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Hintergrund: Bisher fehlen robuste epidemiologische Kennzahlen sowie Daten zur Komorbidität bei Kindern mit Urtikaria. Sie bilden die Grundlage zur Gestaltung einer effizienten Gesundheitsversorgung. Methodik: Retrospektive Studie zur Analyse von epidemiologischen Kennzahlen bei Kindern mit Urtikaria. Grundlage der Analyse sind Routinedaten einer deutschlandweit tätigen Krankenkasse (DAK-Gesundheit). Versicherte unter 18 Jahren, die in den Jahren 2010-2015 mindestens eine ambulant oder stationär gestellte Urtikaria-Diagnose gemäß der ICD-10-Klassifikation erhielten, wurden in die Analysen aufgenommen und mit Kindern ohne entsprechende Diagnose verglichen. Ergebnisse: Von 2,3 Millionen Versicherten waren 313 581 (13,5 %) unter 18 Jahre (153 214 weiblich). Bei 1,7 % der 313 581 Patienten wurde Urtikaria diagnostiziert. Die administrative Prävalenz der Urtikaria nahm mit zunehmendem Alter von 3,0 % in der Altersgruppe der 0-3-Jährigen auf 1,0 % bei den 14-18-Jährigen ab. Jungen und Mädchen waren in allen Altersgruppen fast gleich häufig betroffen. Atopische Erkrankungen als Komorbidität traten bei Kindern mit Urtikaria häufiger auf als in der Kontrollgruppe (16,0 % vs. 8,0 %). Auch Autoimmunerkrankungen, psychische Probleme und Adipositas traten bei Kindern mit Urtikaria häufiger auf als in der Kontrollgruppe. Schlussfolgerungen: Die erhöhte administrative Prävalenz spezifischer Komorbidität bei Kindern mit Urtikaria deutet auf einen erhöhten Screening-Bedarf hin. Es gilt, multimodale Behandlungsstrategien zu entwickeln und interdisziplinäre Zusammenarbeit zu fördern.

Treatment of patients with systemic autoimmune and/or autoinflammatory diseases (AI/AInf) often requires multidisciplinary collaboration of various medical specialties.We evaluated whether the establishment of a multidisciplinary board, which we termed rheuma board (RB), can contribute to optimization of care for patients with psoriatic arthritis (PsA) or other AI/AInf.A total of 272 patients were included in the study. Patients were divided into three groups-group 1: 41 patients with or with suspected PsA, initially assessed in the dermatology department and afterwards presented for consultation in the rheumatology department; group 2: 166 patients with or with suspected PsA presenting in the dermatology department and afterwards discussed by RB; group 3: 65 patients with other AI/AInf presenting in the dermatology department and afterwards discussed by RB. We evaluated the average duration from initial presentation to therapy initiation after completing evaluation and diagnostics by both specialties. In addition, diagnosis confirmation/verification and therapy continuation/optimization were analyzed for all three groups.The average duration from initial presentation until therapy initiation was 85 ± 42.24 (5-173) days in group 1, 15 ± 13.09 (0-78) days in group 2, and 20 ± 16.71 (1-75) days in group 3. In addition, in groups 2 and 3 confirmation of diagnosis was faster and waiting time for diagnosis and therapy initiation was significantly reduced.Establishment of a RB results in a significant reduction in the time duration between first presentation and initiation of therapy, and an improvement of care for patients with AI/AInf including confirmation of diagnosis and therapy optimization.HINTERGRUND: Patienten mit systemischen Autoimmun- und/oder autoinflammatorischen Erkrankungen (AI/AInf) bedürfen in der Regel einer multidisziplinären Zusammenarbeit durch verschiedene Fachrichtungen.Wir evaluierten, ob die Etablierung eines multidisziplinären Boards (sog. Rheumaboard [RB]) zur Optimierung der Versorgung von Patienten mit Psoriasisarthritis (PsA) oder anderen AI/AInf führt.Es wurden n = 272 Patienten mit AI/AInf eingeschlossen, die in 3 Gruppen eingeteilt wurden; Gruppe 1: 41 Patienten mit oder mit Verdacht auf (V. a.) PsA, von der Dermatologie in der Rheumatologie konsiliarisch avisiert; Gruppe 2: 166 Patienten mit oder mit V. a. PsA, vorstellig in der Dermatologie und im RB; Gruppe 3: 65 Patienten mit anderen AI/AInf, vorstellig in der Dermatologie und im RB. Evaluiert wurde die durchschnittliche Zeit von der initialen Vorstellung bis zur Therapieeinleitung nach erfolgter Beurteilung und Diagnostik durch beide Fachrichtungen. Darüber hinaus wurden die Diagnosesicherung/-bestätigung und die Therapieweiterführung/-optimierung bei allen 3 Gruppen analysiert.Die durchschnittliche Zeitspanne von der initialen Vorstellung bis zur Therapieeinleitung betrug in Gruppe 1 85 ± 42,24 (5 bis 173) Tage, in Gruppe 2 15 ± 13,09 (0 bis 78) Tage und in Gruppe 3 20 ± 16,71 (1 bis 75) Tage. In Gruppe 2 und 3 konnte die Diagnose schneller gesichert oder bestätigt sowie die Wartezeit auf Diagnostik und Therapie deutlich reduziert werden.Durch die Etablierung eines RB zeigt sich eine signifikante Verkürzung der Zeitspanne zwischen Erstvorstellung und Therapieeinleitung und damit eine deutliche Verbesserung des Versorgungsmanagements bei Patienten mit AI/AInf inklusive Diagnosesicherung und Therapieoptimierung.

