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Generalized acanthosis nigricans in childhood.

Acanthosis nigricans in children is usually associated with obesity. Generalized acanthosis nigricans in childhood is rare. We report a 13-year-old boy with an 8-year history of generalized hyperpigmentation and velvety thickening of the skin. Despite an extensive physical and laboratory examination no evidence of underlying endocrinological or neoplastic disease was found.

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Steroid-induced acanthosis nigricans in dermatomyositis.

Acanthosis nigricans of the groin, axillae, and neck developed in a patient on long-term steroid therapy for dermatomyositis after eight years. The dermatosis resolved spontaneously ten years after the steroid use had been discontinued. Review of three personal cases and the literature suggests that acanthosis nigricans may have a hormonal basis.

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Acanthosis nigricans, hypothyroidism, and insulin resistance.

Acanthosis nigricans has been associated with multiple endocrinopathies. The common denominator appears to be insulin resistance. Three patients are described (one woman and two men) who presented with hypothyroidism and acanthosis nigricans. All the patients had elevated insulin levels in the fasting state and in response to an oral glucose load. The mechanism for the resistance to insulin was a post-receptor binding defect or a structural abnormality in circulating insulin, since glucose tolerance and plasma cortisol, somatomedin, growth hormone, and insulin receptor concentrations in peripheral monocytes were all normal. Antibodies to insulin or the insulin receptor were absent. Neither insulin resistance nor acanthosis nigricans diminished following correction of the hypothyroidism. It is concluded that hypothyroidism may be associated with acanthosis nigricans, that hyperinsulinemia is a regular correlate of the skin disorder, and that treatment of the hypothyroidism does not resolve the hyperinsulinemia or the acanthosis nigricans.

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Acanthosis nigricans and insulin resistance.

Acanthosis nigricans has been reported in association with many conditions and most recently with insulin resistance. A case is presented to illustrate the clinical characteristics of acanthosis nigricans and insulin resistance. The various subtypes of this syndrome complex and its pathogenic mechanisms are reviewed.

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Familial acanthosis nigricans.

A mother and daughter had benign familial acanthosis nigricans. Familial acanthosis nigricans begins in early childhood and may be accentuated at puberty. The eruption is not associated with underlying illness. Forms of acanthosis nigricans are associated with obesity, endocrinologic abnormalities, drug ingestion, and malignant neoplasms.

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[Inhibition of pancreatic beta-cell secretion during a "glucose-clamp" in subjects with acanthosis nigricans].

It has long been known that acanthosis nigricans is accompanied by insulin resistance. In certain insulin-resistant states, including obesity, a more sluggish response to the insulin/insulin inhibition feedback normally present in pancreatic beta cells has been documented. Some have claimed a sort of beta-cell insulin resistance "parallel" to that of the peripheral tissues. The present study assesses the efficiency of the insulin/insulin feedback in acanthosis nigricans patients, measuring the inhibition of the production of C-peptide (the indicator of beta cell secretion) induced by the administration of exogenous insulin during glucose clamping. This was done in order to compare the roles of the peripheral tissues and the beta cells in producing the insulin resistance typical of acanthosis nigricans. The study using the glucose-insulin clamp technique was conducted on 4 Acanthosis Nigricans patients with normal glucose tolerance and 4 healthy controls, the drop in C-peptide levels after the administration of exogenous insulin being assessed in the course of both steady states. The results showed that the acanthosis nigricans patients retained a beta cell response to the exogenous insulin through their peripheral tissues presented a reduced sensitivity to insulin as revealed by the glucose-insulin clamp. It therefore seems reasonable to attribute the endocrine metabolic alteration found in Acanthosis Nigricans to a peripheral receptor and/or post receptor alteration rather than central alterations in the beta cells that have yet to be demonstrated. It is concluded that in acanthosis nigricans the peripheral insulin resistance is primarily independent phenomenon and not "parallel" to insulin/insulin feedback.

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Acanthosis nigricans in Kabuki syndrome.

