营养缺乏所致坏死松解性游走性红斑样皮损

报告1例不伴高胰岛素瘤综合征的坏死松解性游走性红斑样皮损表现的营养缺乏性疾病。患者女，62岁。双小腿红斑反复发作3年，躯干与四肢红斑、结痂、脱屑近1年，皮疹呈周期性发作和缓解，伴有舌炎，呈营养不良状态。组织病理示表皮浅层坏死，棘层增厚，真皮乳头水肿，血管扩张，真皮浅层中等量中性粒细胞、淋巴细胞为主浸润；胰高血糖素238.56 ng/L，胸腹部及盆腔CT检查未见实体肿瘤。诊断为营养缺乏所致坏死松解性游走性红斑样皮损。给予补充复方氨基酸、维生素等对症支持治疗，皮损10 d内缓解。随访6个月，未见大面积反复。     

坏死性松解性游走性红斑1例

患者女,69岁。全身反复起红斑水疱伴痒半年,加重半月。患者伴有消瘦,低血钾,贫血,低蛋白,舌炎,尿糖血糖升高。皮损组织病理示:表皮上部坏死。诊断:坏死性松解性游走性红斑。     

胰腺性脂膜炎1例

患者男，67岁。双侧小腿反复出现红斑、结节3个月，伴疼痛1个月。血淀粉酶5078．2U／L，尿淀粉酶47217U／L。腹部CT检查示慢性胰腺炎。皮肤组织病理检查示皮下脂肪坏死，诊断为胰腺性脂膜炎。     

反应性穿通性胶原病1例

患者女,21岁。下肢红斑、丘疹伴瘙痒1个月,无系统疾病。皮肤科检查：双下肢红斑、丘疹．中心有脐凹,内含角质栓。皮损组织病理示：表皮缺如,下方为坏死胶原纤维穿通表皮。诊断：反应性穿通性胶原病。     

反应性穿通性胶原病1例

患者女,21岁。下肢红斑、丘疹伴瘙痒1个月,无系统疾病。皮肤科检查:双下肢红斑、丘疹,中心有脐凹,内含角质栓。皮损组织病理示:表皮缺如,下方为坏死胶原纤维穿通表皮。诊断:反应性穿通性胶原病。     

胰高血糖素瘤综合征伴坏死松解性游走性红斑1例

患者男,60岁。全身反复红斑、水疱、糜烂伴痒3年,加重伴双下肢肿胀7d。皮损组织病理示:表皮角化过度并角化不全,部分表皮萎缩变薄,真皮小血管及附属器周围可见少量淋巴细胞浸润。诊断:胰高血糖素瘤综合征伴坏死松解性游走性红斑。     

家族性慢性良性天疱疮1例

报告1例家族性慢性良性天疱疮。患者女，36岁。因双侧颈部、腹部、腹股沟及肘窝、胭窝反复出现红斑水疱，易擦破结痂，伴瘙痒20余年就诊。腹部皮损组织病理检查：角化过度，角层下见中性粒细胞聚集，基底层上可见水疱，水疱内可见棘刺松解细胞，并见倒塌的城墙样结构，真皮内可见中等量淋巴细胞浸润。     

胰高血糖素瘤综合征

报告1例胰高血糖素瘤综合征。患者女，45岁。双下肢反复皮疹4年6个月，发现胰腺占位性病变1个月就诊。入院查血胰高血糖素明显升高，皮损表现为坏死松解性游走性红斑。胰腺肿瘤手术切除，组织病理断为胰体尾高分化神经内分泌癌，胰周淋巴结转移性神经内分泌癌，免疫组化胰高血糖素（Clu）（ ）。手术后给予化疗，皮疹明显好转。     

胰高血糖素瘤综合征1例

患者男 ,48岁。躯干下部及四肢反复发生红斑伴瘙痒 1年 ,加重 2月。伴体重减轻、舌炎、低蛋白血症、血糖升高、尿糖阳性 ,血清胰高血糖素升高。皮肤病理为表皮浅层坏死。CT扫描示胰体部肿块。静脉滴注复方氨基酸对皮损有效。     

胰高血糖素瘤综合征1例

报道1例46岁男性病人，全岙皮肤红斑、水疱3年，加重1年，具有典型的坏死松解性游走性红斑，伴舌炎、糖尿病，体重下降，血胰高血糖素增高。皮肤组织病理变化为表皮浅层坏死，免疫组化发现表皮浅层抗人胰高血糖素抗体着色阳性。腹部CT及锝标记生长抑素类似物（Octreotide）扫描显示患者胰尾及肝脏肿瘤。长效生长抑素治疗后，血胰高血糖素水平在短期内明显下降，皮诊逐渐好转。     

Necrolytic migratory erythema without glucagonoma versus canine superficial necrolytic dermatitis: is hepatic impairment a clue to pathogenesis?

The case of a 57-year-old diabetic man with necrolytic migratory erythema in the absence of glucagonoma is reported. The clinical and pathologic features of his dermatitis and subsequent clinical course are compared with those of canine superficial necrolytic dermatitis, an unusual cutaneous necrotizing eruption of dogs that is identical histologically to necrolytic migratory erythema. In addition to a necrolytic dermatitis, both our patient and most dogs with superficial necrolytic dermatitis have diabetes mellitus and hepatic failure in the absence of glucagonoma. Thus hyperglucagonemia most likely is not a factor in the origin of the necrotizing dermatitis in this patient or in dogs. The role of hepatocellular dysfunction in the pathogenesis of necrolytic migratory erythema and superficial necrolytic dermatitis is considered.     