Background To date, robust epidemiological metrics as well as data on comorbidity in pediatric urticaria are lacking. They form the basis for the design of efficient healthcare. Methods Retrospective study to analyze epidemiological data in pediatric urticaria. The analysis is based on routine data of a health insurance company operating throughout Germany (DAK-Gesundheit). Insured people under 18 years of age who received at least one confirmed outpatient or inpatient urticaria diagnosis according to the ICD-10 classification in the years 2010 to 2015 were included in the analysis and compared to children without a corresponding diagnosis. Results Of 2.3 million insured individuals, 313,581 (13.5 %) were under 18 years of age (153,214 female). Urticaria was diagnosed in 1.7 % of the 313,581 patients. The prevalence of urticaria decreased with age from 3.0 % in the 0-3-year age group to 1.0 % in the 14-18-year age group. Boys and girls were almost equally affected in all age groups. Atopic diseases as comorbidity occurred more frequently in children with urticaria than in the control group (16.0 % vs. 8.0 %). Autoimmune diseases, mental health problems, and obesity also occurred more frequently in children with urticaria than in the control group. Conclusions The increased prevalence of specific comorbidities in children with urticaria suggests an increased need for screening. Multimodal treatment strategies need to be developed and interdisciplinary collaboration promoted.

Zusammenfassung Hintergrund Patienten mit systemischen Autoimmun- und/oder autoinflammatorischen Erkrankungen (AI/AInf) bedürfen in der Regel einer multidisziplinären Zusammenarbeit durch verschiedene Fachrichtungen. Ziel der Arbeit (Fragestellung) Wir evaluierten, ob die Etablierung eines multidisziplinären Boards (sog. Rheumaboard [RB]) zur Optimierung der Versorgung von Patienten mit Psoriasisarthritis (PsA) oder anderen AI/AInf führt. Material und Methoden Es wurden n = 272 Patienten mit AI/AInf eingeschlossen, die in 3 Gruppen eingeteilt wurden; Gruppe 1: 41 Patienten mit oder mit Verdacht auf (V. a.) PsA, von der Dermatologie in der Rheumatologie konsiliarisch avisiert; Gruppe 2: 166 Patienten mit oder mit V. a. PsA, vorstellig in der Dermatologie und im RB; Gruppe 3: 65 Patienten mit anderen AI/AInf, vorstellig in der Dermatologie und im RB. Evaluiert wurde die durchschnittliche Zeit von der initialen Vorstellung bis zur Therapieeinleitung nach erfolgter Beurteilung und Diagnostik durch beide Fachrichtungen. Darüber hinaus wurden die Diagnosesicherung/-bestätigung und die Therapieweiterführung/-optimierung bei allen 3 Gruppen analysiert. Ergebnisse Die durchschnittliche Zeitspanne von der initialen Vorstellung bis zur Therapieeinleitung betrug in Gruppe 1 85 ± 42,24 (5 bis 173) Tage, in Gruppe 2 15 ± 13,09 (0 bis 78) Tage und in Gruppe 3 20 ± 16,71 (1 bis 75) Tage. In Gruppe 2 und 3 konnte die Diagnose schneller gesichert oder bestätigt sowie die Wartezeit auf Diagnostik und Therapie deutlich reduziert werden. Diskussion Durch die Etablierung eines RB zeigt sich eine signifikante Verkürzung der Zeitspanne zwischen Erstvorstellung und Therapieeinleitung und damit eine deutliche Verbesserung des Versorgungsmanagements bei Patienten mit AI/AInf inklusive Diagnosesicherung und Therapieoptimierung. Graphic abstract

