CONGENITAL ADRENAL HYPERPLASIA: DERMATOLOGICAL EVALUATION AND CLINICAL TREATMENT

Abstract

Congenital adrenal hyperplasia (CAH) is a group of genetic disorders that affect the synthesis of steroid hormones by the adrenal glands. These hormones include cortisol, which regulates metabolism and the stress response, aldosterone, which controls sodium and potassium balance, and androgens, which are the male sex hormones. This form causes deficiency of cortisol and aldosterone, and excess of androgens, resulting in symptoms such as virilization of the external genitalia in girls, salt wasting, dehydration, hypotension, hyponatremia, hyperkalemia and adrenal crisis. CAH can have repercussions on the skin, such as hyperpigmentation, acne, hirsutism and alopecia. The diagnosis of CAH is made through biochemical and genetic tests, and the treatment consists of appropriate hormone replacement for each case. Objective: to evaluate the scientific literature on dermatological assessment and clinical treatment of CAH. Methodology: For this, the PRISMA methodology was used, the databases PubMed, Scielo, Web of Science and DeCS (Health Sciences Descriptors) were consulted, using the following descriptors: “congenital adrenal hyperplasia”, “skin”, “ dermatology”, “treatment” and “corticosteroids”. Articles published in the last 10 years, in Portuguese or English, that addressed clinical and therapeutic aspects of CAH related to the skin were included. Articles that were not original, had no relevance to the topic or had low methodological quality were excluded. Results: 18 articles were selected for the complete analysis. The main topics covered by the articles were: the cutaneous manifestations of CAH, such as hyperpigmentation, acne, hirsutism and alopecia; skin-based CAH diagnostic methods, such as the ACTH (adrenocorticotropic hormone) stimulation test and serum 17-hydroxyprogesterone measurement; therapeutic options for CAH, such as oral hydrocortisone, oral fludrocortisone and topical or systemic antiandrogens; and the adverse effects of CAH treatment on the skin, such as skin atrophy, stretch marks, fungal and bacterial infections. Conclusion: CAH is an endocrine disorder that can significantly affect patients' skin, both due to deficiency and excess of steroid hormones. Dermatological evaluation is important for early diagnosis and monitoring the evolution of the disease. Clinical treatment aims to correct hormonal imbalance and improve patients' quality of life. However, the treatment can cause side effects on the skin that must be monitored and prevented.