vspВопросы современной педиатрииCurrent Pediatrics1682-55271682-5535Издательство «ПедиатрЪ»10.15690/vsp.v15i5.1626vsp-1676Research ArticleКЛИНИЧЕСКОЕ НАБЛЮДЕНИЕCLINICAL OBSERVATIONSОСОБЕННОСТИ ТЕЧЕНИЯ НЕЙРОКОЖНОГО МЕЛАНОЗА У ДЕТЕЙCOURSE PECULIARITIES OF NEUROCUTANEOUS MELANOSIS IN CHILDRENПрыгуноваТ. М.PrygunovaTatyana M.

Прыгунова Татьяна Михайловна - врач-невролог отделения для детей с поражением ЦНС и нарушением психики НОДКБ.

p-tanchita@yandex.ruКарповичЕ. И.КarpovichEkaterina I.ЧернигинаМ. Н.CherniginaMarina N.БересневаЕ. Е.BeresnevaElena E.ТрынковаЛ. А.TrynkovaLarisa A.Нижегородская областная детская клиническая больницаРоссияNizhny Novgorod Regional Children's Clinical HospitalRussian Federation201624112016155513521Copyright © Прыгунова Т.М., Карпович Е.И., Чернигина М.Н., Береснева Е.Е., Трынкова Л.А., 20162016Прыгунова Т.М., Карпович Е.И., Чернигина М.Н., Береснева Е.Е., Трынкова Л.А.Prygunova T.M., Кarpovich E.I., Chernigina M.N., Beresneva E.E., Trynkova L.A.Данная работа распространяется под лицензией Creative Commons Attribution 4.0.This work is licensed under a Creative Commons Attribution 4.0 License.https://vsp.spr-journal.ru/jour/article/view/1676

Нейрокожный меланоз входит в группу наследственных заболеваний, характеризуется большими и/или множественными пигментными невусами, меланозом или меланомой мягкой мозговой оболочки, без признаков злокачествен-ных кожных поражений и вовлечения других органов. Заболевание описано более 150 лет назад, но патогенез его остается недостаточно изученным, а эффективные методы лечения до сих пор не разработаны. Разный характер течения заболевания в связи с выраженным полиморфизмом клинических симптомов затрудняет диагностику, а низкая эффективность симптоматической терапии ухудшает прогноз заболевания. В статье описан опыт ведения детей с факоматозами, не схожими между собой ни дебютом и течением, ни ответом на терапию и прогнозом. Ранняя диагностика нейрокожного меланоза у детей позволяет проводить своевременное симптоматическое лечение, осуществлять динамическое наблюдение и улучшать выживаемость пациентов.

Neurocutaneous melanosis is part of a group of hereditary diseases characterized by large and/or multiple pigmented nevi, melanosis or melanoma of the pia mater, with no evidence of malignant skin lesions and involvement of other organs. The disease was described over 150 years ago, but its pathogenesis has not been studied yet, and treatment methods have not been developed yet. Different disease courses due to the pronounced polymorphism of clinical symptoms complicate the diagnosis, and the low efficacy of the symptomatic treatment worsens the disease prognosis. The article describes the experience of managing children with phakomatoses not similar to each other neither in debut and course nor in response to the therapy and prognosis. Early diagnosis of neurocutaneous melanosis in children allows to carry out timely symptomatic treatment and dynamical monitoring, and to improve the survival of patients.

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The authors declare that there are no conflicts of interest present.