Persistence, effectiveness, and real-world outcomes in psoriasis patients treated with secukinumab in Portugal.

To characterize moderate to severe psoriasis (PsO) adult patients treated with secukinumab, estimate drug persistence and assess any reasons for treatment discontinuation. Non-interventional, retrospective, longitudinal record-based study including patients diagnosed with PsO who started secukinumab between January 2018 and January 2020. Baseline characteristics were analyzed by descriptive statistics; drug persistence and predictive factors were assessed through Kaplan-Meier curves and univariate and multivariate analysis, respectively. A total of 302 patients were included in the study: mean age was 48.4 years, 41.7% were female, median time since diagnosis was 12.9 years. 51.3% of patients were bio-naïve while 48.7% had previously been treated with biologics. PsO in difficult-to-treat locations (DTL) was present in 82.1% of patients, with scalp PsO in about half of patients. At 5-years follow-up, 84 patients discontinued secukinumab, 45 of which due to loss of efficacy. At week 104, overall treatment persistence was 71.7%. A higher probability of drug persistence was identified among those patients who initiated secukinumab ≥5 years after diagnosis, were bio-naïve or treated with only one previous biologic, had no PsO on DTL, and had diabetes mellitus. The predictive factors for discontinuation identified in our study were the start of secukinumab <5 years after diagnosis (p = 0.001), the bio-experimented status with ≥2 biologics (p = 0.007), and the presence of PsO on DTL (p = 0.014). A time since diagnosis of ≥5 years, naïve status or previous use of only one biologic are predictors for secukinumab persistence, whereas the presence of PsO on DTL predicts drug discontinuation.

Pachyonychia congenita type 2 (Jackson-Lawler syndrome) or PC-17: case report.

Pachyonychia congenita (PC) is a rare genodermatosis caused by mutations in any of the four genes KRT6A, KRT6B, KRT16, or KRT17, which can lead to dystrophic, thickened nails and focal palmoplantar keratoderma, among other manifestations. Although classically subdivided into two major variants, PC-1 (Jadassohn-Lewandowski syndrome) and PC-2 (Jackson-Lawler syndrome), according to the localization of the mutations in the KRT6A/KRT16 or KRT6B/KRT17 genes, respectively, a classification system based on the mutant gene (PC-6a, PC-6b, PC-16 and PC-17) has been recently proposed. We report a 2-year-old female patient with a history of thickened and discolored nails, small cystic papulonodules on the central face, dry, unruly and curly hair, slight palmoplantar hyperkeratosis, and natal teeth. Both her father and paternal grandfather presented onychodystrophy, palmoplantar keratoderma, and previous excision of "sebaceous" cysts. Molecular genetic analysis of the patient revealed a missense mutation (c.1163T>C) in heterozygosity in exon 6 of the KRT17 gene, confirming the diagnosis of PC-2 (Jackson-Lawler type), or PC-17. We conclude that PC is a relatively easy and consistent clinical diagnosis, but a high index of suspicion is required if the diagnosis is to be made correctly. With this case, the authors intend to draw attention to this condition and the role of the dermatologist in the diagnosis.