Necrolytic Migratory Erythema

Abstract

A man in his 40s presented with a 7-year history of a pruritic, annular cutaneous eruption and flaccid vesicles. The eruption was episodic in nature with spontaneous exacerbations and remissions. He reported modest weight loss but was otherwise well. Physical examination revealed crusted papules and polycyclic plaques with a map-like margin mainly involving the perioral region, chest, groin, buttocks, and extremities (Figure). His tongue appeared beefy red and his nails were brittle with transverse fissuring. A skin biopsy was performed and showed necrolysis in the upper third of the epidermis, vacuolation of the epidermal keratinocytes, and a superficial perivascular lymphocytic infiltrate. Laboratory analysis found a fasting blood glucose level of 6.5 mmol/L (normal range, 3.6 to 6.1 mmol/L) and a serum glucagon level of 1024 ng/L (normal range, 49 to 114 ng/L). The diagnoses of glucagonoma syndrome and associated necrolytic migratory erythema (NME) were made. A contrastenhanced abdominal computed tomographic scan revealed an enhancing pancreatic tail mass measuring 6.5 cm in maximum diameter. The patient underwent a subtotal pancreatectomy and splenectomy. Pathologic analysis confirmed a pancreatic neuroendocrine carcinoma consistent with a diagnosis of glucagonoma. Two weeks after the surgery, the patient's cutaneous lesions had nearly re-3.

Necrolytic erythema migrans in celiac disease

“…Becker et al [1] were the first to describe NME as a characteristic skin reaction pattern seen in the presence of pancreatic islet cell carcinoma. More recently, Kelly et al described NME in the absence of glucagonoma as the pseudoglucagonomia syndrome [2][3][4].…”

“…The most frequently observed conditions related to NME apart from pancreatic tumor are celiac disease or malabsorption syndrome in addition to zinc, attyric acid, and biotin deficiencies [1,5,6], cirrhosis, malignancies, and pancreatitis [3]; less common conditions are hepatitis, inflammatory bowel disease, heroin abuse, and odontogenic abscess. In the case of a first flare of necrolytic erythema migrans, the search for an underlying glucagonoma is paramount.…”

Glucagonoma: From skin lesions to the neuroendocrine component (Review)

“…Lesions that are similar to NME have been reported in non-GS associated liver morbidities (for instance, cirrhosis), malnutrition and inflammatory bowel disease (50,51). The clinical aspects of these conditions involving NME, but not a glucagon producing tumour, have been called 'pseudoglucagonoma syndrome' (50,51). Some cases even reported a similar response to octreotide therapy (52).…”

Updates and Proposed Diagnostic Approach to Psoriasiform Dermatoses

“…Necrolytic migratory erythema manifests as scaly, erosive, annular erythematous plaques with occasional pustule and/or blister formation. Necrolytic migratory erythema can develop in the setting of a glucagon-secreting islet cell tumor of the pancreas (glucagonoma syndrome) or can be seen in other conditions including liver diseases, nutritional deficiency, chronic pancreatitis, and inflammatory bowel diseases 81…”