Case 9 -

Necrolytic Migratory Erythema Michael Morgan University of South Florida College of Medicine Tampa, FL

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Clinical History A 67 year old man with abdominal pain and recent onset of diabetes mellitus presented with a rash involving the face, intertriginous areas and genitals. Case 9 - Figure 1 - There is psoriasiform hyperplasia of the epidermis with a pattern of abrupt epidermal keratinization, pallor and necrolysis. Case 9 - Figure 2 - There is psoriasiform hyperplasia of the epidermis with a pattern of abrupt epidermal keratinization, pallor and necrolysis. Histology epidermal acanthosis with conspicuous parakeratosis and epidermal pallor abrupt keratinization with loss of granular epithelial layer , 'necrolysis' near normal dermis Paraneoplastic cutaneous syndrome consisting of migratory rash, glucose intolerance and stomatitis associated with glucagon-secreting visceral tumor. Common to see normochromic normocytic anemia, migratory thrombophlebitis (Trosseau's syndrome), glossitis and weight loss Pathogenesis involves elaboration of glucagons with consequent metabolic effects including amino acid deficiency. Usually associated with malignant islet cell tumors of the pancreas (80% of cases). Also seen with small intestinal islet cell tumors, pancreatic insufficiency, cirrhosis, and villous atrophy of the small intestine. Dermatologic findings include an evolving rash consisting of peripherally expanding circular and serpiginous scaling erythema of the trunk, proximal extremities and intertriginous areas. Laboratory findings include normochromic normocytic anemia, hyperglycemia, amino acid deficiency and spectacular elevation in serum glucagon. Differential diagnosis includes acrodermatitis enterohepatica (zinc or micronutrient deficiency), see with infants and malabsorptive syndromes hartnup disease (impaired renal and gut absorption of tryptophan and other neutral amino acids) pellagra (niacin deficiency), the 4-D's References Mallison CN, Bloom SR, Warin AP, et al. A glucagonoma syndrome. Lancet 1974; 2: 1-5. Hashizume T, Kiryu H, Noda K, et al. Glucagonoma syndrome. J Am Acad Dermatol 188; 19: 377-43. Goodenberger DM, Lawley TJ, Strober W, et al. Necrolytic migratory erythema without glucagonoma. Report of two cases. Arch Dermatol 1979; 115: 1429-32.Gonzalez J. The histopathology of acrodermatitis enteropathica. Am J Dermatopathol 1982; 4: 303-11. Moynahan E. Acrodermatitis enteropathica: a lethal inherited human zinc deficiency disorder. Lancet 1974; 2: 399-400. Mock D. Skin manifestations of biotin deficiency. Semin Dermatol 1991; 10: 296-302. Hendricks W. Pellagra and pellagra-like dermatoses: etiology, differential diagnosis, dermatopathology and treatment. Semin Dermatol 1991; 10: 282-92.