Nikolay N. Murashkin, Alexander I. Materikin, Roman V. Epishev, Maria A. Leonova, Leonid A. Opryatin, Dmitri V. Fedorov, Roman A. Ivanov, Alena A. Savelova, Oksana R. Katunina, Kirill A. Kulikov, Anastasiya U. Ufimtseva
{"title":"Adalimumab Experience in the Management of Hidradenitis Suppurativa Burdened with Rapp-Hodgkin Syndrome","authors":"Nikolay N. Murashkin, Alexander I. Materikin, Roman V. Epishev, Maria A. Leonova, Leonid A. Opryatin, Dmitri V. Fedorov, Roman A. Ivanov, Alena A. Savelova, Oksana R. Katunina, Kirill A. Kulikov, Anastasiya U. Ufimtseva","doi":"10.15690/vsp.v22i5.2623","DOIUrl":null,"url":null,"abstract":"Background. Rapp-Hodgkin syndrome is a rare genetic disease from the ectodermal dysplasia group. It manifests with damage of ectodermal structures — layer of embrional tissue that provides the development of many organs and tissues in the body, such as: skin, sweat glands, hair, teeth, and nails. Disease symptoms may range from mild to severe and include dental abnormalities, fragile, thin or no hair, abnormal nails, hypohidrosis (due to the reduced number of sweat glands), cleft lips and palate. Hidradenitis suppurativa is a chronic inflammatory disease that predominantly affects apocrine glands areas. This nosology is characterized by painful nodes, abscesses, and fistulas, prone to relapses and leading to scarring.Clinical case description. The authors present a clinical case demonstrating the combination of Rapp-Hodgkin syndrome and severe hidradenitis suppurativa in 17-year-old male patient.Conclusion. The heterogeneous clinical findings of hidradenitis suppurativa and history of rare genetic disease result in patients' admissions to various medical specialists. Thus, it leads to delayed verification of the diagnosis and incorrect treatment methods. As a result, patients have persistent cosmetic skin defects, possible complications of irrational treatment, and decreased quality of life by the time of diagnosis.","PeriodicalId":37561,"journal":{"name":"Voprosy Sovremennoi Pediatrii - Current Pediatrics","volume":null,"pages":null},"PeriodicalIF":0.0000,"publicationDate":"2023-11-06","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":"0","resultStr":null,"platform":"Semanticscholar","paperid":null,"PeriodicalName":"Voprosy Sovremennoi Pediatrii - Current Pediatrics","FirstCategoryId":"1085","ListUrlMain":"https://doi.org/10.15690/vsp.v22i5.2623","RegionNum":0,"RegionCategory":null,"ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":null,"EPubDate":"","PubModel":"","JCR":"Q4","JCRName":"Medicine","Score":null,"Total":0}
引用次数: 0
Abstract
Background. Rapp-Hodgkin syndrome is a rare genetic disease from the ectodermal dysplasia group. It manifests with damage of ectodermal structures — layer of embrional tissue that provides the development of many organs and tissues in the body, such as: skin, sweat glands, hair, teeth, and nails. Disease symptoms may range from mild to severe and include dental abnormalities, fragile, thin or no hair, abnormal nails, hypohidrosis (due to the reduced number of sweat glands), cleft lips and palate. Hidradenitis suppurativa is a chronic inflammatory disease that predominantly affects apocrine glands areas. This nosology is characterized by painful nodes, abscesses, and fistulas, prone to relapses and leading to scarring.Clinical case description. The authors present a clinical case demonstrating the combination of Rapp-Hodgkin syndrome and severe hidradenitis suppurativa in 17-year-old male patient.Conclusion. The heterogeneous clinical findings of hidradenitis suppurativa and history of rare genetic disease result in patients' admissions to various medical specialists. Thus, it leads to delayed verification of the diagnosis and incorrect treatment methods. As a result, patients have persistent cosmetic skin defects, possible complications of irrational treatment, and decreased quality of life by the time of diagnosis.
期刊介绍:
The main purpose of the academic journal "Current Pediatrics" is to cover the issues related to health, emotional state, and social adaptation of children. In the journal we publish the most pressing issues of research and applied problems (such as social paediatrics; clinical findings, anamnesis, and diagnosis of paediatric diseases; prevention and rehabilitation; supply of healthy and sick child), reviews on the current state of medical science and management of health care in Russia. Along with the concern for providing a high level of basic research publications, the journal, being a mass media tool, tends to meet the interests and requirements of practitioners from different regions and bring up vital and urgent questions. To accomplish this purpose the journal includes the materials of practical interest presented in the following sections: "Exchange of experience", "Doctor''s aid", "Continuous professional education", "Expert Opinion". The journal presents actual official information from the Union of Paediatricians of Russia and publishes materials on the history of medical science, congresses, seminars, conferences, both in our country and abroad.