nodgoРоссийский журнал детской гематологии и онкологии (РЖДГиО)Russian Journal of Pediatric Hematology and Oncology2311-12672413-5496LTD “Graphica”10.21682/2311-1267-2022-9-2-60-74nodgo-831Research ArticleОБЗОРЫ ЛИТЕРАТУРЫLITERATURE REVIEWSГенодерматозы, ассоциированные со злокачественными опухолямиCancer-associated genodermatoseshttps://orcid.org/0000-0001-5911-553XБелышеваТ. С.BelyshevaT. S.

Татьяна Сергеевна Белышева,д.м.н., ведущий научный сотрудник научно-консультативного отделения НИИ детской онкологии и гематологии

SPIN-код: 2645-4049

115478, Москва, Каширское шоссе, 23

Dr. of Sci. (Med.), Leading Researcher Scientific Advisory Division Research Institute of Pediatric Oncology and Hematology of N.N. Blokhin National Medical Research Center of Oncology, Ministry of Health of Russia

23 Kashirskoe Shosse, Moscow, 115478

klinderma@bk.ruhttps://orcid.org/0000-0002-2642-4202НаседкинаТ. В.NasedkinaT. V.

д.б.н., профессор, ведущий научный сотрудник лаборатории биологических микрочипов

SPIN-код: 3741-8214

119991, Москва, ул. Вавилова, 325

Dr. of Sci. (Biol.), Leading Researcher Laboratory of Biological Microchips of Engelhardt Institute of Molecular Biology, Russian Academy of Sciences

SPIN-code: 3741-8214

32 Vavilova St., Moscow, 119991, GSP-1

tanased06@rambler.ruhttps://orcid.org/0000-0002-9705-1001СеменоваВ. В.SemenovaV. V.

врач-генетик НИИ детской онкологии и гематологии; аспирант лаборатории биологических микрочипов

SPIN-код: 9014-2847

115478, Москва, Каширское шоссе, 23

119991, Москва, ул. Вавилова, 325

Geneticist Research Institute of Pediatric Oncology and Hematology; Graduate Student of the Laboratory of Biological Microchips

23 Kashirskoe Shosse, Moscow, 115478

32 Vavilova St., Moscow, 119991, GSP-1

sulpiridum@yandex.ruhttps://orcid.org/0000-0002-0442-5810КозловаВ. М.KozlovaV. M.

врач-генетик научно-консультативного отделения НИИ детской онкологии и гематологии

115478, Москва, Каширское шоссе, 23

Geneticist of the Scientific Advisory Department Research Institute of Pediatric Oncology and Hematology

23 Kashirskoe Shosse, Moscow, 115478

valentina-mk2011@yandex.ruhttps://orcid.org/0000-0002-5836-0338ШараповаЕ. В.SharapovaE. V.

врач-онколог научно-консультативного отделения НИИ детской онкологии и гематологии

SPIN-код: 5236-8312

115478, Москва, Каширское шоссе, 23

Oncologist of the Scientific Advisory Department Research Institute of Pediatric Oncology and Hematology

SPIN-code: 5236-8312

23 Kashirskoe Shosse, Moscow, 115478

sharapovae.v@yandex.ruhttps://orcid.org/0000-0002-9502-072XМихайловаС. Н.MikhailovaS. N.

к.м.н., заведующая научно-консультативным отделением НИИ детской онкологии и гематологии

115478, Москва, Каширское шоссе, 23

Cand. of Sci. (Med.), Head of Scientific Advisory Division Research Institute of Pediatric Oncology and Hematology

23 Kashirskoe Shosse, Moscow, 115478

astra-sn@mail.ruhttps://orcid.org/0000-0002-1469-2365ВалиевТ. Т.ValievT. T.

д.м.н., заведующий отделением детской онкологии и гематологии (химиотерапия гемобластозов) № 1 НИИ детской онкологии и гематологии

115478, Москва, Каширское шоссе, 23

Dr. of Sci. (Med.), Head of the Department of Pediatric Oncology and Hematology (Chemotherapy of Hemoblastosis) No. 1 of the Research Institute of Pediatric Oncology and Hematology

23 Kashirskoe Shosse, Moscow, 115478

timurvaliev@mail.ruhttps://orcid.org/0000-0001-6131-1783ВарфоломееваС. Р.VarfolomeevaS. R.

д.м.н., профессор, директор НИИ детской онкологии и гематологии

115478, Москва, Каширское шоссе, 23

Dr. of Sci. (Med.), Professor, Director of the Research Institute of Pediatric Oncology and Hematology

23 Kashirskoe Shosse, Moscow, 115478

s.varfolomeeva@ronc.ruФГБУ «НМИЦ онкологии им. Н.Н. Блохина» Минздрава РоссииРоссияN.N. Blokhin National Medical Research Centre of Oncology, Ministry of Health of RussiaRussian FederationФГБУН «Институт молекулярной биологии имени В.А. Энгельгардта Российской академии наук»РоссияEngelhardt Institute of Molecular Biology, Russian Academy of SciencesRussian FederationФГБУ «НМИЦ онкологии им. Н.Н. Блохина» Минздрава России; ФГБУН «Институт молекулярной биологии имени В.А. Энгельгардта Российской академии наук»РоссияN.N. Blokhin National Medical Research Centre of Oncology, Ministry of Health of Russia; Engelhardt Institute of Molecular Biology, Russian Academy of SciencesRussian Federation202215082022926074Copyright © Белышева Т.С., Наседкина Т.В., Семенова В.В., Козлова В.М., Шарапова Е.В., Михайлова С.Н., Валиев Т.Т., Варфоломеева С.Р., 20222022Белышева Т.С., Наседкина Т.В., Семенова В.В., Козлова В.М., Шарапова Е.В., Михайлова С.Н., Валиев Т.Т., Варфоломеева С.Р.Belysheva T.S., Nasedkina T.V., Semenova V.V., Kozlova V.M., Sharapova E.V., Mikhailova S.N., Valiev T.T., Varfolomeeva S.R.Данная работа распространяется под лицензией Creative Commons Attribution 4.0.This work is licensed under a Creative Commons Attribution 4.0 License.https://journal.nodgo.org/jour/article/view/831

Генодерматозы представляют собой гетерогенную группу наследственных заболеваний, отличительным признаком которых является преимущественное поражение кожи. К настоящему времени насчитывается более 200 генетически детерминированных заболеваний кожи, что составляет около 35 % всех наследственных синдромальных патологий. В некоторых случаях кожные поражения могут быть единственным проявлением заболевания, но все же более часто они возникают в сочетании с нарушениями других систем органов. Во многих наблюдениях генодерматозы ассоциированы с повышенным риском развития злокачественных новообразований, что делает особенно важным раннее выявление наследственной синдромальной патологии для профилактики онкологических заболеваний.В настоящем обзоре приводится краткое описание дерматологических проявлений, а также других фенотипических особенностей ряда генодерматозов, их генетической природы и тактики ведения пациентов.

Genodermatoses are a heterogeneous group of hereditary diseases that are characterized by predominantly skin lesions. To date, there are more than 200 genetically determined skin diseases, representing about 35 % of all hereditary syndromes. In some cases, skin lesions may be the only manifestation of the disease, but still more often, they occur in combination with disorders of other organ systems. In many cases, genodermatoses are associated with an increased risk of malignancy which makes early detection of hereditary syndromic pathology especially important for cancer prevention.This review provides a brief description of the dermatological manifestations as well as other phenotypic features of a number of genodermatoses, their genetic nature, and the strategy of management.

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