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Virilization

Masculinization

Virilization, as a disease, occurs when androgen-induced male characters appear at the wrong time of development, or in the wrong sex, i.e. girls and women. In most cases, this is due to overproduction of androgens because of adrenal hyperplasia. But virilization may indicate other, serious problems, i.e. tumors in the ovaries or adrenal glands.


Presentation

Hirsutism, (unusually thick, male-pattern growth of hair), accompanied by no other symptoms, or much milder ones than those occurring in virilization is often associated with the polycystic ovarian syndrome (PCOS).

Most cases of virilization [1] are due to congenital adrenal hyperplasia (CAH). The presentation of adrenal virilism depends on the sex of the afflicted individual and the stage of life when the disease is manifested. A baby girl born with the disease (the more severe, classical form [2]) displays various degrees of masculinization in the external genitalia (pseudohermaphroditism), sometimes to the extent that her sex is erroneously assigned as male [3]. In the less severe, non-classical form the symptoms may appear some years after birth. Affected girls born with normal external genitalia will show some characteristics of virilization, whereas boys will show early puberty.

The presence of excessive androgenic hormones – such as testosterone - is characteristic of this syndrome, which is as expected for the virilizing effects seen [1]. These hormones speed up growth; stimulate the maturation of bone structures; enlarge the clitoris in girls and the penis in boys, and cause hirsutism and acne. Further symptoms include deeper voice, balding of the head and reddening of the skin, developing stronger muscles, the cessation of periods, and increased sexual drive in women.

Some newborn babies present with a version of the disease in which there is a deficiency in maintaining normal salt and water concentrations [3] [4]. In these patients mineralocorticoid and salt supplementation are necessary; in the past many of these infants have died [2] [4].

