Psoriasis (Auckland, N.Z.) · 2026
Genotype-Phenotype Correlations and Clinical Outcomes of Spesolimab in Acrodermatitis Continua of Hallopeau: A Multicenter Retrospective Cohort Study.
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Deng Xuan, Chen Yusha, Liu Xinli, Xiong Jianxia, Hu Runlu, Liao Sijian, Li Wei, Huang Kun
Аннотация
PURPOSE: To explore genotype-phenotype associations of IL36RN variants and clinical outcomes of spesolimab treatment in acrodermatitis continua of Hallopeau (ACH).
PATIENTS AND METHODS: This multicenter retrospective study included 14 ACH patients with IL36RN genotyping from 2019 to 2025. Clinical features were compared across all genotypes. Medical records of individuals who received spesolimab were reviewed to describe treatment responses, with a follow-up observation period up to 56 weeks.
RESULTS: Compared with monoallelic or wild-type genotypes, biallelic IL36RN variants were associated with earlier disease onset age (23 vs 48 and 47 years, P <0.05) and higher baseline disease severity (median GPPGA score, 4 vs 3 and 2, P <0.001). Among 6 patients with biallelic, refractory ACH who received spesolimab, including 3 isolated cases that were administered at a 450-mg dose, overall percentage improvement across disease activity and patient-reported measures occurred rapidly, reaching 54% at week 1 and 74% at week 4. Relapse occurred in 4 patients between weeks 31 and 44, with disease activity remaining lower than baseline and improvement observed after re-treatment.
CONCLUSION: Spesolimab was associated with clinical improvement in refractory ACH with biallelic IL36RN mutations, including isolated ACH treated with a 450-mg dose, supporting further evaluation of dose individualization below the approved 900-mg regimen for GPP flares.
PATIENTS AND METHODS: This multicenter retrospective study included 14 ACH patients with IL36RN genotyping from 2019 to 2025. Clinical features were compared across all genotypes. Medical records of individuals who received spesolimab were reviewed to describe treatment responses, with a follow-up observation period up to 56 weeks.
RESULTS: Compared with monoallelic or wild-type genotypes, biallelic IL36RN variants were associated with earlier disease onset age (23 vs 48 and 47 years, P <0.05) and higher baseline disease severity (median GPPGA score, 4 vs 3 and 2, P <0.001). Among 6 patients with biallelic, refractory ACH who received spesolimab, including 3 isolated cases that were administered at a 450-mg dose, overall percentage improvement across disease activity and patient-reported measures occurred rapidly, reaching 54% at week 1 and 74% at week 4. Relapse occurred in 4 patients between weeks 31 and 44, with disease activity remaining lower than baseline and improvement observed after re-treatment.
CONCLUSION: Spesolimab was associated with clinical improvement in refractory ACH with biallelic IL36RN mutations, including isolated ACH treated with a 450-mg dose, supporting further evaluation of dose individualization below the approved 900-mg regimen for GPP flares.