Cases reported "Acanthosis Nigricans"

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1/23. Inhibition of growth hormone excess reduces insulin resistance and ovarian dysfunction in a lean case of polycystic ovary syndrome with a growth-hormone-producing pituitary adenoma.

    A 23-year-old female with polycystic ovary syndrome (PCOS) and a growth-hormone (GH)-producing pituitary adenoma is described. A reduction in the elevated GH levels to normal levels following the administration of dopaminergic agents decreased plasma insulin-like growth factor (IGF)-1 and ovarian dysfunction. Menstrual cycles were therefore restored and the number of ovarian cysts reduced, suggesting that insulin and/or IGF-1, stimulators of theca cell proliferation, may be pathogenetic factors in PCOS.
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ranking = 1
keywords = polycystic ovary syndrome, polycystic ovary, ovary syndrome, polycystic, ovary
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2/23. Is this patient insulin resistant? How much does it matter?

    Alex was an obese 10-year-old girl with a family history of type 2 diabetes, hypertension, and perhaps polycystic ovarian syndrome. Her physical examination was significant for a central accumulation of body fat and acanthosis nigricans. Although the laboratory studies indicated that Alex was not diabetic and probably not glucose intolerant, she could be insulin resistant (IR). Should any further evaluation be done? If Alex is IR, what kind of treatment should be offered? The following discussion addresses these questions by reviewing the pathophysiology, diagnosis, and consequences of isolated IR.
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ranking = 0.0015864516837688
keywords = polycystic
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3/23. Clinical course of genetic diseases of the insulin receptor (type A and Rabson-Mendenhall syndromes): a 30-year prospective.

    The interaction of insulin with its cell surface receptor is the first step in insulin action and the first identified target of insulin resistance. The insulin resistance in several syndromic forms of extreme insulin resistance has been shown to be caused by mutations in the receptor gene. We studied 8 female patients with the type A form of extreme insulin resistance and 3 patients (2 male and 1 female) with the Rabson-Mendenhall syndrome and followed the natural history of these patients for up to 30 years. The 11 patients ranged in age from 7 to 32 years at presentation. All 11 patients had extreme insulin resistance, acanthosis nigricans, and hyperandrogenism in the female patients, and all but 1 were of normal body weight. This phenotype strongly predicts mutations in the insulin receptor: of the 8 patients studied, 7 were found to have mutations. Similar results from the literature are found in other patients with type A and Rabson-Mendenhall syndromes and leprechaunism. The hyperandrogenic state resulting from hyperinsulinemia and insulin resistance in these patients was extreme: 6 of 8 patients had ovarian surgery to correct the polycystic ovarian syndrome and elevation of serum testosterone. By contrast, a larger group of insulin-resistant patients who were obese with hyperandrogenism, insulin resistance, and acanthosis nigricans (hair-AN syndrome) did not have a high probability of mutations in the insulin receptor. The morbidity and mortality of these patients were high: 3 of 11 died, 9 of 11 were diabetic and 1 had impaired glucose tolerance, and 7 of 9 patients had 1 or more severe complication of diabetes. Our literature review revealed that the mortality of leprechaunism is so high that the term leprechaunism should be restricted to infants or young children under 2 years of age. Analogous to patients with the common forms of type 2 diabetes, these patients had a heterogeneous course. In 2 patients who were able to maintain extremely high endogenous insulin production, the fasting blood glucose remained normal even though post-glucose-challenge levels were elevated. Most patients, however, required large doses of exogenous insulin to ameliorate the severe hyperglycemia. Preliminary results of a recent study suggest that recombinant leptin administration may benefit these patients with severe insulin resistance.
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ranking = 0.0015864516837688
keywords = polycystic
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4/23. Malignant acanthosis nigricans, tripe palms and the sign of Leser-Tre'lat, a hint to the diagnosis of early stage ovarian cancer: a case report and review of the literature.

    BACKGROUND: Malignant acanthosis nigricans is a mucocutaneous eruption associated with internal malignancies. Tripe palms refers to a characteristic velvety thickening of the palms, with exaggeration of normal skin markings. The sign of Leser-Tre'lat is the presence of multiple seborrheic keratoses and scattered skin tags caused by a malignancy. We are reporting the first case of ovarian cancer in association with three coexisting cutaneous paraneoplastic dermatosis. CASE: A 52-year-old female presented with skin manifestation consistent with MAN, TP and the sign of Leser-Tre'lat. Patient underwent extensive work up and was discovered to have stage I, high grade adenocarcinoma of the ovary. skin manifestations gradually recovered after initiation of chemotherapy. CONCLUSION: The presence of acanthosis nigricans in conjunction with tripe palms and the sign of Leser-Tre'lat are highly suggestive of an internal malignancy and necessitate an extensive investigation in order to discover the underlying malignancy.
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ranking = 0.00010578607091041
keywords = ovary
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5/23. A syndrome of hyperandrogenism, insulin resistance, and acanthosis nigricans associated with polycystic ovary syndrome: clinical and laboratory features.