Turkish
  • PATIENTS: The index patient from a consanguineous Turkish family showed complete external virilization and was diagnosed incidentally at the age of 19 months during hospital admission for severe combined bacterial (urosepsis) and viral (CMV and EBV) infection[ncbi.nlm.nih.gov]
Deepening of Voice
  • . • Hirsuitism • Acne • Deepening of voice • Increased muscle mass • Breast atrophy 3.[slideshare.net]
  • […] of voice -Differential dx Androgen dep tumor, CAH, Exogenous androgens, severe hyperinsulinemia Easier to recognize Female w/ CAH as abnormal than boy w/ precocious puberty as abnormal -The Prader Scale of Genital Masculinization -No to 5 Androgens in[quizlet.com]
  • Amenorrhea and then the gradual onset of virilization followed including increased truncal hair and deepening of voice 2 years prior to this presentation. There was no acne or baldness. She was not taking any kinds of medication.[link.springer.com]
  • Here we report a case of virilizing Leydig cell type, steroid cell tumor of the left ovary, in a 40 year old female who presented with clinical signs and symptoms of virilization: deepening of voice, hirsutism (Ferriman-Gallwey score 26), clitoromegaly[casesjournal.biomedcentral.com]
Increased Muscle Mass
  • . • Hirsuitism • Acne • Deepening of voice • Increased muscle mass • Breast atrophy 3.[slideshare.net]
  • -Ferriman-Gallwey Scoring System -Look at hair at body and giving it a score - 8 No Hirsutism - 8 25 Hirsutism - 25 Severe Hirsutism -More extreme response to excessive androgens -Ex. frontal balding, severe acne, clitoremegaly, increased muscle mass,[quizlet.com]
  • Signs of virilization might include: Deepening voice Balding Acne Decreased breast size Increased muscle mass Enlargement of the clitoris Causes At puberty, a girl's ovaries begin to produce a mix of female and male sex hormones, causing hair to grow[mayoclinic.org]
  • Other causes of virilization include: the use of male hormone supplements the use of steroids to increase muscle mass polycystic ovary syndrome (PCOS), which is a condition in which women of childbearing age have multiple cysts in their ovaries Cases[healthline.com]
Hirsutism
  • OBJECTIVE: To describe a woman with postmenopausal virilization and hirsutism caused by hilus-cell hyperplasia.[ncbi.nlm.nih.gov]
  • Abstract All patients with virilization signs, increased levels of androgen hormones and rapidly progressive hirsutism should be evaluated for an androgen-producing tumor.[ncbi.nlm.nih.gov]
  • Abstract A 58-year-old postmenopausal woman with high plasma testosterone levels and virilization, as demonstrated by hirsutism and alopecia, is presented.[ncbi.nlm.nih.gov]
  • Following treatment the patient experienced regression of the hirsutism and the plasma testosterone dropped to a normal level.[ncbi.nlm.nih.gov]
  • PATIENT AND METHODS: A 68-yr-old postmenopausal woman was referred because of a history of progressive development of hirsutism and frontal balding for the previous 8 yr, together with moderate hyperandrogenemia.[ncbi.nlm.nih.gov]
Hypertrichosis
  • . • DD: Hypertrichosis, which is generalised excessive growth of vellus hair. • The aetiology is androgen excess 4.[slideshare.net]
  • Glucocorticoids cause hypertrichosis by promoting the passage from the telogen to the anagen phase of the hair follicle cycle ( Karpas 1987 ).[erc.bioscientifica.com]
Acne Vulgaris
  • vulgaris – Cushing's syndrome • When recent & with virilisation, suggestive of a rare androgen-secreting tumour 17.[slideshare.net]
Increased Libido
  • Upon questioning, she reported two years of worsening facial hirsutism, voice deepening, clitoromegaly, and increased libido. Laboratory testing revealed normal FSH, LH, DHEA-S, IGF-1, and 8 a.m. cortisol.[endocrine.org]
  • She reported frequent mechanical removal of facial hair, increased libido and a 14-kg weight gain and denied acne or voice change. Before this she had had an unremarkable reproductive history. She weighed 96 kg and was normotensive.[academic.oup.com]
  • This is followed by hirsutism, acne, clitoral enlargement, increased libido, sterility, enlargement of the larynx, deepening of the voice, and temporal alopecia [ 9 , 10 ].[casesjournal.biomedcentral.com]
  • Starting in September, 1998, the patient developed hirsutism , defluvium capillorum, deepening of the voice, increased libido and aggressiveness.[erc.bioscientifica.com]
Amenorrhea
  • We describe a 14-year-old patient with 1-year secondary amenorrhea, who had an ovarian mature teratoma as a cause of her clinical history.[ncbi.nlm.nih.gov]
  • Abstract A Chinese adolescent girl presented with secondary amenorrhea. During follow-up, she gradually developed Cushingoid features and virilization.[ncbi.nlm.nih.gov]
  • BACKGROUND: A 33-year-old woman presented to an endocrinology clinic with a 5-year history of secondary amenorrhea. 2 years before presentation, she had noticed progressively worsening signs of virilization.[ncbi.nlm.nih.gov]
  • The patient was admitted because of secondary amenorrhea, hirsutism and acne.[ncbi.nlm.nih.gov]
  • Various virilizing symptoms such as hirsutism, temporal balding, and amenorrhea are common in these patients; however massive ascites is an infrequent symptom.[ncbi.nlm.nih.gov]
Clitoromegaly
  • PATIENT(S): An 11-and-a-half-year-old girl with 45,X karyotype showed signs of accelerated growth and clitoromegaly and was found to have elevated serum T.[ncbi.nlm.nih.gov]
  • We report a typical case that we found at cesarean section to be associated with a virilized infant who manifested clitoromegaly and labial fusion. We detected an increased level of testosterone in the maternal patient.[ncbi.nlm.nih.gov]
  • RESULTS: A 60-year-old postmenopausal woman presented with extensive hirsutism, male-pattern hair loss, and clitoromegaly.[ncbi.nlm.nih.gov]
  • CASE: An 18-year-old woman was admitted to our clinic due to primary amenorrhea. the On examination she had significant facial hirsutism, clitoromegaly and and Adam's apple. The serum level of testosterone was elevated.[ncbi.nlm.nih.gov]
  • RESULTS: Three cases referred for treatment of clitoromegaly were in fact cases of pseudoclitoromegaly caused by a hamartoma, a neurofibroma, and a hemangioma of the clitoral prepuce.[ncbi.nlm.nih.gov]
Secondary Amenorrhea
  • We describe a 14-year-old patient with 1-year secondary amenorrhea, who had an ovarian mature teratoma as a cause of her clinical history.[ncbi.nlm.nih.gov]
  • Abstract A Chinese adolescent girl presented with secondary amenorrhea. During follow-up, she gradually developed Cushingoid features and virilization.[ncbi.nlm.nih.gov]
  • BACKGROUND: A 33-year-old woman presented to an endocrinology clinic with a 5-year history of secondary amenorrhea. 2 years before presentation, she had noticed progressively worsening signs of virilization.[ncbi.nlm.nih.gov]
  • The patient was admitted because of secondary amenorrhea, hirsutism and acne.[ncbi.nlm.nih.gov]
Irregular Menstruation
  • This illness may have the following additional symptoms: (1) failure of the ovaries to produce, develop or release eggs (ova); (2) ovary enlargement; (3) failure to menstruate; (4) irregular menstruation ; (5) obesity ; and (6) infertility .[medicinenet.com]
Urinary Incontinence
  • Eight women reported dyspareunia; seven women reported urinary incontinence. The women's perceived sexual functioning was less satisfactory than in the reference group, and they reported more sexual distress.[ncbi.nlm.nih.gov]

