Glucagonoma syndrome with atypical necrolytic migratory erythema.

Necrolytic migratory erythema is most commonly associated with glucagonoma syndrome. We report a rare case of glucagonoma syndrome with necrolytic migratory erythema presenting as pruritic papules and follicular pustules in a 57-year-old woman; showing eosinophilic infiltration on histology. However, the final diagnosis was confirmed by demonstrating neuroendocrine tumour on histopathological examination of the liver metastases. Nutrition therapy was administered as a palliative treatment. This case also highlights the atypical clinical features and nonspecific histology of necrolytic migratory erythema which makes the diagnosis difficult.

Glucagonoma syndrome with serous oligocystic adenoma: A rare case report.

RATIONALE: Glucagonoma and pancreatic serous oligocystic adenoma (SOA) are rare neuroendocrine and exocrine tumors of the pancreas, respectively. The coexistence of glucagonoma syndrome (GS) and SOA is a rare clinical condition and has not yet been reported. Additionally, necrolytic migratory erythema (NME), a hallmark clinical sign of GS, is often misdiagnosed as other skin lesions by clinicians due to their lack of related knowledge, which delays diagnosis of GS and thus exacerbates the prognosis. PATIENT CONCERNS: A 50-year-old male patient was admitted to our department because he presented with diabetes mellitus and a recurrent ulcerated skin rash. Prior to the accurate diagnosis, the skin manifestation was considered to be diabetic dermopathy. DIAGNOSES: The patient's fasting serum glucagon level was up to 871.5 pg/mL. A biopsy of the pancreatic tumor revealed a pancreatic neuroendocrine tumor, and immunoperoxidase staining revealed glucagon-positive cells. In addition, the histological examination of the pancreatic cystic lesions showed typical features of SOA. INTERVENTIONS: The patient received a pancreaticoduodenal resection and radiofrequency ablation for the hepatic nodular lesion. OUTCOMES: One week after surgery, the glucagon concentration decreased to near normal levels. The cutaneous lesions spontaneously resolved within 4 weeks after surgery. LESSONS: Because almost all glucagonomas are malignant or have malignant potential, their early recognition and correct diagnosis are very important for a better prognosis, especially in cases with NME as the only manifestation during the development of glucagonomas. It is therefore imperative that clinicians recognize NME early to make an accurate diagnosis.

Skin diseases in consequence of endocrine alterations.

The skin is a target organ of several hormones. Specific diseases appear in consequence of hypo- or hypersecretion of endocrine organs, particularly in the elderly patient. There, knowledge of skin alterations is important not only for dermatologists, but also for endocrinologists and other physicians, because a clinical diagnosis of the underlying disease is often possible. In this review, a number of representative skin diseases having an endocrinological basis are described. These include acanthosis nigricans, diseases due to alterations of androgen metabolism, carcinoid syndrome, diseases due to alterations of corticosteroid metabolism, diseases in association with diabetes mellitus, diseases due to alterations of estrogen metabolism, genetic syndromes including dermatological and endocrine symptoms, the glucagonoma syndrome, diseases due to dysfunctions of growth hormone secretion, diseases in association with Merkel cells of the skin, diseases due to dysfunctions of the thyroid gland, diseases to alteration of vitamin D metabolism, and vitiligo and disorders of pigmentation.

Timing and extent of surgery in symptomatic and asymptomatic neuroendocrine tumors of the pancreas in MEN 1.

Pancreaticoduodenal tumors develop in a majority of patients with multiple endocrine neoplasia type 1 (MEN 1) and have a pronounced effect on life expectancy as the principal cause of disease related death. Previous discussion of therapy has focused mainly on syndromes of hormone excess and especially the management of MEN 1 associated Zollinger-Ellison syndrome (ZES). The syndromes of hormone excess, however, may be late features of the endocrinopathy and, when developed, indicate presence of metastases in more than one-third of patients. Recent possibilities for genetic diagnosis have emphasized requirements of prophylactic operation for prevention of malignant development. We recommend screening with biochemical markers and endoscopic ultrasound for early detection, and strong efforts of operative tumor removal before metastases have occurred. Surgery is generally recommended in patients with or without hormonal syndromes in the absence of spread hepatic metastases. Operative procedures include enucleation of tumors in the head of the pancreas, excision of duodenal gastrinomas together with clearance of lymph gland metastases, and as prophylaxis against tumor recurrence combination with distal 80% subtotal pancreatic resection. More extensive surgical tumor reduction is believed to reduce the risks for malignant progression of the pancreaticoduodenal tumors, but this requires further evaluation in MEN 1.

The glucagonoma syndrome: a review of its features and discussion of new perspectives.

Glucagonoma syndrome is a paraneoplastic phenomenon characterized by an islet alpha-cell pancreatic tumor, necrolytic migratory erythema, diabetes mellitus, weight loss, anemia, stomatitis, thromboembolism, and gastrointestinal and neuropsychiatric disturbances. These clinical findings in association with hyperglucagonemia and demonstrable pancreatic tumor establish the diagnosis. Glucagon itself is responsible for most of the observed signs and symptoms, and its induction of hypoaminoacidemia is thought to lead to necrolytic migratory erythema. Liver disease and fatty acid and zinc deficiency states may also contribute to the pathogenesis of the eruption in some cases. Most patients are diagnosed too late in the clinical course for cure, but successful palliation of symptomatology can usually be achieved with surgical and medical intervention. This paper reviews the glucagonoma syndrome, paying particular attention to its cutaneous features, and provides new perspectives in our current understanding of this phenomenon.

Dermatologic correlates of selected metabolic events.

Various metabolic events may lead to dermatologic pathology. Three illustrative examples are glucagonoma syndrome, uremic pruritus, and zinc deficiency. The glucagonoma syndrome, resulting from a glucagon secreting-tumor, is characterized by a distinctive dermatitis, necrolytic migratory erythema. This skin rash is a generalized, pruritic eruption which first appears as erythematous patches, then progresses to form superficial vesicles and bullae. Uremic pruritus is a clinical phenomenon seen in patients with chronic renal failure; it provokes vigorous scratching and may lead to numerous cutaneous lesions including extensive excoriations, lichen simplex chronicus, prurigo nodularis, keratotic papules, or secondary impetigo. Zinc deficiency may be evident as an inherited disease called acrodermatitis enteropathica, which presents clinically with a predominately acral and periorificial rash of sharply demarcated erythematous, exfoliative, and exudative patches. It may also result from an acquired defect with similar clinical findings. A brief review concerning the etiology, clinical manifestations, diagnosis, and treatment are given for glucagonoma syndrome, uremic pruritus, and zinc deficiency. Any pathophysiologic dysfunction that results in a loss of metabolic control of homeostasis in the body may demonstrate cutaneous manifestations. These skin findings may be of great clinical significance and may aid in the diagnosis or even be the first sign of a disease process. The glucagonoma syndrome, uremic pruritus, and zinc deficiency are three examples of dermatologic correlates of metabolic events.