Erythema necroticans migrans bei Glucagonomsyndrom

The glucagonoma syndrome is a rare disease in which a typical skin lesion, necrolytic migratory erythema, is often one of the presenting symptoms. A 68-year-old woman developed erythematous polycyclic migratory lesions with advancing scaling borders and crusts over several years. Skin biopsies, laboratory studies and imaging confirmed the diagnosis of necrolytic migratory erythema as part of a glucagonoma syndrome.     

Necrolytic migratory erythema with myelodysplastic syndrome without glucagonoma

Necrolytic migratory erythema is a cutaneous paraneoplastic manifestation, which is usually associated with a glucagon-secreting pancreatic tumor. However, it also may occur in other circumstances in which serum glucagon is elevated, as in hepatic cirrhosis. Rarely, necrolytic migratory erythema is reported in association with a jejunal and rectal adenocarcinoma or villous atrophy of the small intestine without any evidence for increased serum glucagon levels. In this context we report the case of an 85-year-old male with myelodysplastic syndrome who developed typical necrolytic migratory erythema without glucagonoma syndrome or evidence for other pancreatic or liver disease. We suggest that, in addition to the diseases listed, myelodysplastic syndrome might be able to cause necrolytic migratory erythema.     

Necrolytic migratory erythema associated with an islet cell tumour of the pancreas

A man of 59 is described with a characteristic skin eruption, necrolytic migratory erythema, associated with a malignant islet cell pancreatic tumour. There was no definite evidence that this was a glucagonoma although previous reports have suggested that the pancreatic tumour usually associated with necrolytic migratory erythema is always a glucagonoma. The relevant literature is reviewed.     

Pseudoglucagonoma syndrome: Description of an ‘Idiopatic’ case

A case of pseudoglucagonoma syndrome, that is necrolytic migratory erythema, in a patient with no coexistent glucagonoma, is described. The patient was a 59-year-old man with waxing and waning dermatitis of the buttocks, characterised by arciform erythematous papulo-squamous lesions with micro-pustulation. Histopathology was characteristic for necrolytic migratory erythema, but no other underlying disease was detected. Other cases of pseudoglucagonoma syndrome described in literature are briefly reviewed.     

Necrolytic migratory erythema: unresolved problems in diagnosis and pathogenesis. A case report and literature review.

Necrolytic migratory erythema is a distinctive cutaneous eruption that occurs in patients with malignant glucagon-secreting tumors of the pancreas. Recognition of this erosive dermatitis as a cutaneous manifestation of an internal malignancy can result in tumor detection and surgical removal prior to metastasis. The case history of a forty-year-old diabetic woman with necrolytic migratory erythema associated with a metastatic glucagon-secreting islet cell tumor of the pancreas is presented. Prior to diagnosis, she had been treated with topical steroids and Mycostatin powder for a recurrent perioral, acral, and intertriginous dermatitis. Because of apparent responsiveness to these medications, the diagnosis of necrolytic migratory erythema was not considered, and the diagnosis of pancreatic carcinoma was delayed for over one year. Wider recognition of the distinctive clinical and histopathologic features of necrolytic migratory erythema should result in earlier detection and possible surgical cure of the associated glucagonoma.     

Paediatric necrolytic migratory erythema as a presenting sign of glucagonoma syndrome

Glucagonoma syndrome is an extremely rare pancreatic neuroendocrine tumour often associated with necrolytic migratory erythema. While glucagonomas are neoplasms of adulthood, we report the first case in a paediatric patient. We present the case of a 15‐year‐old girl with a 4‐year history of a rash, consistent with necrolytic migratory erythema, found to have a localized glucagonoma. Immediately following resection of the tumour, there was complete resolution of her rash and systemic symptoms. Detection of the cutaneous rash of necrolytic migratory erythema can aid in the early diagnosis of a glucagonoma, as well as the prevention of metastatic disease. To our knowledge, this is the first reported paediatric patient with glucagonoma syndrome.     

The glucagonoma syndrome

We report on our experience with two patients with glucagonoma syndrome and review the recent dermatologic literature. The clinical features are described with special emphasis on necrolytic migratory erythema, the characteristic cutaneous sign of glucagonoma syndrome. Using our histological and ultrastructural findings, we discuss the pathogenesis of necrolytic migratory erythema. Pathognomonic laboratory data and the diagnostic procedures recommended for the evaluation of patients with glucagonoma syndrome are presented. Finally, we discuss the differential diagnosis and describe therapeutic possibilities in the management of this syndrome.     

The necrolytic erythemas: a continuous spectrum?

The necrolytic erythemas is a group of disorders with similar histologic and clinical features. The objective of this case report is to present a patient with features of both necrolytic migratory erythema (NME) and necrolytic acral erythema (NAE). These 2 entities appear more likely to be on a spectrum caused by the same underlying process of abnormal liver function and glucagon metabolism.     

Plattenepithelkarzinomassoziiertes nekrolytisches migratorisches Erythem

A 59-year-old Caucasian man suffered from persistent disseminated necrolytic skin lesions for 8 months. They failed to respond to a variety of therapeutic regimens but then cleared spontaneously within a few days. After one week an invasive squamous cell carcinoma of the hypopharynx became clinically apparent. At autopsy, the expected glucagonoma often associated with necrolytic migratory erythema was not found.