Peutz - Jeghers Syndrome: Problems of Diagnosis and Therapy
The etiology, pathogenesis, as well as dermatoscopic and clinical varieties of Peutz - Jeghers syndrome are presented in the article and a clinical case of the syndrome in an 8-year-old girl is described, which shows the difficulties of diagnosis and treatment. It is known that the diagnosis and treatment of Peutz - Jeghers syndrome immediately captures three diverse clinical disciplines: oncology, dermatology, and surgery. Patients have a significantly increased risk of developing gastrointestinal cancer in the future and its other localization: cancer of the pancreas, stomach, small intestine and colon, less commonly - cancer of the breast, lung, uterus, ovaries, and testicles. The authors carried out serious work: an exhaustive diagnostic study, recommendations on a medical-protective regimen are given, tactics of the behavior of the parents of the child in case of future complications are described in detail. Algorithms of behavior of each specialist are important. This article reflects the real work done in accordance with modern protocols, and can be an example of the proper management of patients with rare dermatological pathologies, requiring an interdisciplinary approach.