BACKGROUND: Acanthosis nigricans has been classified in different ways. All classifications depend on the clinical picture and the association with other conditions. OBJECTIVE: We report a case of acanthosis nigricans in a patient with Kabuki syndrome. This syndrome is characterized by five main manifestations: typical facial features, post natal growth deficiency, skeletal abnormalities, dermatoglyphic abnormalities, and mild to moderate mental retardation. CONCLUSION: Our case may suggest that acanthosis nigricans could be associated with Kabuki syndrome.

Abnormalities, Multiple↗

[Acral acanthosis nigricans paraneoplastic: about one case].

The paraneoplastic acanthosis nigricans occurs in association with visceral neoplasms. We report a case of acanthosis nigricans that seat at a dorsal face of the second, third and fourth right toe of a 63 year-old patient. The exploration has been put in evidence a carcinoma of the right lung. The acanthosis nigricans has been nearly disappeared after resection of the pulmonary lobe. The relapses of the acanthosis nigricans in a same sit after five years has been reveal a carcinoma of the bladder. The resection of the bladder tumour has been followed by disappearance of the acanthosis nigricans.

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Acanthosis nigricans and a rectal carcinoid.

Malignant acanthosis nigricans is often related to adenocarcinomas of the gastrointestinal tract, but it has also been found to coexist with tumors of the amine precursor uptake and decarboxylation system. It can precede, accompany, or follow the appearance of the underlying tumor. Evaluation of our patient's new-onset acanthosis nigricans led to the discovery of an asymptomatic rectal carcinoid tumor. To our knowledge, this is the first report of acanthosis nigricans associated with a carcinoid tumor of the rectum. It emphasizes the importance of considering underlying malignancy in the evaluation of patients with acanthosis nigricans.

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Ovarian pathology associated with insulin resistance and acanthosis nigricans.

Insulin resistance and the skin lesions of acanthosis nigricans are not commonly seen by the gynecologist, but the ovarian pathology that can be associated with insulin resistance and acanthosis nigricans is well known. The clinical course of disease in a patient with virilization-amenorrhea associated with insulin resistance and acanthosis nigricans is presented to illustrate the association. Hyperthecosis was the ovarian pathology demonstrated; testosterone levels were in excess of 400 ng/gl. Postoperative testosterone levels were normal at 62 ng/dl. Additional ovarian pathology reported in association with insulin resistance and acanthosis nigricans includes polycystic ovary disease alone, in association with stromal luteomas, or with bilateral dermoid cysts. Furthermore, masculinizing ovarian neoplasms such as hilar cell tumors have been reported in association with ovarian hyperthecosis. When evaluating patients with androgen excess, it would be worthwhile to keep in mind the association with abnormal carbohydrate metabolism and acanthosis nigricans.

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Clinical, biochemical, and ovarian morphologic features in women with acanthosis nigricans and masculinization.

Nine women with acanthosis nigricans and masculinization, who did not appear to have any of the reported syndromes associated with acanthosis nigricans, were studied to characterize the clinical, biochemical, and ovarian morphologic features of their disorders. These patients had the clinical and biochemical profiles of polycystic ovarian disease. All acanthosis nigricans subjects had significant insulin resistance when insulin binding to both circulating monocytes and erythrocytes was compared to the control subjects. Microscopic examination of the ovaries revealed no evidence of recent normal ovulation, sclerosis of the ovarian cortex, follicle cysts, and stromal hyperthecosis. The authors conclude that ovarian stromal hyperthecosis and insulin resistance are consistent findings in the present type of patient. This study provides further evidence supporting a relationship between insulin resistance and human ovarian function.

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[Acanthosis nigricans in pregnancy].

A case of Acanthosis nigricans developed in a pregnant 16 year old woman is reported. Laboratory studies disclosed an abnormal glucose tolerance and elevated levels of insulinemia with an insulin-resistant state. Two months after partum, the metabolic parameters became normal and parallel the complete resolution of acanthosis nigricans was observed. The possible pathogenetic role of pregnancy in the development of acanthosis nigricans is discussed.

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Malignant acanthosis nigricans with florid papillary oral lesions.

