Treatment Options for Juvenile Pityriasis Rubra Pilaris.
Journal
Paediatric drugs
ISSN: 1179-2019
Titre abrégé: Paediatr Drugs
Pays: Switzerland
ID NLM: 100883685
Informations de publication
Date de publication:
Mar 2023
Mar 2023
Historique:
accepted:
22
11
2022
pubmed:
19
12
2022
medline:
18
2
2023
entrez:
18
12
2022
Statut:
ppublish
Résumé
Pityriasis rubra pilaris represents a group of familial and acquired disorders of cornification that affect both adult and pediatric patients. Treatment options are difficult to assess through clinical trials, given the rarity of the disorder and its tendency for spontaneous remission. Case reports and case series are therefore the primary means of assessment. Because of the heterogeneity of the disease, there is no universal approach to treatment, and multiple agents may need to be trialed to achieve disease control. At present, topicals are used for most pediatric patients, though monotherapy with topicals is only effective for less severe disease. Despite concerns over their side-effect profiles, oral retinoids are generally accepted as a first-line systemic therapy. However, interleukin-17 inhibitors and ustekinumab, an interleukin-12 and interleukin-23 inhibitor, may soon become first-line systemic treatment as well, given their efficacy and relative safety in trials thus far. Ustekinumab, in particular, is emerging as a first-line agent for patients with pityriasis rubra pilaris with CARD14 gene variations. When these therapies fail, second-line and adjunctive therapies to consider include tumor necrosis factor-alpha inhibitors, methotrexate, and phototherapy. However, further investigation is necessary to assess the safety and efficacy of many of these agents in juvenile pityriasis rubra pilaris.
Identifiants
pubmed: 36529810
doi: 10.1007/s40272-022-00549-4
pii: 10.1007/s40272-022-00549-4
doi:
Substances chimiques
Ustekinumab
FU77B4U5Z0
Dermatologic Agents
0
Methotrexate
YL5FZ2Y5U1
Retinoids
0
CARD14 protein, human
EC 4.6.1.-
Guanylate Cyclase
EC 4.6.1.2
Membrane Proteins
0
CARD Signaling Adaptor Proteins
0
Types de publication
Journal Article
Review
Langues
eng
Sous-ensembles de citation
IM
Pagination
151-164Informations de copyright
© 2022. The Author(s), under exclusive licence to Springer Nature Switzerland AG.
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