Central Nervous System Xanthoma Disseminatum: Response to 2CdA in an Adolescent.


Journal

Case reports in pediatrics
ISSN: 2090-6803
Titre abrégé: Case Rep Pediatr
Pays: United States
ID NLM: 101581030

Informations de publication

Date de publication:
2022
Historique:
received: 03 11 2021
accepted: 15 06 2022
entrez: 1 8 2022
pubmed: 2 8 2022
medline: 2 8 2022
Statut: epublish

Résumé

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.

Identifiants

pubmed: 35910691
doi: 10.1155/2022/9906668
pmc: PMC9337940
doi:

Types de publication

Case Reports

Langues

eng

Pagination

9906668

Informations de copyright

Copyright © 2022 Patrick DeMoss et al.

Déclaration de conflit d'intérêts

The authors declare that they have no conflicts of interest.

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Auteurs

Patrick DeMoss (P)

Departments of Pediatrics (PD, NT, MJ), Radiology (KY), and Dermatology (AC, AM), Stanford University School of Medicine, Stanford, California, USA.

Nancy Tang (N)

Departments of Pediatrics (PD, NT, MJ), Radiology (KY), and Dermatology (AC, AM), Stanford University School of Medicine, Stanford, California, USA.

Kristen Yeom (K)

Departments of Pediatrics (PD, NT, MJ), Radiology (KY), and Dermatology (AC, AM), Stanford University School of Medicine, Stanford, California, USA.

Audris Chiang (A)

Departments of Pediatrics (PD, NT, MJ), Radiology (KY), and Dermatology (AC, AM), Stanford University School of Medicine, Stanford, California, USA.

Ann L Marqueling (AL)

Departments of Pediatrics (PD, NT, MJ), Radiology (KY), and Dermatology (AC, AM), Stanford University School of Medicine, Stanford, California, USA.

Michael R Jeng (MR)

Departments of Pediatrics (PD, NT, MJ), Radiology (KY), and Dermatology (AC, AM), Stanford University School of Medicine, Stanford, California, USA.

Classifications MeSH