Cases reported "Acne Vulgaris"

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1/19. Acne fulminans in late-onset congenital adrenal hyperplasia.

    Acne may be the only clinical sign of androgen excess in men. We report a boy with acne fulminans and androgen excess due to late-onset congenital adrenal hyperplasia.
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2/19. isotretinoin therapy for antibiotic-refractory acne in Apert's syndrome.

    Apert's syndrome is a congenital disorder characterized by the premature fusion of the cranial sutures and early epiphyseal closure. Severe pustular acne is a common feature. We report a case that failed to respond to conventional antibiotic treatment but resolved following isotretinoin therapy. The patient's permanent tracheostomy proved a management challenge during isotretinoin treatment. The role of androgen-sensitivity is discussed in relation to the skin and bones.
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keywords = androgen
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3/19. Pronounced and early acne in Apert's syndrome: a case successfully treated with oral isotretinoin.

    An unusual case of widespread acne unresponsive to treatment with early onset in a child with Apert's syndrome is presented. The patient eventually responded to oral isotretinoin therapy. The morphological profile of the sebaceous glands and the expression of proliferative markers and androgen receptors were evaluated in seboblasts and sebocytes using morphological, ultrastructural and immunohistochemical techniques. There were no significant differences in staining for proliferative markers and nuclear expression of androgen receptors in the glands from the patient and four healthy controls. Our results support the view that acne in Apert's syndrome is not sustained by abnormalities of the sebaceous glands demonstrable with conventional morphological techniques, and that it does not depend on an increased expression of androgen receptors.
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ranking = 1.5
keywords = androgen
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4/19. Three cases of androgen-dependent disease associated with myotonic dystrophy.

    Three cases of androgen-dependent disease in females with myotonic dystrophy are described. serum androgens in individuals affected by myotonic dystrophy are known to be lower on average than in normal controls. Despite this these three females developed diseases that are androgen dependent, including acne, hidradenitis suppurativa, androgenetic alopecia and keratosis pilaris. These cases support the hypothesis that the peripheral response to androgens rather than absolute circulating levels of androgens is important in androgen-dependent conditions.
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ranking = 5.5
keywords = androgen
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5/19. Congenital adrenal hyperplasia and acne in male patients.

    Seborrhoea is one pathogenic factor for acne. androgens induce sebum production, and excess androgen may provoke or aggravate acne. In women an androgen disorder is frequently suspected when acne is accompanied by hirsutism or menstrual irregularities. In men acne may be the only symptom of androgen excess. We report three male acne patients in whom hormonal screening revealed irregularities of androgen metabolism suggestive of late-onset congenital adrenal hyperplasia and who benefitted from low-dose glucocorticoids. Disorders of androgen metabolism may influence acne not only in women, but also in men, and these patients may benefit from low-dose glucocorticoid therapy.
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ranking = 2.5
keywords = androgen
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6/19. Cortisol receptor resistance: the variability of its clinical presentation and response to treatment.

    Primary (partial) cortisol receptor resistance was previously reported in a total of 7 patients and 14 asymptomatic family members. Its occurrence is considered to be extremely rare. In the present study we report on 6 patients (2 males and 4 females) with the syndrome. The first male patient presented with mild hypertension. hydrochlorothiazide therapy resulted in life-threatening hypokalemia. The second male patient had slight hypertension without hypokalemia. All four female patients presented between the age of 20-30 yr with acne, hirsutism, and irregular menstruations. Low dose dexamethasone therapy (1-1.5 mg/day) was of clinical benefit in these patients. All patients showed insufficient suppression of serum cortisol concentrations in the overnight 1-mg dexamethasone test. The diurnal rhythm of ACTH and cortisol was intact, albeit at an elevated level. There was a normal increase in ACTH, cortisol, and GH (except in one obese patient) in response to insulin-induced hypoglycemia, while cortisol production was elevated in three patients. Circulating adrenal androgen levels were increased in all patients. Glucocorticoid receptors were investigated in a whole cell dexamethasone binding assay in mononuclear leukocytes. In the first male patient, the number of receptors was very low, while the affinity was lower than that in controls. A lowered affinity to dexamethasone was found in one female patient, while a lowered number of receptors was found in three patients. In the second male patient, no abnormalities were found. As a bioassay for glucocorticoid action we also measured dexamethasone suppressibility of mitogen-stimulated incorporation of [3H]thymidine in mononuclear leukocytes. In the male patient with normal receptor status, dexamethasone suppressibility of [3H]thymidine incorporation was significantly lower than that in healthy controls with respect to both maximal suppression and IC50. Partial cortisol receptor resistance might be less rare than previously thought. In the six patients presented, at least three different forms can be recognized. Therapy with dexamethasone was successful in female patients with acne and hirsutism, as the secondary increase in the production of adrenal androgens was effectively controlled.
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7/19. Adrenal adenoma and normal androgen levels in a young woman with polycystic ovaries: a case of idiopathic hirsutism?

