期刊
JOURNAL OF DERMATOLOGY
卷 45, 期 7, 页码 850-854出版社
WILEY
DOI: 10.1111/1346-8138.14318
关键词
clinical global impression; generalized pustular psoriasis; IL36RN; interleukin-17A antagonist treatment; psoriasis vulgaris
类别
资金
- AbbVie
- Almirall
- Amgen
- Biogen
- Boehringer Ingelheim Pharma
- Celgene
- Forward Pharma
- GlaxoSmithKline
- Janssen-Cilag
- Leo
- Lilly
- Medac
- Merck Sharp Dohme
- Novartis
- Pfizer
- UCB Pharma
- VBL
- Sobi
- Biogen IDEC
- Bohringer-Ingelheim
- Leo Pharma
- Merck Serono
- MSD Sharp Dohme
- Novartis Pharma
Generalized pustular psoriasis (GPP) is a potentially life-threatening disease that can be attributed to mutations in IL36RN in a subgroup of patients. In small trials, interleukin (IL)-17A and IL-17RA antagonists have been shown to be effective in patients with generalized pustular psoriasis in Japan. We identified seven patients who received the IL-17A antagonists secukinumab (six cases) or ixekizumab (one case) in two dermatological centers. All patients showed a good or excellent clinical response. Anti-IL-17A therapy was well tolerated and ongoing in all patients after an average therapy duration of 12.9 months. Analysis of IL36RN mutation status was performed in six patients, one patient carried a heterozygous mutation, while the other five patients did not show a mutation in IL36RN. This is the first report of a successful treatment of GPP patients without IL36RN mutations responding to anti-IL-17A therapy.
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