Necrolytic migratory erythema without glucagonoma

Abstract

Two patients with clinical and histologic findings consistent with necrolytic migratory erythema are presented. Unlike previously described patients with this disorder, neither patient had substantially elevated glucagon levels nor an associated pancreatic islet cell tumor. The cause of the skin disease in these patients remains unknown but may be related to the underlying small-bowel disorder present in both.

The Glucagonoma Syndrome: Clinical Features, Diagnosis, and Treatment

“…Such levels usually exceed not only those obtained in normal subjects but also the moderately elevated concentrations observed in diabetes (38)(39)(40)(41), pancreatitis (42), malabsorption (43), cirrhosis (44)(45)(46), infection (47), trauma (48,49), or other nontumorous conditions. Basal hormone concentrations in glucagonoma patients range between 320-96,000 pg/ml, although the majority fall between 1,000-5,000 pg/ml.…”

“…That the skin lesions of the syndrome are due in some way to tumor elaboration of glucagon, rather than some other substance, is suggested by a description of a less severe, but typical, eruption that occurred after exogenous glucagon administration (125). In addition, necrolytic migratory erythema has been reported in one patient (44), with chronic active hepatitis and cirrhosis, whose plasma glucagon ranged between 800-1100 pg/ml and in another subject (43), with small bowel villous atrophy and malabsorption, whose plasma glucagon was 475 pg/ml.…”

Plattenepithelkarzinomassoziiertes nekrolytisches migratorisches Erythem

“…nach Tumormanifestation auftreten können [18]. Charakteristischerweise heilen die [7], Gluten-sensitiver Enteropathie [11,16,25] und Bronchialkarzinom [13] finden sich in der Literatur. Ein NME ohne Hinweise auf eine Pankreaserkrankung oder eine sonstige schwerwiegende innere Erkrankung liegen gleichfalls vor [21].…”

“…Aufgrund verschiedener Fallberichte wird die obligate Assoziation des NME mit einem Glukagon-sezernierenden Tumor des Pankreas wiederholt in Frage gestellt [1,2,7,8,11,13,15,16,19,20,21,24,26]. Während bei unserem Patienten autoptisch eine subakute Stauung der Leber sowie serologisch eine Immunität gegen das Hepatitis-A-Virus …”

“…Das NME gilt als eine obligate kutane Paraneoplasie bei zugrundeliegendem Glukagon-produzierenden Tumor der α 2 -Zellen des Pankreas [4]. Aufgrund verschiedener Fallberichte über das nekrolytische migratorische Erythem ohne Nachweis eines Glukagonoms wurde ein notwendiger Kausalzusammenhang wiederholt in Frage gestellt [1,2,7,8,11,13,15,16,19,20,21,24,26]. …”