This guideline is a partial update of the S3 guideline on atopic dermatitis (AWMF register no. 013-027) published in 2023. The chapters on systemic therapy with biologics and Janus kinase inhibitors as well as the chapter on pregnancy, breastfeeding and family planning in the context of systemic therapies for atopic dermatitis have been updated. This was prompted by new approvals (lebrikizumab, nemolizumab), approval extensions (abrocitinib from 12 years of age, baricitinib from 2 years of age) and new evidence on the use of biologics before and during pregnancy. In addition, a new chapter on treatment goals, treatment expectations and criteria for treatment adjustment ("treat-to-target") in systemic therapies has been added to the guideline. This article only presents the updated and newly added chapters. The complete guideline is available on the AWMF website.

BACKGROUND AND OBJECTIVES:Skin diseases can greatly impair quality of life (QoL) of pediatric patients and their families. The Infants and Toddlers Dermatology Quality of Life questionnaire (InToDermQoL) is the first skin-generic instrument assessing QoL in children ≤ 4 years, as reported by their caregiver. This study aimed to psychometrically validate the German version of the InToDermQoL. PATIENTS AND METHODS:The psychometric properties of the InToDermQoL were examined in a sample of 148 children with various skin diseases (age groups [n]: < 1 [44]; 1-2 [68]; 3-4 [36]). RESULTS:Exploratory factor analysis suggested a unidimensional structure of the version for children < 1 year, and a two- and three-factor structure for the two older age groups, respectively. Internal consistency was excellent (Cronbach's alpha, 0.904-0.922). Two-week test-retest reliability was good, except in children aged 1-2 years (intraclass correlation coefficient [ICC], 0.430). The InToDermQoL demonstrated good convergent validity with other QoL questionnaires. CONCLUSIONS:The German InToDermQoL is a valid and reliable QoL measure in children ≤ 4 years with any skin disease. Application of the tool in research and clinical practice will contribute to a better understanding of the impact of pediatric dermatoses, and optimize care and support for affected families.

Infantile hemangiomas are benign vascular tumors that occur exclusively in infants and display a characteristic growth pattern. They usually become noticeable shortly after birth, proliferate over a period of 4-6 months, remain stable in size for a variable duration, and then gradually involute, although residual changes may persist. Diagnosis is typically made clinically, with careful attention to potential complications, functional impairments, and rare associated malformations such as those seen in PHACES and LUMBAR syndromes. A thorough understanding of the different types of hemangiomas, their clinical presentations, and the indications for further diagnostic evaluation is essential for optimal patient care. Therapeutically, propranolol has become established as an effective and safe first-line treatment for complicated infantile hemangiomas.

BACKGROUND:Prevention and management of allergic reactions at school are recognized public health concerns. We analyzed food-induced anaphylaxis (FIA) cases which occurred in education facilities to better understand their management. METHODS:FIA cases recorded by the European Anaphylaxis Registry which occurred in (pre-)school settings were descriptively analyzed in a comparative manner to pediatric FIA cases that occurred outside education facilities. RESULTS:Of the 3450 pediatric FIA cases, 332 (9.6%) occurred in education facilities (median age: 4 years [IQR: 2-9]). 151 (45.5%) children had a known allergy to the culprit food. The main culprit foods were peanut (27.4%), hazelnut (12.7%), and cow's milk (9.4%). According to Ring classification, 209 (63.0%) reactions were grade 2, 120 (36.1%) grade 3, and 3 (0.9%; 1 death) grade 4. Adrenaline injection was reported in 50/305 (16.4%) children. Adrenaline use increased from 8.1% to 21.1% of cases during the study period (not statistically significant, p = .68). Compared to children with a FIA outside education facilities, children who experienced FIA in preschool-school settings were more likely to have a known allergy to the culprit food (p <.001) and to have experienced a peanut-induced anaphylaxis (p = .04), and were less likely to receive adrenaline (p <.001). CONCLUSION:Anaphylaxis occurring in educational settings can be severe, with frequent known allergy to the culprit food, peanut as the most frequent elicitor, and adrenaline underuse. Action at the national policy level and cross-country collaborations are required to implement a common approach to protect children in educational settings.

