Swenson K H, Amon R B, Hanifin J M
Arch Dermatol. 1978 Feb;114(2):224-8. doi: 10.1001/archderm.114.2.224.
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.
胰高血糖素瘤综合征是一种罕见的临床病症，其特征为独特的皮肤疹，伴有胰腺分泌胰高血糖素的胰岛细胞瘤。一名19岁女性表现出该病症的典型特征：多形性皮肤疹，病损在口周和生殖器旁区域有特征性分布；皮肤创伤部位出现病损；皮肤活检显示表皮松解；舌炎；体重减轻；轻度贫血；葡萄糖耐量试验结果异常。通过放射免疫测定法测定的血浆胰高血糖素水平约为正常水平的五倍。血管造影显示胰腺肿瘤伴肝转移。组织学证实为胰岛细胞起源。希望对该综合征独特临床特征的更广泛认识能导致更早发现并可能手术治愈潜在的恶性肿瘤。
