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HirsutismHirsutism is defined as a hairiness in women that is similar to the male distribution pattern (chin, upper lip, neck, back, chest, etc). The severity can range from very mild to very pronounced.

Introduction

As with many medical problems, there is a smooth transition between what is considered "normal" and what is not is considered and a hair pattern that may need more detailed clarification and perhaps also treatment. Whether a woman has normal, increased or pathologically pronounced body hair also depends on the affected person's own assessment. Thus it can happen that pathologically increased body hair of the male distribution pattern is not found disturbing by some women, while others already feel markedly impaired by only a small amount of additional hair. Increased body hair may occur along with other disorders, z.B. in combination with menstrual cycle disorders, oily skin, reduction of scalp hair. Several tables and charts are available to the clinician to determine the severity of the disorder.

There are many factors on which hair growth depends. In the case of the problems mentioned, it should be discussed with the doctor to what extent perceived complaints actually z.B. occur due to expected and tolerable side effects of medications or whether z.B. can be responsible for hormonal disorders.

Too much male hormones?

In the following, we will discuss reasons for increased hair growth on the body, which are based on a change in hormone metabolism. An excess of so-called male hormones (androgens with testosterone as the main representative) often leads not only to increased hair growth or a "lady's beard", but can also cause hair loss on the head, menstrual irregularities, or a so-called "androgen syndrome". "Impure skin" with acne. The term "male hormones is misleading in that women also produce testosterone, but usually in much smaller amounts than men.

Figure 1: The main sites of androgen production in women are the adrenal glands and, to a lesser extent, the ovaries.a. the adrenal glands and sometimes also the ovaries. They reach the respective cells of the body via the bloodstream (z.B. of the skin or hair root cells) and can mediate their effect there.

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The most important androgen-producing organs in women are the adrenal glands and, to a lesser extent, the ovaries, but also fatty tie. During pregnancy, the placenta ("placenta") and the developing child are also possible sources of hormones. To complicate matters, male hormones (androgens) are converted into female hormones (estrogens) and the male hormones exert their effect via a receptor (docking site on the cells) (Figures 1 and 2). Both functions can also be disturbed: a reduced conversion or/and degradation of the male hormones resp. increased activity of the male hormone receptor can contribute to hirsutism. A high proportion of the male hormones are bound to protein molecules, which facilitates their transport. If this binding is altered, the balance of free active and bound inactive hormones is affected. This may result in different activities being found for the same total amount of male sex hormones. This complicated system sometimes makes it very difficult to find out exactly where the disorder is located. For an effective therapy, however, it is crucial to find out the cause of hirsutism. This is aided by detailed discussions and examination of patients, blood tests, ultrasound examinations of the ovaries, hormonal function tests, and, if necessary, a number of other tests. also complementary genetic testing.

It is important for the blood analyses that the blood samples are taken in the morning and in a specific phase of the cycle (shortly after the onset of menstruation) and that the affected persons do not take any medications that can mask the disorders or that are not present in the blood. falsify" the blood values. These include glucocorticoids ( cortisone preparations ), including ointments and certain sprays for inhalation), the "Pill, but also anti-hormonal therapies, substances for muscle building or also certain drugs against fungal diseases or fungal infections. for HIV therapy.

"Non-classical AGS" (late onet AGS)

The term adrenogenital syndrome (AGS) summarizes a group of cortisol production disorders of the adrenal glands, which are associated with changes in the production of male and female hormones. female sex hormones. Figure 2 outlines cortisol production without (A) and with disruption (B). AGS is inherited by both parents at once to the affected child and can occur in different forms, a severe form or a mild form, the non-classical AGS (late-onset AGS). If the expression is very severe, then it can also lead to masculinization in female children – hence the name. The severe form is usually diagnosed in early childhood, while the mild variant often does not become symptomatic until puberty or young adulthood.

However, this situation can also typically be detected by hormonal stimulation tests and laboratory analysis and by genetic testing.