Zusammenfassung Hintergrund und Zielsetzung Hauterkrankungen können die Lebensqualität ( quality of life ; QoL) von pädiatrischen Patienten und ihren Familien erheblich beeinträchtigen. Der Fragebogen zur Lebensqualität in der Dermatologie bei Säuglingen und Kleinkindern ( Infants and Toddlers Dermatology Quality of Life questionnaire ; InToDermQoL) ist das erste hautgenerische Instrument zur Bewertung der QoL bei Kindern ≤ 4 Jahren, wie sie von ihren Bezugspersonen angegeben wird. Ziel dieser Studie war es, die deutsche Version des InToDermQoL psychometrisch zu validieren. Patienten und Methodik Die psychometrischen Eigenschaften des InToDermQoL wurden an einer Stichprobe von 148 Kindern mit verschiedenen Hauterkrankungen untersucht (Altersgruppen [n]: < 1 Jahr [44]; 1–2 Jahre [68]; 3–4 Jahre [36]). Ergebnisse Die explorative Faktorenanalyse ergab eine eindimensionale Struktur der Version für Kinder < 1 Jahr und eine Zwei‐ beziehungsweise Dreifaktorenstruktur für die beiden älteren Altersgruppen. Die interne Konsistenz war ausgezeichnet (Cronbachs Alpha: 0,904–0,922). Die Test‐Retest‐Reliabilität über zwei Wochen war gut, mit Ausnahme der 1‐ bis 2‐jährigen Kinder (Intraklassenkorrelationskoeffizient; ICC = 0,430). Der InToDermQoL zeigte eine gute konvergente Validität mit anderen QoL‐Fragebögen. Schlussfolgerungen Der deutsche InToDermQoL ist ein valides und zuverlässiges Instrument zur Messung der Lebensqualität von Kindern ≤ 4 Jahren mit Hauterkrankungen. Der Einsatz des Instruments in Forschung und klinischer Praxis wird zu besserem Verständnis der Auswirkungen pädiatrischer Dermatosen beitragen und die Versorgung und Unterstützung betroffener Familien optimieren.

BACKGROUND AND OBJECTIVES:The relationship between atopic dermatitis (AD), weight, height, and body mass index (BMI) in children and adolescents and the impact of systemic treatments is controversial. We report the distribution of weight, height, and BMI in the German TREATkids cohort compared to a standardized German cohort (Kromeyer-Hauschild) and the impact of systemic glucocorticoids. PATIENTS AND METHODS:This multicenter, prospective study analyzed weight and height data from pediatric patients (2-17 years) with moderate-to-severe AD enrolled in TREATkids. RESULTS:According to Kromeyer-Hauschild metrics, the median height, weight, and BMI of the TREATkids cohort were 42nd, 52nd, and 59th percentiles, respectively. A height deficit was observed compared to the reference population. Despite shorter stature, the children exhibited weight percentiles comparable to the general population. This combination of reduced height and normal weight led to high BMI-for-age percentiles. A sensitivity analysis excluding patients who had received systemic corticosteroids showed similar results for height-for-age, weight-for-age, and BMI-for-age percentiles. CONCLUSIONS:Children and adolescents with moderate-to-severe AD in TREATkids exhibit distinct anthropometric patterns, characterized by height deficits but normal weight distribution, independent of systemic glucocorticoid treatment.

