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The Glucagonoma SyndromeA Distinctive Cutaneous Marker of Systemic Disease
Keith H. Swenson, MD;
Robert B. Amon, MD;
Jon M. Hanifin, MD
Arch Dermatol. 1978;114(2):224-228.
Abstract
• The glucagonoma syndrome is a rare clinical condition characterized by a distinctive cutaneous eruption associated with a glucagon-secreting islet cell neoplasm of the pancreas. A 19-year-old woman manifested typical features of this condition: a polymorphous skin eruption with characteristic distribution of lesions in perioral and paragenital regions; lesions in sites of cutaneous trauma; a skin biopsy that showed epidermal cleavage; glossitis; weight loss; mild anemia; abnormal glucose tolerance test results. Plasma glucagon levels, determined by radioimmunoassay, were approximately five times normal. Angiography indicated a pancreatic tumor with liver metastases. Islet cell origin was confirmed histologically.
It is hoped that wider recognition of the distinctive clinical features of this syndrome will result in earlier detection and possible surgical cure of the underlying malignancy.
(Arch Dermatol 114:224-228, 1978)
Author Affiliations
From the Department of Dermatology, University of Oregon Health Sciences Center, Portland.
Footnotes
Accepted for publication March 10, 1977.
Reprint requests to Department of Dermatology, University of Oregon Health Sciences Center, 3181 SW Sam Jackson Park Rd, Portland, OR 97201 (Dr Hanifin).
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