Central Nervous System Xanthoma Disseminatum: Response to 2CdA in an Adolescent
Xanthoma disseminatum is a normolipemic non-Langerhans cell histiocytosis characterized by red-brown rubbery papules of the skin which coalesce into plaque-like lesions with symmetric involvement of face, flexor, and intertriginous areas. Less commonly, xanthoma disseminatum may affect mucosal linin...
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| Format: | Article |
| Language: | English |
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Wiley
2022-01-01
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| Series: | Case Reports in Pediatrics |
| Online Access: | http://dx.doi.org/10.1155/2022/9906668 |
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| author | Patrick DeMoss Nancy Tang Kristen Yeom Audris Chiang Ann L. Marqueling Michael R. Jeng |
| author_facet | Patrick DeMoss Nancy Tang Kristen Yeom Audris Chiang Ann L. Marqueling Michael R. Jeng |
| author_sort | Patrick DeMoss |
| collection | DOAJ |
| description | Xanthoma disseminatum is a normolipemic non-Langerhans cell histiocytosis characterized by red-brown rubbery papules of the skin which coalesce into plaque-like lesions with symmetric involvement of face, flexor, and intertriginous areas. Less commonly, xanthoma disseminatum may affect mucosal linings, abdominal organs, and the central nervous system, leading to endocrinopathies. We report a 12-year-old adolescent with mucosal, central nervous system, and painful cutaneous lesions, further complicated by diabetes insipidus and amenorrhea. Treatment with 2-chlorodeoxyadenosine led to relief of pain and significant improvement of mucosal, central nervous system, and cutaneous lesions, with subsequent restoration of menstrual cycles. |
| format | Article |
| id | doaj-art-07a2d46db69e4a85a3102a5408c51bc8 |
| institution | OA Journals |
| issn | 2090-6811 |
| language | English |
| publishDate | 2022-01-01 |
| publisher | Wiley |
| record_format | Article |
| series | Case Reports in Pediatrics |
| spelling | doaj-art-07a2d46db69e4a85a3102a5408c51bc82025-08-20T02:01:35ZengWileyCase Reports in Pediatrics2090-68112022-01-01202210.1155/2022/9906668Central Nervous System Xanthoma Disseminatum: Response to 2CdA in an AdolescentPatrick DeMoss0Nancy Tang1Kristen Yeom2Audris Chiang3Ann L. Marqueling4Michael R. Jeng5Departments of Pediatrics (PDDepartments of Pediatrics (PDDepartments of Pediatrics (PDDepartments of Pediatrics (PDDepartments of Pediatrics (PDDepartments of Pediatrics (PDXanthoma disseminatum is a normolipemic non-Langerhans cell histiocytosis characterized by red-brown rubbery papules of the skin which coalesce into plaque-like lesions with symmetric involvement of face, flexor, and intertriginous areas. Less commonly, xanthoma disseminatum may affect mucosal linings, abdominal organs, and the central nervous system, leading to endocrinopathies. We report a 12-year-old adolescent with mucosal, central nervous system, and painful cutaneous lesions, further complicated by diabetes insipidus and amenorrhea. Treatment with 2-chlorodeoxyadenosine led to relief of pain and significant improvement of mucosal, central nervous system, and cutaneous lesions, with subsequent restoration of menstrual cycles.http://dx.doi.org/10.1155/2022/9906668 |
| spellingShingle | Patrick DeMoss Nancy Tang Kristen Yeom Audris Chiang Ann L. Marqueling Michael R. Jeng Central Nervous System Xanthoma Disseminatum: Response to 2CdA in an Adolescent Case Reports in Pediatrics |
| title | Central Nervous System Xanthoma Disseminatum: Response to 2CdA in an Adolescent |
| title_full | Central Nervous System Xanthoma Disseminatum: Response to 2CdA in an Adolescent |
| title_fullStr | Central Nervous System Xanthoma Disseminatum: Response to 2CdA in an Adolescent |
| title_full_unstemmed | Central Nervous System Xanthoma Disseminatum: Response to 2CdA in an Adolescent |
| title_short | Central Nervous System Xanthoma Disseminatum: Response to 2CdA in an Adolescent |
| title_sort | central nervous system xanthoma disseminatum response to 2cda in an adolescent |
| url | http://dx.doi.org/10.1155/2022/9906668 |
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