Acanthosis nigricans is a distinctive skin disease of importance, because it has served as an external marker for a variety of systemic disorders including endocrinopathies, and malignant tumors of internal organs. It typically appears as hyperpigmented, roughened plaques of velvety consistency and infrequently as verruca-like papillations. The oral cavity and lips can be affected by florid papillary growths. Because of its rarity and nonspecific microscopic appearance, clinical recognition of acanthosis nigricans continues to be a challenge. A case of mucocutaneous "malignant" acanthosis nigricans is presented in which pigmented skin lesions led to the discovery of a gastric adenocarcinoma, which in turn was followed by the appearance of massive oral papillomatosis. No effective treatment was found.

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The autosomal dominant trait of obesity, acanthosis nigricans, hypertension, ischemic heart disease and diabetes type 2.

OBJECTIVE: Among obese subjects, acanthosis nigricans in both males and females is not as uncommon as previously thought. Whereas this finding was extensively evaluated in females, mostly in the context of polycystic ovaries syndrome, little attention has been paid to obese males with acanthosis nigricans. As acanthosis seems to be a marker for insulin resistance, the present study was designed to evaluate the hypothesis that the clinical syndrome of obesity and acanthosis would take a different clinical course than that of simple obesity. METHODS: To characterize the course of acanthosis nigricans and obesity in males, we examined 22 children and adolescents with this complex, together with their parents and grandparents and found them to follow a detrimental sequence of the metabolic syndrome. We compared the findings to 13 age-matched males with obesity but no clinical apparent acanthosis nigricans. We analyzed the clinical course, fat distribution, glucose, insulin and C-peptide and lipoproteins. RESULTS: Onset of obesity in the metabolic syndrome group was at a mean age of 6.4 years, as compared to 2.3 years in the controls. The metabolic syndrome patients had a truncal (android) distribution of fat and their fasting blood glucose was significantly higher. HDL/total cholesterol was lower. Examination of the pedigrees suggested autosomal dominant inheritance of the obesity and acanthosis nigricans complex, extending to hypertension and ischemic heart disease in the parents' generation, and further extending to include diabetes type 2 in the grandparents' generation. CONCLUSIONS: This metabolic syndrome is inherited as an autosomal dominant trait, with onset of truncal obesity at age 6-7 years, acanthosis nigricans during childhood or adolescence, extending to hypertension and ischemic heart disease during young adulthood, and further extending to include diabetes type 2 in late adulthood. It is recommended that such children should be followed up as an 'at-risk' group, and would probably benefit from intensive weight reduction, which may prevent the later manifestations of the syndrome.

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Prevalence and significance of acanthosis nigricans in an adult obese population.

BACKGROUND AND DESIGN: Acanthosis nigricans develops commonly in obese individuals, yet its prevalence and significance in an unselected adult obese population has not been determined. To address these issues, 34 patients enrolled in the Adult Obesity Clinic at Parkland Memorial Hospital (Dallas, Tex) were chosen at random and examined. RESULTS: Acanthosis nigricans was observed in fully 74% of patients; its prevalence correlated positively with severity of obesity. Black obese patients demonstrated a greater propensity for manifesting the cutaneous disorder than did white obese individuals. Patients with acanthosis nigricans exhibited fasting plasma insulin levels that were markedly higher than those of nonacanthotic cohorts. CONCLUSIONS: Obesity is a significant risk factor for the development of acanthosis nigricans. Conversely, acanthosis nigricans is a reliable cutaneous marker of hyperinsulinemia in obese individuals.

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Acanthosis nigricans with endometrial carcinoma: case report and review of the literature.

BACKGROUND: Acanthosis nigricans is classified into benign and malignant forms on the basis of clinical associations. The main interest in acanthosis nigricans has been based on its association with malignancy because of the dramatic clinical appearance of the skin lesions and the usually rapidly fatal nature of the underlying disease. "Tripe palms" is a descriptive term of acanthosis nigricans of the palms. It more often is associated with internal malignancy. Most importantly, it often precedes the diagnosis of a new or recurrent tumor. Malignant acanthosis nigricans is most commonly associated with intra-abdominal malignancies. There are very few reports in the literature of malignant acanthosis nigricans associated with gynecological malignancies. Only five cases of endometrial carcinoma associated with acanthosis nigricans and/or tripe palms have been reported in the literature. CASE: A 69-year-old African-American female presented with skin changes consistent with the diagnosis of acanthosis nigricans and tripe palms. More than 14 months later she was found to have endometrial carcinoma. She subsequently underwent total abdominal hysterectomy and salpingo-oophorectomy followed by chemotherapy with paclitaxel and carboplatin. During treatment of the underlying malignancy the skin condition dramatically improved. CONCLUSION: Tripe palms can be associated with endometrial carcinoma and may be the first sign of malignancy. Malignant acanthosis nigricans may improve with treatment of the underlying malignancy. Patients who present with tripe palms may need to undergo workup to search for underlying malignancy.