Real-world evidence on clinical and molecular outcomes of systemic therapy for pediatric atopic dermatitis remains limited. Within the prospective TREATkids registry, we conducted an observational analysis of children and adolescents treated with dupilumab in routine care. Baseline data from 200 and follow-up data from 124 patients were evaluated for clinician- and patient-/caregiver-reported outcomes, alongside epidermal proteomic profiling using tape strips and the Olink Explore Inflammation 384 (n = 20) panel and 16S ribosomal RNA gene sequencing for skin microbiome assessment in subsets (n = 48). At treatment initiation, disease burden was high (mean Eczema Area and Severity Index = 16.5, objective SCORing Atopic Dermatitis = 44.9, peak pruritus numerical rating scale = 6.6). By month 3, Eczema Area and Severity Index 50, 75, and 90 response rates were 87, 60, and 30%. Response rates at months 6 and 12 were generally consistent with those observed at month 3, with no discontinuations and conjunctivitis occurring in 4.0%. Proteomic analyses demonstrated marked baseline upregulation of alarmins, T helper 2 chemokines, and tissue-remodeling markers in lesional skin, followed by downregulation of 144/161 dysregulated proteins at month 3, including CCL17/TARC, CXCL8, IL-6, IL-18, and matrix metalloproteinases. Microbiome profiling showed baseline dysbiosis with Staphylococcus aureus overabundance and reduced α-diversity, normalizing toward a nonlesional-like state after therapy at month 3. Overall, dupilumab was associated with rapid, sustained clinical and molecular improvement.

Parents of children with skin conditions face an additional caregiving burden and significant health-related quality of life (HRQoL) impairments. This study aimed to (1) test the psychometric properties of the German version of the Family Dermatology Life Quality Index (F-DLQI) in parents of infants and toddlers with any dermatological diagnosis and (2) examine the associations between the parents’ and their children’s HRQoL. Parents (n = 126) of 0- to 4-year-old children with any skin disease filled in the F-DLQI and an instrument assessing their children’s HRQoL, the Infants and Toddlers Dermatology Quality of Life questionnaire (InToDermQoL). The attending physician provided clinical information. Internal consistency, 2-week test–retest reliability, measurement error, and known-groups validity across severity levels of the F-DLQI were examined. Associations between the parents’ and their children’s HRQoL were tested using regression analysis. Internal consistency and test–retest reliability of the F-DLQI were good (Cronbach’s α = 0.896, ICC = 0.867), and construct validity was confirmed. Parents’ HRQoL (F-DLQI) and children’s current complaints as reported by their parents, but not physician-rated disease severity, were associated with children’s HRQoL. Conclusion: The German version of the F-DLQI is a valid and reliable tool in measuring the HRQoL of parents of children ≤ 4 years with dermatological conditions. The majority of the sample had atopic dermatitis, limiting the generalizability of the findings. Future studies need to evaluate its structural validity and responsiveness. Burden experienced by parents should be taken into account by care providers to prevent parental stress from becoming chronic and negatively affecting the child’s HRQoL.

Die Mastozytose umfasst eine Gruppe klonaler Mastzellerkrankungen, die durch die Vermehrung und Akkumulation atypischer Mastzellen in Organen wie Haut, Knochenmark und Gastrointestinaltrakt gekennzeichnet ist. Sie wurde erstmals 1869 als Hautmanifestation beschrieben, während die systemische Form (SM), die auch weitere Organe betrifft, erstmals 1949 dokumentiert wurde. Man unterscheidet die indolente SM (ISM), bei der die Mediatorsymptomatik im Vordergrund steht, von aggressiveren Formen, bei denen die Dysfunktion betroffener Organe dominiert. Die Aufklärung der Pathogenese, die Klassifikation und das Management der Mastozytose entwickelten sich in den letzten Jahrzehnten kontinuierlich, unterstützt durch spezialisierte Netzwerke wie das Europäische Kompetenznetzwerk für Mastozytose (ECNM) und das Kompetenznetzwerk Mastozytose. Ein wesentlicher Fortschritt in der Therapie ist die Entwicklung zielgerichteter Tyrosinkinase-Inhibitoren, wie Midostaurin und Avapritinib, die seit einigen Jahren zur Behandlung der aggressiveren Formen der SM zur Verfügung stehen. Avapritinib wurde zuletzt auch für die Behandlung von ISM-Patienten mit mittelschweren bis schweren Symptomen zugelassen. Zusätzlich und für Patienten mit leichteren Formen der ISM wird eine symptomorientierte Basistherapie empfohlen. Die Diagnose der SM erfordert eine interdisziplinäre Zusammenarbeit und umfasst die Erfüllung diagnostischer Kriterien. Neue patientenzentrierte Ansätze wie die MASTHAVE®-App unterstützen die Verlaufskontrolle und können zur Verbesserung der Lebensqualität beitragen. Langfristig zielen Forschungsanstrengungen auf personalisierte Therapien ab, die molekulare Mechanismen der Erkrankung adressieren. Zitierweise: Siebenhaar F, Brehler R, Christen D, Hartmann K, Altrichter S, Joest M, aufm Kampe K, Lang C, Lippert U, Mülleneisen N, Ott H, Panse J, Pyatilova P, Schmid-Grendelmeier P, Staubach P, Röseler S, Ruëff F, von Bubnoff D, von Bubnoff N, Wagner N, Zuberbier T, Maurer M, Klimek L, Brockow K. Mastocytosis in the age of precision medicine - Position paper of the German Society of Allergy (AeDA) on the management of indolent systemic mastocytosis. Allergo J Int 2025;34:57-68 https://doi.org/10.1007/s40629-025-00327-x

