摘要

Three cases of glucagonoma syndrome were seen in 1 year. Study of the skin biopsies from the first two cases led to a correct diagnosis from skin biopsy of the third case, although it was not suggested clinically. In each case serum glucagon levels were high and a pancreatic tumor was found, with complete remission of symptoms in cases 1 and 3 after resection; case 2 refused surgery and has died. A total of nine skin biopsies from the three patients showed a variety of findings: epidermal necrosis; subcorneal pustules, either isolated or associated with necrosis of the epidermis; confluent parakeratosis, epidermal hyperplasia, and marked papillary dermal angioplasia; and suppurative folliculitis. The clinical lesions in this syndrome vary from bright red macules to annular superficial erosions and flaccid pustules. Similarly, several histopathologic features of the disease can occur, which may represent the progression of the disease. No single histologic feature was specific for the disease, but a combination of the features is probably diagnostic. Therefore, multiple skin biopsies are recommended when this diagnosis is suspected.

译文

1 年内出现三例胰高血糖素综合征。对前两例皮肤活检的研究导致了第三例皮肤活检的正确诊断,尽管临床上没有建议。在每一个病例中,血清胰高血糖素水平都很高,并且发现了胰腺肿瘤,病例 1 和病例 3 在切除后症状完全缓解; 病例 2 拒绝手术并死亡。这三个病人共九个皮肤活检显示了各种各样的发现: 表皮坏死; 角膜下脓疱,或孤立或与表皮坏死有关; 汇合角化旁,表皮增生, 和显著的乳头真皮血管增生; 和化脓性毛囊炎。该综合征的临床病变从鲜红色斑疹到环状表浅侵蚀和松弛脓疱不等。类似地,疾病的几个组织病理学特征可以发生,这可能代表疾病的进展。该疾病没有单一的组织学特征,但这些特征的结合可能是诊断的。因此,当怀疑该诊断时,建议进行多次皮肤活检。

Glucagonoma

内分泌 胰腺肿瘤 临床研究术语
概述  :  

胰高血糖素瘤是一种罕见的α细胞胰腺肿瘤,其导致生产过剩的激素胰高血糖素。α细胞肿瘤通常与胰高血糖素综合征有关,在不存在分泌胰高血糖素肿瘤的情况下,假胰高血糖素综合征患者也存在相似的症状。尽管原因尚不清楚,但遗传因素在某些情况下仍起作用。多发性内分泌肿瘤1型(MEN 1)的家族史是一个危险因素。这些肿瘤通常是癌性的,若癌症扩散会变得更严重。临床表现胰高血糖素瘤的主要生理作用是肽激素胰高血糖素的过量生产,其通过引起糖异生和脂解的合成代谢和分解代谢过程的激活而导致血糖水平升高。糖异生可从蛋白质和氨

Glucagonoma  

释    义   胰高血糖素瘤

例    句   Objective To study the clinical features, diagnosis, and treatment of glucagonoma syndrome (GS) for providing clues for the recognition of this disorder in clinical practice. 目的分析胰高血糖素瘤综合征(GS)的临床特点、诊断和治疗,以提高临床医师对本病的认识和诊治水平。

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