    A case of unusual combination of polycystic ovaries (PCO), adrenal non-functioning adenoma and severe hirsutism in a young woman from Southern italy is reported here. A 18-yr-old woman was referred to our Department because of oligomenhorrea, acne and severe hirsutism. During evaluation of the cause of her symptoms, PCO and small left adrenal adenoma were revealed. Although adrenal androgen excess has been shown to dysregulate the hypothalamic-pituitary-gonadal axis, causing PCO-like syndrome, normal circulating androgen values were found. androgens and cortisol levels were completely suppressed by low-dose dexamethasone test, excluding autonomous steroid secretion by the adrenal mass. Normal response of cortisol and adrenal androgens to corticotropin stimulation test permitted the exclusion of functional adrenal hyperandrogenism. Despite normal LH/FSH ratio, anovulatory cycles were revealed by persistently low progesterone values. Glucose and insulin response to oral glucose tolerance test did not differ from those of normal population. The patient showed an improvement of acne and hirsutism on therapy with estro-progestins (EP). In conclusion, despite normal pattern of serum androgens and LH/FSH ratio, this patient had anovulatory cycles and good response to the EP treatment. These findings suggest that ultrasonography evidence of PCO together with anovulatory cycles contributed to her clinical picture whereas adrenal adenoma seemed to have no relevant role. This case report underlines the need of cautious interpretation of imaging results and clinical signs of severe hirsutism, reminding one that the true cause of a medical problem may not be the most evident.
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ranking = 4.5
keywords = androgen
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8/19. Cortisol and estradiol secretion by a benign virilizing adrenocortical tumor in a prepubertal girl.

    We report a 5.5 year-old girl with a benign adrenocortical adenoma who presented with virilization and rapid growth. She did not have any clinical features of isosexual precocity or, except for hypertension, Cushing's syndrome. Measurement of hormones in adrenal vein blood obtained at surgery showed high concentrations of testosterone, cortisol, estradiol and intermediary substrates. Elevated levels of hormones detected in the peripheral blood were released directly from the tumor and were not the result of peripheral interconversion. hyperandrogenism can obscure the clinical features of Cushing's syndrome and estrogen hypersecretion in patients with functional adrenal tumors.
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keywords = androgen
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9/19. Polycystic ovary (Stein-Leventhal) syndrome: etiology, complications, and treatment.

    polycystic ovary syndrome (PCOS) occurs in approximately 3% to 5% of the female population and may be the leading cause of infertility in those of reproductive age. PCOS presents clinically with a variety of signs and symptoms; the most common being menstrual irregularities, hyperandrogenism, infertility, and obesity. The true pathophysiology has not been clearly elucidated; however, there is growing agreement that gonadotropin dynamic dysfunction, hyperandrogenism, and insulin resistance are key features. The diagnosing of PCOS involves radiologic and laboratory studies. Radiologic studies typically include pelvic ultrasound; laboratory data should be obtained regarding pertinent gonadotropins and other hormone levels. PCOS is not a benign condition. It may lead to complications involving glucose metabolism, dyslipidemias, cardiovascular disease, and cancer. The goals of treatment should focus on restoring menstrual regularity, decreasing androgen excesses, and decreasing insulin resistance.
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ranking = 1.5
keywords = androgen
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10/19. bulimia nervosa and acne may be related: a case report.

    Acne is a very common, often cosmetically disfiguring, cutaneous condition of adolescence that is associated with increased sebaceous gland activity. We present the case of a patient with bulimia who reported that the negative effect of acne on her appearance increased her body image concerns and exacerbated her eating disorder. Improvement of the acne was associated with a significant improvement in her eating disorder. Eating disordered patients may go on restrictive diets in order to control their acne since levels of androgens, which are one of the primary stimulants of sebaceous gland activity, are lower in starvation. As a significant number of adolescents with eating disorders also develop acne, it is important for the clinician to be aware of this previously unreported association between acne and eating disorders, and to evaluate the impact of acne upon the patient's body image and eating behaviour.
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keywords = androgen
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