Basal cell carcinoma (BCC) is the most common malignant tumor among fair-skinned individuals, and its incidence had been steadily rising in the past decades. In order to maintain the highest quality of patient care possible, the German S2k guidelines were updated following a systematic literature search and with the participation of all professional societies and associations involved in the management of the disease. Part 2 addresses issues such as proper risk stratification, the various therapeutic approaches, and prevention as well as follow-up of patients with basal cell carcinoma.

This is a retrospective study of 18 children with chronic spontaneous urticaria (CSU), where standard therapies, including up-dosing of antihistamines and omalizumab, were unable to cure the disease and where alternative strategies with experimental and off-label medication had to be used. Being aware that our questionnaire is validated only for elder children or adults, we utilized the UAS7 to monitor disease control with the help of the parents. The UAS7 score decreased from a mean of 25 to an average of 13 after 8 weeks of therapy in 13 patients. Five patients had no significant reduction of UAS7 by week 8. In two of five patients, where periodic improvement was seen, omalizumab therapy was continued and showed complete response after 1–2 years. In three children, alternative treatments with cyclosporine and dupilumab, approved by the ethics committee, showed symptom improvement from a mean UAS7 of 29 to a mean value of 6. When omalizumab therapy including up-dosing strategies or shortened interval shows no symptom improvement, alternative therapies, sometimes off-label have to be considered in time.

Basal cell carcinoma is the most common malignant tumor among fair-skinned individuals, and its incidence has been rising steadily in the past decades. In order to maintain the highest quality of patient care possible, the German S2k guidelines were updated following a systematic literature search and with the participation of all professional societies and associations involved in the management of the disease. Part 1 highlights new developments in genetics in particular as well as aspects regarding epidemiology, diagnosis, and histology.

Background Extemporaneous formulations broaden the spectrum of therapeutic options for topical treatment in particular and thus improve patient care. The latest amendment to the Regulation on the Operation of Pharmacies issued in 2012 brought about changes in prescribing and manufacturing practices. The aim of the present study was to assess the relevance of extemporaneous formulations in everyday clinical practice. Methods We used data from the German Institute for Drug Use Evaluation (DAPI) to analyze the prescribing practice for compounded preparations in Germany between the fourth quarter of 2011 and the third quarter of 2014. In doing so, we determined the total cost associated with extemporaneous formulations covered by statutory health insurance funds in the outpatient setting. Results Approximately three out of ten prescriptions (30.54 %) by German dermatologists during the observation period were extemporaneous formulations. While dermatologists make up only 2.7 % of physicians working in the statutory health care system in Germany, they prescribe more than half of all compounded preparations (53.6 %). Each dermatologist prescribed an average of 270.4 formulations per quarter; that number was 13.5 (1.3 %) for all other medical specialties. On average, 1,983,687 extemporaneous formulations overall (1.3 % of all prescriptions) were prescribed per quarter, corresponding to a total cost of € 40,944,982 (0.55 %). Conclusions Apart from finished medicinal products, extemporaneous formulations play a key role in outpatient care. Based on the principles of evidence-based and patient-oriented medicine, the quality of compounded preparations and the prescribing practice of physicians (standardized vs. individual formulations) should be further investigated to optimize the quality of these preparations.

