Description:
<jats:title>Abstract</jats:title><jats:p>Generalized pustular psoriasis (<jats:styled-content style="fixed-case">GPP</jats:styled-content>) is a potentially life‐threatening disease that can be attributed to mutations in <jats:italic><jats:styled-content style="fixed-case">IL</jats:styled-content>36<jats:styled-content style="fixed-case">RN</jats:styled-content></jats:italic> in a subgroup of patients. In small trials, interleukin (<jats:styled-content style="fixed-case">IL</jats:styled-content>)‐17A and <jats:styled-content style="fixed-case">IL</jats:styled-content>‐17<jats:styled-content style="fixed-case">RA</jats:styled-content> antagonists have been shown to be effective in patients with generalized pustular psoriasis in Japan. We identified seven patients who received the <jats:styled-content style="fixed-case">IL</jats:styled-content>‐17A antagonists secukinumab (six cases) or ixekizumab (one case) in two dermatological centers. All patients showed a good or excellent clinical response. Anti‐<jats:styled-content style="fixed-case">IL</jats:styled-content>‐17A therapy was well tolerated and ongoing in all patients after an average therapy duration of 12.9 months. Analysis of <jats:italic><jats:styled-content style="fixed-case">IL</jats:styled-content>36<jats:styled-content style="fixed-case">RN</jats:styled-content></jats:italic> mutation status was performed in six patients, one patient carried a heterozygous mutation, while the other five patients did not show a mutation in <jats:italic><jats:styled-content style="fixed-case">IL</jats:styled-content>36<jats:styled-content style="fixed-case">RN</jats:styled-content></jats:italic>. This is the first report of a successful treatment of <jats:styled-content style="fixed-case">GPP</jats:styled-content> patients without <jats:italic><jats:styled-content style="fixed-case">IL</jats:styled-content>36<jats:styled-content style="fixed-case">RN</jats:styled-content></jats:italic> mutations responding to anti‐<jats:styled-content style="fixed-case">IL</jats:styled-content>‐17A therapy.</jats:p>