Workup

A diagnosis of virilization needs to differentiate between adrenal and ovarian origins, as well as between hyperplastic states and tumors as sources of the disease. It should be remembered that mild symptoms, or just hirsutism coupled with scanty periods and elevated testosterone levels, may indicate the most frequent condition of androgen excess in women: the polycystic ovary syndrome. This is a complex disease, with a somewhat problematic diagnosis [5] [6]. The examination of adult women should include ultrasound tests, determination of hormone levels, and biopsy for evaluating histopathological results. The latter is especially important if the symptoms are atypical or develop unusually fast, indicating the presence of ovarian or adrenal tumors [7] [8] [9]. Exogenously added androgens also have to be considered in the diagnosis.

Finding increased concentrations of adrenal androgens [2] [10] in the urine verifies adrenal virilism. Results at the high end of the urinary 17-ketosteroid assay can indicate adrenal disease, of which the most common is due to a deficiency of the 21-hydroxylase enzyme [2] [4]. Conversely, when urinary ketosteroid levels are in the low to normal range, it suggests an ovarian source for the disease [1]. Urinary 17-ketosteroid elevation can also be used to differentiate men with the adrenogenital syndrome from patients with other forms of sexual precocity.

The following androgens are elevated in the urine in adrenal hyperplasia: dehydroepiandrosterone (DHEA), its sulfate (DHEAS), and frequently pregnanetriol. DHEA and DHEAS, together with 17-hydroxyprogesterone, testosterone, and androstenedione may also be in the high range in the plasma.

The suppression of symptoms with exogenous steroid can be used to make the distinction between hyperplasia and autonomous adrenal tumor. Thus, tumors as the source of virilization can be ruled out if dexamethasone suppresses the androgen levels [1]. If no suppression is observed, the adrenal glands and ovaries should be examined by computerized tomography (CT), magnetic resonance imaging (MRI) or ultrasonography, to find a possible tumor. Stimulation by the administration of synthetic adrenocorticotropic hormone (cosyntropin) is expected to raise the 17-hydroxyprogesterone level if adrenal hyperplasia is the source of virilism [9].

Treatment

  • Treatment is surgical, except for most infantile hemangiomas. Before deciding to offer surgical treatment, one should consider the degree of genital deformity and the risks of sensitivity loss and/or clitoral ischemia. Copyright 2016 Elsevier Inc.[ncbi.nlm.nih.gov]
  • BACKGROUND: Virilization in female newborns typically results from congenital adrenal hyperplasia, requiring immediate diagnosis and treatment.[ncbi.nlm.nih.gov]
  • Following treatment the patient experienced regression of the hirsutism and the plasma testosterone dropped to a normal level.[ncbi.nlm.nih.gov]
  • The treatment and prognosis of the disease are discussed.[ncbi.nlm.nih.gov]
  • Once we've approved your claim, you'll get the private treatment you need. 3 And we’ll pay for treatment After you've been treated, send us your invoices.[aviva.co.uk]

Prognosis

  • The treatment and prognosis of the disease are discussed.[ncbi.nlm.nih.gov]
  • Regarding the age of our patient and pathohistological findings of the tumor, her prognosis is favorable. Copyright 2001 S. Karger AG, Basel[ncbi.nlm.nih.gov]

Etiology

  • Etiology The disease is caused by a mutation in the CYP21A2 gene located on chromosome 6p21.3. As a result of this mutation, the synthesis of aldosterone is normal while the synthesis of cortisol is abolished.[orpha.net]
  • The etiology is usually of adrenal or ovarian origin.[casesjournal.biomedcentral.com]
  • Patients with aldosterone deficiency of any etiology may present with dehydration, hyponatremia, and hyperkalemia, especially with the stress of illness.[emedicine.medscape.com]
  • The patient we report here is of interest because of the dual etiology of virilization: iatrogenic and neoplastic. We also report for the first time that the VAT literally vanished after a short-term treatment with cyproterone acetate (CPA).[erc.bioscientifica.com]

