Treatment Approaches for Diffuse Cutaneous Mastocytosis in Children: Literature Review and Actual Clinical Experience
Background. Mastocytosis is a very rare disease with various manifestations, based on abnormal clonal proliferation of mast cells in organs and tissues, such as: skin, bone marrow, lymph nodes, liver, spleen, and gastrointestinal tract. The diagnosis can be established according to clinical manifest...
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"Paediatrician" Publishers LLC
2024-11-01
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| Series: | Вопросы современной педиатрии |
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| Online Access: | https://vsp.spr-journal.ru/jour/article/view/3613 |
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| author | Timur T. Valiev Nikolay N. Murashkin Tatiana S. Belysheva Zarina G. Misikova Daria A. Pronina Roman V. Epishev Roman A. Ivanov |
| author_facet | Timur T. Valiev Nikolay N. Murashkin Tatiana S. Belysheva Zarina G. Misikova Daria A. Pronina Roman V. Epishev Roman A. Ivanov |
| author_sort | Timur T. Valiev |
| collection | DOAJ |
| description | Background. Mastocytosis is a very rare disease with various manifestations, based on abnormal clonal proliferation of mast cells in organs and tissues, such as: skin, bone marrow, lymph nodes, liver, spleen, and gastrointestinal tract. The diagnosis can be established according to clinical manifestations, laboratory, and instrumental data. Darier’s sign and histological examination are crucial for mastocytosis diagnosis. The presented clinical case describes very rare cutaneous form of mastocytosis. Clinical case description. The girl, 2.5 years old, was hospitalized with multiple erythematous papules on her body, face, and limbs. Comprehensive examination, including bone marrow biopsy and positron-emission tomography, allowed us to exclude mastocytosis systemic manifestations. Conclusion. Despite the fact that mastocytosis in children is mostly represented by skin form, it is necessary to perform complex patient examination on any systemic damage. Antihistamines in combination with topical and/or systemic glucocorticoids are often effective, but complete response does not always occur. Implementation of other therapeutic options, such as targeted drugs (tyrosine kinase inhibitors), is suggested In case of no or insufficient therapeutic effect. |
| format | Article |
| id | doaj-art-e0568523fc4a4f96affc56dd210a1d40 |
| institution | DOAJ |
| issn | 1682-5527 1682-5535 |
| language | English |
| publishDate | 2024-11-01 |
| publisher | "Paediatrician" Publishers LLC |
| record_format | Article |
| series | Вопросы современной педиатрии |
| spelling | doaj-art-e0568523fc4a4f96affc56dd210a1d402025-08-20T03:01:17Zeng"Paediatrician" Publishers LLCВопросы современной педиатрии1682-55271682-55352024-11-0123538439010.15690/vsp.v23i5.27942204Treatment Approaches for Diffuse Cutaneous Mastocytosis in Children: Literature Review and Actual Clinical ExperienceTimur T. Valiev0Nikolay N. Murashkin1Tatiana S. Belysheva2Zarina G. Misikova3Daria A. Pronina4Roman V. Epishev5Roman A. Ivanov6National Medical Research Center of Oncology named after N.N. Blokhin; Sechenov First Moscow State Medical University; Russian Medical Academy of Continuing Professional EducationSechenov First Moscow State Medical University; National Medical Research Center of Children’s Health; Central State Medical Academy of Department of Presidential AffairsNational Medical Research Center of Oncology named after N.N. BlokhinRussian Medical Academy of Continuing Professional EducationCentral State Medical Academy of Department of Presidential AffairsNational Medical Research Center of Children’s HealthNational Medical Research Center of Children’s HealthBackground. Mastocytosis is a very rare disease with various manifestations, based on abnormal clonal proliferation of mast cells in organs and tissues, such as: skin, bone marrow, lymph nodes, liver, spleen, and gastrointestinal tract. The diagnosis can be established according to clinical manifestations, laboratory, and instrumental data. Darier’s sign and histological examination are crucial for mastocytosis diagnosis. The presented clinical case describes very rare cutaneous form of mastocytosis. Clinical case description. The girl, 2.5 years old, was hospitalized with multiple erythematous papules on her body, face, and limbs. Comprehensive examination, including bone marrow biopsy and positron-emission tomography, allowed us to exclude mastocytosis systemic manifestations. Conclusion. Despite the fact that mastocytosis in children is mostly represented by skin form, it is necessary to perform complex patient examination on any systemic damage. Antihistamines in combination with topical and/or systemic glucocorticoids are often effective, but complete response does not always occur. Implementation of other therapeutic options, such as targeted drugs (tyrosine kinase inhibitors), is suggested In case of no or insufficient therapeutic effect.https://vsp.spr-journal.ru/jour/article/view/3613mastocytosisdiffuse cutaneous mastocytosistherapytyrosine kinase inhibitors |
| spellingShingle | Timur T. Valiev Nikolay N. Murashkin Tatiana S. Belysheva Zarina G. Misikova Daria A. Pronina Roman V. Epishev Roman A. Ivanov Treatment Approaches for Diffuse Cutaneous Mastocytosis in Children: Literature Review and Actual Clinical Experience Вопросы современной педиатрии mastocytosis diffuse cutaneous mastocytosis therapy tyrosine kinase inhibitors |
| title | Treatment Approaches for Diffuse Cutaneous Mastocytosis in Children: Literature Review and Actual Clinical Experience |
| title_full | Treatment Approaches for Diffuse Cutaneous Mastocytosis in Children: Literature Review and Actual Clinical Experience |
| title_fullStr | Treatment Approaches for Diffuse Cutaneous Mastocytosis in Children: Literature Review and Actual Clinical Experience |
| title_full_unstemmed | Treatment Approaches for Diffuse Cutaneous Mastocytosis in Children: Literature Review and Actual Clinical Experience |
| title_short | Treatment Approaches for Diffuse Cutaneous Mastocytosis in Children: Literature Review and Actual Clinical Experience |
| title_sort | treatment approaches for diffuse cutaneous mastocytosis in children literature review and actual clinical experience |
| topic | mastocytosis diffuse cutaneous mastocytosis therapy tyrosine kinase inhibitors |
| url | https://vsp.spr-journal.ru/jour/article/view/3613 |
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