Atopic dermatitis is the most common chronic condition in childhood and imposes a considerable burden on both children and their families. The German registry TREATgermany/kids systematically collects routine care data on children, adolescents, and adults with atopic dermatitis

Introduction: Netherton syndrome (NS; OMIM#256500) is a rare and severe disorder of epidermal maturation and keratinization caused by pathogenic variants in the serine protease inhibitor Kazal type 5 (SPINK5), leading to severe skin barrier impairment. Although effective treatment is crucial for NS patients, there is a lack of knowledge on what the best treatment options are for these patients. Large heterogeneity in reported outcomes and measurement instruments hinders accurate comparison of treatment results across studies and the development of a treatment guideline. Therefore, we aimed to develop a core outcome set (COS) for NS that can be used in clinical care and research. Methods: This study was performed in accordance with the recommendations of the Core Outcome Measures in Effectiveness Trials (COMET) initiative. After identification of outcomes through a literature search and classification based on the International Classification of Functioning and taxonomies published by the COMET initiative, discussion groups were organized at the 2nd International Netherton Congress 2022 to finalize the provisional outcome list. Through a 2-round e-Delphi, 41 stakeholders (patients and family members, professionals, and representatives of industry) from 14 countries rated the importance of the outcomes using a 9-point Likert scale. An online consensus meeting attended by 14 stakeholders finalized the COS. Results: The COS for NS comprised 21 outcomes in 10 domains. These included four “skin” outcomes, two “sensation” outcomes, two “side-effects of treatment” outcomes, one “vitality” outcome, one “emotional functioning” outcome, two “physical development” outcomes, two “nutrition” outcomes, two “infections” outcomes, two “allergies” outcomes, and three “assessment results” outcomes. Conclusion: In this study, consensus was reached on 21 outcomes to be included in the COS for NS. The selection of outcomes in the COS underlines that NS not only affects the skin but is a disease requiring a broad multidisciplinary approach in clinical care and research. International implementation of this COS will lead to more uniform reporting, thereby enabling comparison of study results, which may facilitate future treatment guideline development. The next step is to further conceptually define the outcomes and reach consensus on how to measure these.

In 2019, a group of experts published the first European guidelines for the management of congenital ichthyoses after a multidisciplinary expert meeting held in 2016. An update of these guidelines and literature search was planned every 5 years, given the clinical, molecular and therapeutic advances, including the use of biologic therapies. We present here updated guidelines that have been developed by a reorganized multidisciplinary group of international experts after a systematic review of recent literature, discussions and consensus reached at an expert conference held in June 2023. The guidelines provide summarized evidence and expert-based recommendations that aim to guide clinicians in the management of these rare and often complex diseases. These guidelines consist of two sections. Part one is reported elsewhere. Here, Part two covers the management of complications (eye, ear-nose-throat, pruritus, pain, cutaneous infections, vaccinations, growth failure and nutritional deficiency, hair and nail anomalies, reaction to hot and cold climates, physical limitations, comorbidities) and the particularities of the neonatal period and Netherton syndrome.

