman admitted to the dermatology ward with nme which was later found to be associated with glucagonoma, a slowgrowing, rare pancreatic neuroendocrine tumor. The differential diagnosis of nae includes necrolytic migratory erythema nme, acrodermatitis enteropathicazinc deficiency, biotin or fatty acid deficiency, pel. Histologically similar to other necrolytic erythemas necrolytic migratory erythema, pellagra, acrodermatitis enteropathica. Necrolytic migratory erythema as the first manifestation of pancreatic. S y kills multiple erythematous papules on a 6yearolds face. Herein, we present a case that we believe is the first report of the sign.
Paraneoplastic skin syndromes melissa piliang, md dermatology and anatomic pathology cleveland clinic. Necrolytic migratory erythema jama dermatology jama network. The classic cutaneous findings include symmetric, erythematous, scaly, eczematous, vesicularbullous or pustular eruption on the distal surfaces and. The present report describes a case of retroperitoneal liposarcoma associated with small plaque parapsoriasis. Dermatoses with high associations with internal malignancy include tripe palms, bazex syndrome, paraneoplastic pemphigus, erythema gyratum repens, necrolytic migratory erythema, and hypertrichosis lanuginose acquisita. Necrolytic migratory erythema nme, diabetes mellitus and glucagonsecreting tumors form the hallmarks of glucagonoma syndrome, and represent the major clinical manifestations of glucagonoma. The spl will be accessible from publicly available labeling repositories. Some skin findings may be more specific to the diagnosis of dm e. Perineum, distal extremities, lower abdomen, and face are the most commonly affected sites. This information pertains to the risk of necrolytic migratory erythema.
Necrolytic migratory erythema is observed in about 70% of patients with glucagonoma, an uncommon disease. Necrolytic migratory erythema is a presenting feature in 70% of cases of glucagonoma syndrome and can be characterized by episodic eruption of scaly, erosive, annular erythematous plaques with pustular and blister formation. Necrolytic migratory erythema glucagonoma syndrome clinicopathological correlation. Necrolytic migratory erythema as the first manifestation.
Initially the lesion is characterized by erythema of varying shapes, along with a pale bulge at the center of erythema, showing herpeslike. Files are available under licenses specified on their description page. This page was last edited on 12 october 2019, at 11. Necrolytic migratory erythema nme, a skin rash commonly associated with glucagonomas glucagon producing tumors and characterized by scaly, pruritic erythematous plaques, bullae, and erosions, has been reported postmarketing following continuous glucagon infusion. Necrolytic migratory erythemalike skin lesion during gefitinib.
Pdf necrolytic migratory erythema is a rare skin condition that consists of migrating areas of erythema with blisters that heal with. This patient did not have necrolytic migratory erythema, which is reportedly absent in 20% of persons having glucagonomas. Necrolytic migratory erythema see warnings and precautions side effects may include nausea and vomiting at doses above 1 mg or with rapid injection. In humans, necrolytic migratory erythema nme is a syndrome with a characteristic skin rash that is associated most often with a pancreatic glucagonoma and is recognized as part of the glucagonoma syndrome.
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