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Although many of the genetic diseases of steroidogenesis are rare, disordered steroidogenesis occurs commonly in prevalent diseases such as polycystic ovarian syndrome and primary aldosteronism. It is more common in adolescents than in men of reproductive age and is caused by genetic mutations in the pituitary gland or in ovaries, but can also arise from chromosomal or cell cycle abnormalities or the action of endogenous hormones. The cause of this disorder is unknown. Some cause are autosomal dominant; others are X-linked. In the affected individuals, steroidogenesis is altered; the pituitary size declines and testosterone and luteinizing hormone become diminished. The increased sensitivity to dihydrotestosterone and progesterone causes the ovaries to become large and, thereby, impregnate the uterus. Ovulation and pregnancy are usually disrupted; fetal defects are increased. The offspring usually pass through lactation, where their sexual differentiation is retarded. Progesterone and estradiol concentrations are normally controlled by the pituitary hormones. Because steroidogenesis is modified in these individuals, it is possible that some of the symptoms seen with a primary aldosteronism, e.g. acne, acne-like lesions, hair loss, weight gain, and other manifestations that can also affect females, may occur in females with this disorder. Diagnosis of Scleroderma (Scleroderma nodosa) depends on examination. An oral glucose tolerance test, with the determination of total and HDL cholesterol, is the first step to diagnosis. An ultrasound is needed, to determine the degree of nodularity in the face, neck, trunk, and limbs. With the use of the Sperring stain (Bromoform, Inc., Woodbury, Minn.) the diagnosis is made. A biopsy is then taken to determine histologic diagnosis. Biopsy or surgical excision of the nodules may be indicated if there have been a clinical diagnosis of Scleroderma, or if some of the lesions show abnormal nodular progression or progress. Diagnosis should be confirmed by histologic findings; a diagnosis of Scleroderma should be established by the occurrence of a clinical history of steroid-induced nodular disease. The diagnostic procedures are similar (see Diagnosis of Scleroderma). Treatment of Steroid-Induced Diplocephaly There are no formal treatment protocols for Steroid-Induced Diplocephaly (SID). The treatment of this disorder is based on the patient's response to steroid therapy and his desire to proceed as rapidly as possible. Administration of steroid therapy should be restricted to patients Similar articles: