Hirsutism (hirsutism)

HIRSUTISMUS, hirsutism (from Latin hirsutus - hairy), a name proposed by French authors to denote a complex syndrome observed in females and consisting of the following symptoms: hairiness of the entire body (hypertrichosis, hence the name of the syndrome itself), obesity, virilism, and dysfunction of ovarian activity in the sense of amenorrhea or premature menstruation. Another name for the same syndrome, proposed by Gallais, is adreno-genital or genito-suprarenal, indicating its genetic connection with lesions of two endocrine glands: the adrenal cortex and the gonads. To date, more than 50 cases of H. have been described, of which about 30 with autopsy or surgery. The oldest case was described by Cooke (1756). The clinical picture is as follows. In girls from 1 to 12 years of age, with moderately accelerated growth, the external genitalia and secondary sexual characteristics begin to develop prematurely, taking on characteristics typical of the male sex: hair appears not only on the pubis but also on the face in the form of mustaches and beards (see separate table, fig. 1), as well as on the perineum, extremities, and the entire body; the voice becomes rough; the clitoris takes on the size of the male sexual organ; the mammary glands enlarge, but due to fatty, not glandular, tissue. Menstruation usually does not occur or, having appeared, subsequently ceases; the internal sexual organs remain underdeveloped. General obesity is sometimes significant. The psyche and intellect are at the level of the actual age or even backward. Such patients usually die in childhood from accidental infections (septicemia). At autopsy, hyperplasia of the adrenal cortex or a tumor of the hypernephroma type is found; sometimes these tumors are located in the pelvis, broad ligaments, or ovaries. In Colletti's case (a girl 2 years old) after a successfully performed operation (a hypernephroma of the adrenal was removed), the symptoms of H. began to disappear: the hair fell out, the obesity decreased, the voice became childish, and the development of the external genitalia was arrested. Thus, the connection of the entire syndrome with a tumor of the adrenal cortex is beyond doubt. Considering that the basis of H. is an intensification of the functional activity of the adrenal cortex - hyperepinephria, or hypersuprarenalism, Anep (Apert) proposes to distinguish 5 types of this syndrome, depending on the period of development of the organism during which hyperplasia of the adrenal cortex occurs. 1) Embryonic type, when hyperplasia occurs still in the earliest period of intrauterine life, leading to false female hermaphroditism: internal sexual organs are female, while external ones are male. 2) Fetal type - an anomaly relating to a later period of intrauterine life; there is no doubt about the sex, a large clitoris, underdeveloped uterus and ovaries, hypertrichosis. 3) Childhood type - the most common H. in the narrow sense of the word, with the clinical picture described above. 4) H. of mature age, developing after the period of puberty; menstruation ceases, hypertrichosis and obesity. 5) H. or hyperepinephria of the climacteric period: obesity, hypertrichosis, disturbance of sexual function (sometimes in the form of metrorrhagia). However, such an extensive interpretation of the syndrome is hardly advisable; the name "hirsutism" should be preserved only for its childhood form. In some cases that underwent detailed microscopic examination, in addition to the adrenal glands and ovaries (atrophy, cystic degeneration of the latter), changes were also found in other endocrine glands (pituitary, thyroid and parathyroid glands), as well as in the central nervous system, which must in part be attributed to early childhood (Molchanov and Davydovsky). Thus, H. should be regarded as a complex, deeply constitutional, neuro-pluriglandular disease, in which the primary role still belongs to the adrenal cortex and ovaries. The genesis of individual symptoms of hirsutism remains very obscure. One can speak of the direct influence of adrenal cortex hormones on the development of the hair cover and fat metabolism, but in view of the close genetic, morphological, and functional relationship that exists between the adrenal glands and the gonads, particularly with the ovaries, it is more correct to consider the origin of the symptoms of H. as the result of indirect influence through the ovaries, and in individual cases, perhaps also through the vegetative centers of the diencephalon. The question of the genesis of virilism in adenomas of the adrenal cortex is very interesting and at the same time mysterious. Some (Conn) attribute virilism to the influence of male sex hormones latently existing in the female organism, the embryonic remnants of which are scattered in the adrenal glands. Others (Peritz, Molchanov and Davydovsky) view the enlargement of the clitoris, male-type hirsutism, and other symptoms of virilism in hirsute women as the appearance of "independent" secondary sexual characteristics as a result of the loss of ovarian function, similar to what is observed on a smaller scale in the climacteric period and as happens in experiments on animals, when castrated roosters develop spurs, castrated ducks acquire a magnificent plumage. It should be noted that if a tumor of the adrenal glands develops in boys (4 cases), it leads not to feminization, but to premature physical and sexual development (tasso-genitosomia praecox), with a sharp manifestation of male traits - powerful musculature and skeleton, hypertrichosis; obesity was observed in only one case. Such children are called Hercules boys. Diagnosis is made on the basis of the characteristic clinical picture and the presence of a tumor in the abdominal cavity. Early diagnosis is important in order to promptly apply treatment, which can only be surgical. In two cases (Colletti, Hamilton), operations were successfully performed with the subsequent disappearance of the symptoms of H. In six cases (Linser, Schiff, Sachs, Dobbertin, Adam, Hyman), death occurred shortly after the operation. Lit.: Molchanov V. and Davydovsky I., On the clinic and pathogenesis of hirsutism, "Russian Clinic", vol. VI, No. 30, 1926; Klumon E., Premature sexual development in women, "Bulletin of rhino-laryngo-otiatry", 1926, No. 4-5; Vereshchaginsky A., The adreno-genital syndrome from the point of view of surgical pathology and therapy, "Bulletin of Surgery and Bordering Fields", vol. III, books 8-9, vol. IV, books 10-11, 1924; Matthias E., Über Geschwülste der Nebennierenrinde mit morphogenetischen Wirkungen, Virchows Archiv, vol. CCXXXVI, 1922; Senidt H., Der suprarenal-genitale Syndrom (Kraus), ibid., vol. CCLI, 1924; Apert E., Insuffisance testiculaire et ovarienne (Nouveau traité de médecine. sous la dir. de G. Roger, F. Widal et P. Teissier. fasc. 8, p., 1925); ibid., Sur l'hirsutisme, Bulletin et mémoires de la société médicale des hôpitaux de Paris, vol. XLIX, 1925; Colletti A., Genito-suprarenal syndrome (suprarenal virilism) in a girl 1¹/2 years old with successful operation, American journal of diseases of children, vol. XXVII, 1924.