The Effectiveness of Guselkumab in Patients With Hidradenitis Suppurativa Under Clinical Practice Conditions: A Spanish Multicentre Retrospective Study. / [Artículo traducido] Efectividad de guselkumab en pacientes con hidradenitis supurativa en condiciones de práctica clínica: estudio retrospectivo y multicéntrico en España.

BACKGROUND: Hidradenitis suppurativa (HS) is a chronic skin condition causing lesions in which high levels of interleukin (IL)-23 and T-helper17 cells are found. Adalimumab remains the only approved treatment. Guselkumab, an antibody targeting the p19 protein subunit of extracellular IL-23, is approved for the treatment of moderate-severe psoriasis, but evidence on its efficacy in treating HS is limited. OBJECTIVES: To assess the effectiveness and safety of guselkumab in treating moderate-severe HS under clinical practice conditions. METHODS: A multicentre retrospective observational study was carried out in 13 Spanish hospitals including adult HS patients treated with guselkumab within a compassionate use programme (March 2020-March 2022). Data referred to patient demographic and clinical characteristics at treatment initiation (baseline), patient-reported outcomes (Numerical Pain Rating Scale [NPRS] and Dermatology Life Quality Index [DLQI]), physician scores (International Hidradenitis Suppurativa Severity Score System [IHS4], HS Physical Global Score [HS-PGA] and Hidradenitis Suppurativa Clinical Response [HiSCR]) were recorded at baseline and at 16, 24, and 48weeks of treatment. RESULTS: A total of 69 patients were included. Most (84.10%) had severe HS (HurleyIII) and had been diagnosed for over ten years (58.80%). The patients had been subjected to multiple non-biological (mean: 3.56) or biological (mean: 1.78) therapies, and almost 90% of those treated with biologics had received adalimumab. A significant decrease in IHS4, HS-PGA, NPRS, and DLQI scores was observed from baseline to 48weeks of guselkumab treatment (all P<.01). HiSCR was achieved in 58.33% and 56.52% of the patients at 16 and 24weeks, respectively. Overall, 16 patients discontinued treatment, mostly due to inefficacy (n=7) or loss of efficacy (n=3). No serious adverse events were observed. CONCLUSIONS: Our results indicate that guselkumab may be a safe and effective therapeutic alternative for patients with severe HS that fail to respond to other biologics.

The Effectiveness of Guselkumab in Patients With Hidradenitis Suppurativa Under Clinical Practice Conditions: A Spanish Multicentre Retrospective Study.

BACKGROUND: Hidradenitis suppurativa (HS) is a chronic skin condition causing lesions in which high levels of interleukin (IL)-23 and T-helper 17 cells are found. Adalimumab remains the only approved treatment. Guselkumab, an antibody targeting the p19 protein subunit of extracellular IL-23, is approved for the treatment of moderate-severe psoriasis, but evidence on its efficacy in treating HS is limited. OBJECTIVES: To assess the effectiveness and safety of guselkumab in treating moderate-severe HS under clinical practice conditions. METHODS: A multicentre retrospective observational study was carried out in 13 Spanish Hospitals including adult HS patients treated with guselkumab within a compassionate use programme (March 2020-March 2022). Data referred to patient demographic and clinical characteristics at treatment initiation (baseline), patient-reported outcomes (Numerical Pain Rating Scale [NPRS] and Dermatology Life Quality Index [DLQI]), physician scores (International Hidradenitis Suppurativa Severity Score System [IHS4], HS Physical Global Score [HS-PGA] and Hidradenitis Suppurativa Clinical Response [HiSCR]) were recorded at baseline and at 16, 24, and 48 weeks of treatment. RESULTS: A total of 69 patients were included. Most (84.10%) had severe HS (Hurley III) and had been diagnosed for over ten years (58.80%). The patients had been subjected to multiple non-biological (mean 3.56) or biological (mean 1.78) therapies, and almost 90% of those treated with biologics had received adalimumab. A significant decrease in IHS4, HS-PGA, NPRS, and DLQI scores was observed from baseline to 48 weeks of guselkumab treatment (all p<0.01). HiSCR was achieved in 58.33% and 56.52% of the patients at 16 and 24 weeks, respectively. Overall, 16 patients discontinued treatment, mostly due to inefficacy (n=7) or loss of efficacy (n=3). No serious adverse events were observed. CONCLUSIONS: Our results indicate that guselkumab may be a safe and effective therapeutic alternative for patients with severe HS that fail to respond to other biologics.

