胰高血糖素瘤综合征坏死性迁移性红斑的鉴别

R. Vasanwala, Joseph R. Malhis, Vivek Malhotra, Ethan Anderson, Kerry J Williams-Wuch
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摘要

由胰高血糖素分泌的胰高血糖素引起称为胰高血糖素综合征的症状组合。1胰高血糖素瘤综合征的一个共同特征是坏死性迁移性红斑(NME),发生在约70%至80%的患者中。虽然这种皮疹可以在少数其他疾病中发现,但NME应该引起胰高血糖素综合征的警报,特别是当出现全身症状时。我们报告一位51岁男性患者,既往有2型糖尿病病史,因下肢和腹股沟处出现进行性疼痛瘙痒性丘疹而就诊于皮肤科。先前的穿孔活检未发现坏死性迁移性红斑的特征性表现,皮疹被认为是脓疱性牛皮癣。仅仅过了一年多,由于病人病情继续恶化,才进行了恶性肿瘤检查。最终证实胰高血糖素瘤综合征。坏死性迁移性红斑可以伪装成几种不同类型的皮疹。异常或无反应的牛皮癣样皮疹应提示调查替代诊断。
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Identifying necrolytic migratory erythema in glucagonoma syndrome 
The glucagon secreted by a glucagonoma causes the combination of symptoms known as glucagonoma syndrome. 1 A common presenting feature of glucagonoma syndrome is necrolytic migratory erythema (NME) occurring in about 70 to 80 percent of patients. 2,3 Although this rash is can be found in a handful of other disorders, NME should raise an alarm for glucagonoma syndrome especially if present with systemic symptoms. We present a 51 year old male with a past medical history of type 2 diabetes who presented to oncology after seeing several dermatologists regarding a progressive painful pruritic papular rash along his lower extremities and groin. Previous punch biopsies did not reveal the characteristic findings of necrolytic migratory erythema and the rash was thought to be pustular psoriasis. It was only after over a year as the patient continued to worsen that a malignancy work up was done. Eventually glucagonoma syndrome was confirmed. Necrolytic migratory erythema can disguise itself as several different types of rashes. An unusual or unresponsive psoriasiform rash should prompt an investigation for an alternative diagnosis.
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