    We describe an adolescent Japanese girl with acanthosis nigricans and irregular anovulatory menstruation following menarche. serum LH levels were elevated, whereas serum FSH levels were within normal range. An exaggerated response to LHRH was observed. Further, serum androstenedione levels were markedly elevated. Ultrasonogram revealed bilateral polycystic changes of ovaries. She had a mild degree of insulin resistance. Insulin binding studies using erythrocytes demonstrated a decreased binding capacity of insulin. From the above findings, this patient presents the syndrome consisting of hyperandrogenism, insulin resistance, and acanthosis nigricans and also has clinical and biochemical features compatible with polycystic ovary syndrome.
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ranking = 1.0015864516838
keywords = polycystic ovary syndrome, polycystic ovary, ovary syndrome, polycystic, ovary
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6/23. acanthosis nigricans in obese women with the polycystic ovary syndrome: disease spectrum not distinct entity.

    fasting and stimulated insulin concentrations in four patients with acanthosis nigricans and polycystic ovary syndrome were compared with four patients, matched for weight and ovarian morphology, without acanthosis. The median fasting serum insulin concentrations were 114.2 and 25.1 mU/l in the respective groups (P = 0.02). One additional patient was investigated before and after an 18% increase in weight which resulted in a 170% increase in fasting insulin concentrations and the development of acanthosis nigricans. These observations suggest that there is variation between individuals in the degree of obesity that results in the development of acanthosis and that obese patients with this skin condition represent the severe end of the spectrum of the polycystic ovary syndrome.
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ranking = 1.2
keywords = polycystic ovary syndrome, polycystic ovary, ovary syndrome, polycystic, ovary
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7/23. Familial insulin resistance and acanthosis nigricans. Presence of a postbinding defect.

    Type A insulin resistance, associated with acanthosis nigricans and menstrual irregularity, has been ascribed to a decreased concentration of insulin receptors. We now report four affected females from one family, a mother and three daughters (including identical twins) who appear to have the type A syndrome. Two of the kindred had no apparent ovarian dysfunction, while the other two had hyperprolactinemia without other findings of polycystic ovary disease, suggesting a genetic disease with variable penetrance. All had normal erythrocyte and monocyte insulin binding. Insulin dose-response studies to assess glucose metabolism and insulin sensitivity were performed in the affected twins. The dose response to insulin was shifted to the right with a decrease in maximal response. These results are consistent with a postbinding defect in insulin action in these patients.
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ranking = 0.068645544325288
keywords = polycystic ovary, polycystic, ovary
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8/23. Prolonged suppression of hirsutism with combination therapy in an adolescent with insulin resistance and acanthosis nigricans.

    spironolactone and oral contraceptives have been used separately with some success in the treatment of hirsutism. hirsutism associated with the Kahn type-A syndrome of insulin resistance, acanthosis nigricans, and polycystic ovaries may be severe and refractory to conventional treatment. In view of their different sites and mechanisms of action, spironolactone and an oral contraceptive were used in combination to treat severe hirsutism in a 13-year-old girl with features of this syndrome. The two therapeutic agents, apparently acting together, resulted in a marked reduction in facial hair and serum androgen concentrations, which persisted during 18 months of treatment. testosterone and androstenedione levels fell from 100 ng/dl and 628 ng/dl, respectively, to 47 ng/dl and 230 ng/dl by six months and to 35 ng/dl and 156 ng/dl by 18 months. This result suggests that combination therapy may be an effective treatment for severe hirsutism.
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ranking = 0.0015864516837688
keywords = polycystic
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9/23. Remission of acanthosis nigricans, hypertrichosis, and Hashimoto's thyroiditis with thyroxine replacement.

    hypothyroidism is not commonly associated with acanthosis nigricans (AN). We examined a 13-year-old girl with AN, hypertrichosis, and Hashimoto's thyroiditis. Overt biochemical hypothyroidism, thyroid enlargement, and positive titers of antimicrosomal and antithyroglobulin antibodies confirmed Hashimoto's thyroiditis. Both AN and hypertrichosis resolved with thyroid hormone replacement. There was no evidence of insulin resistance, polycystic ovarian disease, lipoatrophy, or other endocrine dysfunction, or of malignancy. In two patients from the literature with AN and hypothyroidism, AN was attributed to associated thyroid carcinoma or insulin resistance, rather than coexisting hypothyroidism. Since the skin lesions improved with thyroid hormone therapy in those two patients and in ours, hypothyroidism appears to be directly involved in the pathogenesis of AN.
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ranking = 0.0015864516837688
keywords = polycystic
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10/23. insulin resistance, acanthosis nigricans, and polycystic ovaries associated with a circulating inhibitor of postbinding insulin action.

    A 21-yr-old moderately obese woman with hirsutism, acanthosis nigricans, and oligomenorrhoea was diagnosed as having polycystic ovary syndrome. Despite hyperinsulinemia, binding of insulin to her red cells was within the range for normal, young adult subjects. Her serum did not bind or degrade [125I]insulin or alter its binding to fat cells, and was negative for insulin receptor antibodies. However, her serum caused a dose-dependent inhibition of insulin-stimulated lipogenesis (conversion of [3-3H]glucose to [3H]lipid) in rat fat cells significantly greater than that produced with control serum (relative potency, 3.5:1) and (at a 1:20 dilution) markedly impaired the response of both lipogenesis and 2-deoxy-D-glucose uptake to maximum concentrations of insulin. After the patient was treated with clomiphene for 4 months, her menses resumed, hair growth slowed, fasting blood glucose and plasma insulin concentrations decreased, and serum inhibitory activity decreased to the control range. serum inhibitory activity was stable to freezing and thawing and to heating at 56 C for 30 min, and could be extracted into acid-ethanol. By dialysis, its mol wt was below 1000, whereas by ultracentrifugation, it was above 3500; both high and low mol wt forms were detected after Sephadex G-50 gel chromatography of serum, suggesting that the inhibitor was of low mol wt but loosely bound to a higher mol wt component in serum. These findings indicate that insulin resistance in this patient with acanthosis nigricans and polycystic ovaries could be attributed to a circulating low mol wt inhibitor of postbinding insulin action.
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ranking = 0.20793225841884
keywords = polycystic ovary syndrome, polycystic ovary, ovary syndrome, polycystic, ovary
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