[Clinical outcomes in patients with psoriasis following discontinuation of efalizumab due to suspension of marketing authorization]. / Evolución clínica de los pacientes psoriásicos tratados con efalizumab al suspender el fármaco.

INTRODUCTION: In February 2009, the European Medicines Agency suspended the marketing authorization for efalizumab after 3 confirmed cases of progressive multifocal leukoencephalopathy were reported. To assess the consequences of this decision, we performed a prospective follow-up study of patients in our department who were being treated with efalizumab at the time and compared clinical outcomes with data from the literature. PATIENTS AND METHODS: Thirty-two patients (28 with plaque psoriasis and 4 with palmoplantar psoriasis) were enrolled between February and March 2009. We recorded psoriasis area and severity index (PASI) scores at the moment of efalizumab discontinuation, at 6 weeks post-discontinuation, and at 3-monthly intervals thereafter. PASI scores prior to treatment with efalizumab were also noted. For patients who experienced rebounds with generalized psoriasis, we noted the time that had elapsed since efalizumab discontinuation and the treatment they were receiving. RESULTS: Even though 92.8% of the patients were considered good responders (>75% reduction in PASI score), 25% of the group (8/32) experienced rebound and 15.7% (5/32) experienced relapse. The percentage of patients in whom rebound was observed on transition therapy was 18% (2/11) for cyclosporin, 50% (1/2) for methotrexate, 50% (1/2) for adalimumab, 50% (1/2) for etanercept, and 27% (3/11) for topical treatment. CONCLUSIONS: We observed a very high rate of rebound and generalized inflammation in patients whose disease had previously been well controlled for several years.

[Reduction of anxiety in the anteroom of the operating theater in pediatric patients]. / Reducción de la ansiedad en la antesala del quirófano en pacientes pediátricos.

Research indicates that anxiety before surgical intervention in paediatric patients negatively affects on post hospital recovery. Numerous investigations are, therefore, conducted to alleviate anxiety through different psychological techniques at this critical moment that the child is undergoing in surgery. In our case, we have considered one of the resources of the Sant Joan de Déu Hospital: a voluntary team that works in the anteroom of the operating theatre to keep the children company. The aim of our research has been to train these volunteers (who previously did not have any specific training for their task) and to observe the effect on the children's anxiety. The subjects of our study were 140 boys and girls between the ages of 0 and 18 years old. Results indicate that training not only produces significative changes on volunteers' behaviour, but also has resulted in the children's presurgical anxiety behaviour. In this way, we recommend that all children be accompanied by a trained volunteer.

Epidermolysis bullosa acquisita: diagnosis by fluorescence overlay antigen mapping and clinical response to high-dose intravenous immunoglobulin.

Epidermolysis bullosa acquisita (EBA) is a chronic, subepidermal blistering disease characterized by the presence of autoantibodies to type VII collagen, located below the lamina densa of the basement membrane zone (BMZ). There is a large clinical and histological overlap between EBA and other subepidermal autoimmune bullous diseases, therefore, complex immunological techniques are required to make an accurate diagnosis. Therapy of EBA is also a difficult issue. Most patients do not respond to several common immunosuppressive agents. We describe a patient who has shown a good response to high-dose intravenous immunoglobulin therapy.

[Erythrodermic lichen planus pemphigoides]. / Liquen plano penfigoide eritrodérmico.

Lichen planus pemphigoides is a clinico-histological subtype of lichen planus in which there are bullous lesions similar to those of bullous pemphigoid. Lichen planus is included among the rare causes of erythroderma. We present a case of erythrodermic lichen planus pemphigoides in a 49-year-old female patient who satisfactorily responded to treatment with cyclosporine. A detailed physical examination and immunofluorescence study are key to the diagnosis of lichen planus pemphigoides.