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Malignant acanthosis nigricans: potential role of chemotherapy.

Acanthosis nigricans is an uncommon skin condition characterized by hyperkeratosis and skin hyperpigmentation. Most causes are benign, but it may also be associated with gastrointestinal and other malignancies. When associated with malignant disease, the skin pathology may be more severe and treatment often unsuccessful. We describe a 66-year-old man with acanthosis nigricans associated with carcinoma of the stomach, with distressing generalized cutaneous, perioral and perineal disease, whose skin condition resolved completely with combination chemotherapy. In patients with malignant acanthosis, chemotherapy may relieve many of the distressing cutaneous symptoms. A close liaison between gastroenterologists, dermatologists and oncologists is required.

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[Clinical significance of pancreatic beta-cell function in obese children with acanthosis nigricans].

OBJECTIVE: The strong relation between type 2 diabetes mellitus and obesity with acanthosis nigricans is widely concerned. This study investigated the pancreatic beta-cell function in obese children with acanthosis nigricans, so as to find out the role of insulin secretion and insulin resistance in obese children with acanthosis nigricans. METHODS: Thirty-five obese children with acanthosis nigricans (19 males and 16 females with mean age 12.8 +/- 1.5 years) were enrolled in this study. Thirty-eight obese children (21 boys and 17 girls with mean age 11.9 +/- 2.6 years) and 39 normal children (20 boys and 19 girls with mean age 11.2 +/- 2.2 years) were recruited as obese and normal control groups. The levels of serum fasting insulin, C-peptide, proinsulin and true insulin were measured in all the subjects. The ratios of proinsulin/insulin and proinsulin/C-peptide were calculated. Homeostasis model assessment was applied to assess the status of insulin resistance and basic function of pancreatic beta-cell. RESULTS: The levels of fasting insulin, C-peptide proinsulin, true insulin, the ratios of proinsulin/insulin and proinsulin/C-peptide, insulin resistance index and insulin secretion index of obese children with acanthosis nigricans, obese control children and normal control children were: 18.5 (5.0-60.5) pmol/L, 12.4 (6.1-35.8) pmol/L and 5.1 (2.0-32.8) pmol/L; 3.9 (1.3-14.0) microg/L, 2.4 (1.1-4.0) microg/L and 1.1 (1.0-4.2) microg/L; 28.8 (9.9-64.2) pmol/L, 9.5 (2.2-34.5) pmol/L and 4.2 (2.0-16.0) pmol/L; 33.0 (6.2-66.0) pmol/L, 10.6 (4.8-29.4) pmol/L and 4.5 (1.3-30.1) pmol/L; 1.2 (0.4-8.9), 0.9 (0.2-1.9) and 0.8 (0.4-2.0); 6.9 (2.5-36.6), 4.7 (1.2-12.3) and 3.6 (1.2-9.6); 5.0 (0.8-14.1), 2.6 (1.3-8.1) and 1.2(0.4-6.9); 303.3 (52.2-1,163.8), 213.6 (84.6-572.0) and 51.1 (19.1-561.4). The levels of fasting insulin, C-peptide, proinsulin, true insulin, the ratios of proinsulin/insulin and proinsulin/C-peptide, insulin resistance index and insulin secretion index in obese children with acanthosis nigricans were significantly higher than those in obese children (P < 0.001) and normal children (P < 0.001). CONCLUSION: Obese children with acanthosis nigricans had higher insulin resistance and pancreatic beta-cell dysfunction; acanthosis nigricans may be a skin sign of high risk of type 2 diabetes mellitus.

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