Background Congenital ichthyoses comprise a heterogeneous group of genetic diseases that require lifelong treatment and have a major impact on patients' quality of life. Conventional treatments reduce scaling and skin discomfort; however, they usually have little or no effect on erythema and pruritus. The identification of cytokine alterations in congenital ichthyoses has raised the possibility of repurposing currently available biologics. Several case reports have reported success with different biologics.Objectives To report the real-life effects of biologics on congenital ichthyoses.Methods This was a retrospective observational international multicentric study of patients with congenital ichthyoses treated with at least one biologic for a minimum of 3 months. The effect of the biologics was evaluated using an Investigator Global Assessment for change (IGA-C) scale. A comprehensive literature search was performed in parallel.Results Ninety-eight patients were included [mean (SD) age of 19.7 years, 50 female patients]. Patients with Netherton syndrome (NS) or congenital ichthyosiform erythroderma (CIE) represented the majority of patients (30% and 21%, respectively). Most patients (85%) had a severe or very severe form of congenital ichthyoses. The most frequently used biologics were inhibitors targeting interleukin (IL)-17, IL-12/IL-23 or the IL-4 receptor (IL-4R). The mean (SD) duration of treatment was 22.1 (20.1) months. There were 45 responders (46%), including 18 (18%) who were good responders; all had a subset of erythrodermic congenital ichthyoses and received one of the three main biologics. In patients with NS and CIE, IL-12/IL-23 and IL-4R inhibitors tended to be most effective. The literature review revealed a shorter mean (SD) duration of biologic treatment [11.5 (8.5) months] and higher percentage of responders (86%), suggesting reporter bias.Conclusions This series identified subsets of congenital ichthyoses that may respond to biologics and will help with the design of future clinical trials of biologics for congenital ichthyoses. We report the real-life effects of biologics in 98 patients with congenital ichthyosis. In parallel, we also performed a comprehensive literature search. The majority of patients had Netherton syndrome or congenital ichthyosiform erythroderma. The most commonly used biologics targeted IL-17, IL-12/IL-23 or IL-4R. Mean treatment duration was 22 months. Forty-six per cent responded to treatment, including 18% who responded 'very well'. These good responders had an erythrodermic form of congenital ichthyosis. The literature review revealed a shorter mean duration of use of biologics and a higher percentage of responders (86%). This series better identifies the subset of patients with congenital ichthyosis who may respond to biologics. It will help with the design of future clinical trials of biologics in congenital ichthyosis.

BACKGROUND:Food allergies are a major health concern with rising prevalence. Dietary habits are changing, and information about cashew-induced anaphylaxis is limited. METHODS:Cases of tree nut-induced anaphylaxis (TIA) registered from 2007 until April 2024 were extracted from the European Anaphylaxis Registry and analyzed. RESULTS:1389 cases of TIA out of 5945 registered food-induced reactions (23%) were identified. 1,083 cases with confirmed elicitor status, including 845 children (median age 4 years, 61% male) and 238 adults (38 years, 40% male), were selected for further analysis. The most frequent elicitors among children were cashew (n = 334), hazelnut (n = 211) and walnut (n = 146). The proportion of cashew-induced anaphylaxis increased from 2007 to 2024, and reactions were frequently caused by small amounts (< 1 teaspoon). Adults reacted frequently to hazelnut (n = 105), walnut (n = 47) but also almond (n = 35) and to higher amounts. Potential cofactors were present in 50% of the adult patients and 17% of children. The reaction severity was age-independent, and only a minority of patients was previously aware of their allergy (children 23%, adults 21%). The use of adrenaline was low in lay treatment (children 13%, adults 3%) and reached approximately 40% upon professional treatment. CONCLUSION:Cashew is an increasing, relevant allergen leading to anaphylaxis and is now the most frequent cause of TIA among children. These findings highlight the need for effective prevention and treatment measures. Almond was a frequent elicitor among adults and should be further monitored. The acute management requires improvement to comply with current guidelines.