etherton syndrome (NS) is a rare autosomal recessive skin disorder caused by mutations in SPINK5 (OMIM 605010), leading to severely impaired skin barrier function. Patients are at risk for complications such as hypernatremic dehydration, impaired thermoregulation, failure to thrive, and sepsis. Skin infections are common, as are allergies, asthma, and increased levels of circulating eosinophils and total IgE. 1,2 Affected individuals have lifelong ichthyosis lineariz circumflexa, associated with erythroderma, and pruritus. 3 Treatment of NS is difficult, with the mainstay being bland emollients. Topical corticosteroids and calcineurin in-hibitors may provide short-term benefit, but the former have a high risk of increased transcutaneous absorption and the latter have a high risk of resultant Cushing syndrome and immune suppression. As for systemic agents, low-dose retinoids, infliximab, and intravenous immunoglobulins have been tried with variable success. [4][5][6][7] Recent studies showed that NS shares an immune profile similar to psoriasis with helper T cell (T H ) 17/interleukin 23 (IL-23) and IL-17/tumor necrosis factor (TNF) synergistic activation. 8-10 Herein we report our experience using the IL-17A antagonist secukinumab in patients with NS. IMPORTANCENetherton syndrome (NS) is a rare, severe genetic disorder of cornification with high morbidity. Treatment for NS has been notoriously difficult. Recent studies showed an upregulated helper T cell (T H ) 17/interleukin 23 (IL-23) pathway in NS, suggesting the possibility of treatment strategies that target IL-17. OBJECTIVE To evaluate the clinical response of NS to treatment with the IL-17 antagonist secukinumab.

We report the case of a 10-year-old girl with bullous Sweet syndrome, recalcitrant to high-dose systemic corticosteroids. Subsequent treatment with infliximab resulted in a rapid improvement in cutaneous lesions and systemic symptoms. Cutis laxa was noted in the healed skin. We propose early second-line treatment with infliximab in children with steroid-refractory Sweet syndrome. Pediatric Dermatology KNÖPFEL Et aL. therapy for SS and typically lead to rapid resolution of systemic symptoms and skin lesions. There are little published data on second-line therapeutics when corticosteroids fail. 2,3 The majority of pediatric SS occurs after a nonspecific transient gastrointestinal or upper respiratory infection; however, it can be associated with inflammatory bowel disease. Up to 30% of children with SS will have a hematologic malignancy. 3 In our patient, an upper respiratory tract infection was the most likely trigger of SS, with the clinical presentation of bullous, ulceronecrotic plaques leading to postinflammatory cutis laxa. Despite immediate high-dose corticosteroid therapy, our patient had persistent disease activity. Drugs such as potassium iodide, colchicine, indomethacin, clofazimine, cyclosporine, and dapsone have been used for steroid-refractory SS with variable benefit. 2,4 More recently, the use of interleukin-1 receptor and tumor necrosis factor alpha (TNF-α) antagonists has been reported. 4 Our patient showed a very rapid response to infliximab supporting the hypothesis that TNF-α blockade effectively suppresses acute neutrophil activation. 4 Postinflammatory cutis laxa following SS can be associated with cardiovascular elastolysis. 5 Thus, cardiac screening and early and effective treatment are warranted in such cases. Based on our observation, we propose infliximab as an early second-line treatment in children with steroid-refractory SS.

To support caregivers in their situation it is advisable to assess the functional daily routine of caregivers. Their need for time off their household and caring duties and their informational and educational needs to pertaining to disease progression, possible sources of support and symptom management should be recognised. Further inquiries into caregiver's involvement and responsibilities in the discharge process are needed.

Aim: Nurse-led care aims to optimize the discharge preparation with a focus on increasing patients' independency and self-care abilities. This study compared patients' improvements of self-care abilities and frequency of readmission rate between nurseled care and regular nursing care within the acute hospital setting.Design: A quasi-experimental design within a real-world setting was used for this work. Methods:We included a pool of 2501 patients from a control group (medically stable in usual care) and 420 patients from an intervention group (nurse-led care). After propensity score matching, the study cohort consisted of 612 patients. Results:From admission to discharge, nurse-led care patients showed superior improvements of total self-care abilities compared to usual care patients. In particular, we found improvements in the following categories: mobility, grooming and excretion.Patients with nurse-led care were furthermore less frequently readmitted to hospital compared with the control group patients.

Zusammenfassung. Hintergrund: Die atopische Dermatitis (AD) ist eine chronische, rezidivierende Hauterkrankung. Jedes fünfte bis sechste Kind ist in der westlichen Zivilisation davon betroffen. Die Folgen der Erkrankung können gravierend sein: Chronische Entzündung der Haut und plagender Juckreiz verbunden mit Schlafstörungen. Für den Erfolg der Therapie ist die Edukation der Eltern von betroffenen Kindern zentral. Ziel: Entwicklung und Evaluation von evidenzbasierten Edukationsvideos für Eltern von einem Kleinkind mit AD. Methode: In den Videos wurde die Methode Storytelling eingesetzt. Ziel von Storytelling ist, Informationen durch den Einsatz von Geschichten einfach zu vermitteln. Anschließend an die Entwicklung wurden die Videos hinsichtlich der Verständlichkeit, der Wichtigkeit sowie der Nützlichkeit der Inhalte von einem multi-professionellem Team sowie von Familien mit betroffenen Kindern evaluiert. Ergebnisse: Es entstanden insgesamt sechs Videos, welche Themen wie Ursachen der AD, Symptome, Hautpflege, Behandlung sowie Leben mit AD beinhalteten. Storytelling wurde umgesetzt indem zwei Familien mit betroffenen Kindern von ihren Erfahrungen mit der Erkrankung erzählten. Fachinformationen wurden durch Spezialistinnen vermittelt. Die Evaluation ergab, dass die Informationen in den Videos einfach verständlich und relevant sind. Schlussfolgerungen: Mit evidenzbasierten Videos kann die Edukation auf innovative, kreative und moderne Art und Weise gefördert werden. Storytelling ist eine nutzerfreundliche Methode um komplexe Informationen einfach und verständlich zu vermitteln.