Epidemiology

  • Summary Epidemiology The prevalence is approximately 1/40,000 as it accounts for about 25% of classic CAH cases. Clinical description Girls present at birth with ambiguous genitalia and variable levels of virilization.[orpha.net]
  • […] in which the ability to conserve sodium was intact. 108 , 110 , 111 , 113 , 115 , 116 , 117 , 118 , 119 , 120 , 121 , 122 , 123 It is claimed that 3 β -HSD deficiency is the second most common steroidogenic defect, 124 but as yet there have been no epidemiologic[glowm.com]
Sex distribution
Age distribution

Pathophysiology

  • In New MI, Levine LS (eds): Adrenal Diseases in Childhood: Pathophysiologic and Clinical Aspects, Vol. 13, pp 47–71.[glowm.com]

Prevention

  • It is likely that virilization of the patient could have been prevented by earlier oopherectomy prior to development of hormone production.[ncbi.nlm.nih.gov]
  • Precautions must be taken to prevent person-to-person transfer of topical steroids.[ncbi.nlm.nih.gov]
  • In spite of extensive explanation and psychological counseling, cultural barriers prevented appropriate treatment. An ovarian Leydig cell tumor should always be considered for a woman in the reproductive age with symptoms of virilization.[ncbi.nlm.nih.gov]
  • The virilization of newborn infants with the classic 21-hydroxylase form of congenital adrenal hyperplasia must be identified early if the associated metabolic crisis in the perinatal period is to be prevented.[ncbi.nlm.nih.gov]
  • AB - Context Prenatal treatment with dexamethasone to prevent virilization in pregnancies at risk for classical congenital adrenal hyperplasia (CAH) remains controversial.[mayoclinic.pure.elsevier.com]

References

Article

  1. Sherins RJ, Horton R. Hirsutism and virilization. Calif Med. 1967 Feb;106(2):87-91.
  2. Forest MG. Recent advances in the diagnosis and management of congenital adrenal hyperplasia due to 21-hydroxylase deficiency. Hum Reprod Update. 2004 Nov-Dec;10(6):469-85.
  3. Jaier JH, Louchart J, Cahill GF. Adrenal virilism. II. Metabolic studies. J Clin Invest. 1952 Oct;31(10):880-4.
  4. Wilkins L. Adrenal disorders II. Congenital virilizing adrenal hyperplasia. Arch Dis Child. 1962 Jun;37:231-41
  5. Hassa H, Tanir HM, Yildiz Z. Comparison of clinical and laboratory characteristics of cases with polycystic ovarian syndrome based on Rotterdam's criteria and women whose only clinical signs are oligo/anovulation or hirsutism. Arch Gynecol Obstet. 2006 Jul;274(4):227-32.
  6. Azziz R, Carmina E, Dewailly D, et al. The Androgen Excess and PCOS Society criteria for the polycystic ovary syndrome: the complete task force report. Fertil Steril. 2009 Feb;91(2):456-88.
  7. Juniarto AZ1, Setiawati BA, Ediati A, et al. Virilization due to androgen hypersecretion in a patient with ovarian leydig cell tumor: diagnostic and psychosocial implications. Acta Med Indones. 2013 Apr;45(2):130-5.
  8. Aminimoghaddam S, Ebrahimi AS, Hashemi F. A rare ovarian tumor, leydig stromal cell tumor, presenting with virilization: a case report. Med J Islam Repub Iran. 2012 Nov;26(4):185-8.
  9. Dennedy MC, Smith D, O'Shea D, McKenna TJ. Investigation of patients with atypical or severe hyperandrogenaemia including androgen-secreting ovarian teratoma. Eur J Endocrinol. 2010 Feb;162(2):213-20. doi: 10.1530/EJE-09-0576.
  10. Linder N, Davidovitch N, Kogan A, et al. Longitudinal measurements of 17alpha-hydroxyprogesterone in premature infants during the first three months of life. Arch Dis Child Fetal Neonatal Ed. 1999 Nov;81(3):F175-178.

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Last updated: 2017-08-09 17:33