The most common is the so-called. "21-Hydroxylase Defect". Depending on the extent of the disorder, in addition to hirsutism, there may also be loss of scalp hair, acne or cycle irregularities, up to and including an unfulfilled desire to have children. Occasionally, blood prere is slightly lower than in other people, with dizziness or even blackness before the eyes. If the disturbance goes beyond that, also the breast growth can be impaired.

Figure 2A: The production of cortisol is normal.

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Figure 2B: The formation of cortisol is hindered by a sluggish enzyme. The precursors therefore take a different synthetic pathway: androgens.

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Figure 2C: The production of cortisol is normal, the production of androgens is also normal, but the androgen receptor is "hyperactive".

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As there is a possibility of inheritance to one's own children in case of proven genetic defect, genetic counseling is sometimes necessary to successfully implement family and therapy planning.

"PCO syndrome (PCOS)" – Polycystic ovary syndrome

In PCO syndrome, male hormones are produced in the ovaries. How the synthesis disorder comes about is not yet completely clear. One hypothesis is that problems with the menstrual cycle lead to a sustained stimulation of hormone synthesis in the ovaries and thus to increased production of male sex hormones. Cycle disturbances and remodeling processes in the ovaries with cyst formation are therefore in the foreground and suggest this disease. However, there are different variants of PCO syndrome. One of the variants is associated with a tendency to sugar metabolism disorders. In many cases it is therefore tested whether the sugar value in the blood is increased or even a diabetes mellitus is present. In such metabolic diseases, the binding behavior of hormones may be altered.

Tumors

There is a rare possibility that tumors of the adrenal glands or ovaries lead to increased production of male hormones.

Therapy

Therapy depends on the cause of the disorder. In non-classical AGS (late-onset) the production of androgens in the adrenal gland can be slowed down by replacing the missing hormone cortisol. Alternatively, drugs that affect the action or transport of male hormones can be used. In the case of rare adrenal tumors or tumors on the ovaries, the source of the androgens is removed by surgical therapy. If the ovaries are the source of the androgens, in the presence of polycystic ovary syndrome (PCOS), the addition of estrogens (z.B. In the form of a "pill") the hormone production in the ovaries can be slowed down, in combination with a progestin, which blocks the testosterone effect at the receptor. In post-menopausal women, ovarian overactivity can also be slowed by other hormones. Individual therapies are often sought depending on lifestyle, symptoms, or the presence of concomitant diseases. Because of possible side effects, glucocorticoids ("cortisone preparations") are often used in low-dose therapy and sometimes only for a temporary period of time, e.g.B. to fulfill a desire to have children. Often, however, it is also possible – in the case of mild adrenal disorders – to carry out hormone therapy with the pill, because the male hormones are increasingly bound by the pill and their effect is weakened. If there is a problem in sugar metabolism, diabetes therapy is often used (z.B. with metformin). Furthermore, these therapies are often combined, and there is also the possibility of blocking the androgen receptor. Overall, the therapeutic options are very diverse, especially if a desire for children is not yet being pursued. Unfortunately, due to the complexity of the metabolism of androgens, not every case can be solved, and it is not always possible to meet the patient's wishes. It is also the case that various forms of therapy for some disorders have only been tested on a small scale, which means that the approved use of the drugs is often limited and must be discussed with patients as an exception. Therefore, the treatment belongs in the hands of a competent doctor or a nurse. a competent doctor, who will plan an individual examination and therapy.

Authors: Prof. Dr. Felix Beuschlein, Munich Prof. Dr. Stefanie Hahner, Wurzburg Dr. nat. Mirko Peitzsch, Dresden Prof. Dr. Nada Rayes, Berlin Prof. Dr. Holger Willenberg, Rostock PD Dr. Nicole Reisch, Munich Prof. Dr. Marcus Quinkler, Berlin for the Advisory Board of the Section Adrenal Glands, Steroids and Hypertension

The German Society of Endocrinology is the scientific society and lobby group for all those who research, teach or practice medicine in the field of endocrinology. More about the DGE.

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