Hidradenitis suppurativa (HS also named as acne inversa) is a chronic skin disease characterized by relapsing formation of abscesses, inflammatory nodules, and fistulas. In moderate-to-severe disease, HS leads to the formation of scarring and thus irreversible tissue destruction. In the past few years, two new treatment options became available: adalimumab, the first biologic therapy approved for HS (Humira®, AbbVie), and a noninvasive, device-based treatment utilizing a combination of intense pulsed light and radiofrequency (LAight® therapy, LENICURA, Germany). Here, we report a case of a Hurley stage III patient where the positive effect of adalimumab could be enhanced by simultaneously applying LAight® therapy. Moreover, long-term symptom control could be achieved under monotherapy with LAight® after adalimumab was terminated.

Background: Disseminated superficial actinic porokeratosis (DSAP) is a rare dermatologic disorder of the epidermis. Often misdiagnosed as chronic UV-damage or actinic keratoses, patients are treate...

Zusammenfassung Seit Jahrzehnten ist bekannt, dass Tumoren vom Immunsystem erkannt und zerstort werden konnen. Diese, vor allem in Tierversuchen gewonnene Erkenntnis konnte jedoch in der Vergangenheit nicht zum Nutzen unserer Patienten umgesetzt werden, da immunonkologische Therapieansatze in den letzten Jahrzehnten in der Anwendung beim Menschen stets versagt haben. Daher hat, mit Ausnahme der adjuvanten Interferontherapie, keines dieser Verfahren den Einzug in die klinische Versorgung gefunden. Langzeituberleben unter guter Lebensqualitat war dabei sehr wenigen Patienten vorbehalten. Mit den neuen immunologischen Therapieansatzen wird jedoch sowohl das Langzeituberleben als auch die Lebensqualitat onkologischer Patienten neu definiert. Auf die neuen „Immun-Checkpoint-Inhibitoren“ spricht erstmals ein relevanter Teil der behandelten Patienten an und diese zeigen in der Regel langandauernde Remissionen bis hin zur Heilung. Schon jetzt ist klar, dass die Immuntherapie in Zukunft eine der wesentlichen Therapiesaulen bei der Behandlung des metastasierten Melanoms und auch vieler anderer fortgeschrittener Tumoren bilden wird. In dieser Ubersicht werden die wichtigsten neuen Therapiemodalitaten besprochen und sowohl deren Wirkprinzip als auch klinische Daten zum Therapieansprechen und zu erwartenden Nebenwirkungen der Therapie referiert.

Summary It has been known for decades that the immune system is able to detect and destroy tumor cells. In the past, this knowledge – mostly acquired through animal experiments – could not be used to benefit our patients, because immuno-oncological therapeutic approaches in humans had constantly failed over recent decades. With the exception of adjuvant interferon therapy, none of these approaches had found its way into everyday clinical practice, and only very few patients were able to enjoy long-term survival associated with good quality of life. With the advent of novel immunological approaches, the meaning of long-term survival as well as quality of life has been redefined for oncological patients. For the first time, a significant percentage of patients responds to treatment with immune checkpoint inhibitors, showing long-term remission and even cure. It has already become apparent that immunotherapy will in the future be one of the therapeutic mainstays in the treatment of metastatic melanoma as well as many other tumor types. The present review article presents the most important new treatment modalities, their mechanism of action, clinical data regarding treatment response, and adverse events to be expected.

Despite new therapeutic options, metastatic melanoma remains to be one of the most fatal tumors. With the development of BRAF inhibitors and immune checkpoint inhibitors, overall survival could be prolonged significantly for the first time. Clinical studies implied that even long-term survival is possible with both types of drugs, but predictive markers are so far missing. In this study, we analyzed survival data from patients that received the first-in-class substances vemurafenib and ipilimumab, respectively, during the time period from registration of the drugs until availability of combination treatments. We aimed to evaluate the possibility of long-term survival in a daily life setting and to characterize patients that benefit from these drugs in order to gain insight into predictive attributes. Eighty patients were evaluated who were treated with either vemurafenib (n = 40) or ipilimumab (n = 40), and overall survival was analyzed. Subgroup analysis was performed for patients who were still alive 24 months after induction of therapy (long-term survival). Median overall survival (OS) was 8.0 months for patients treated with vemurafenib and 10.0 months for patients treated with ipilimumab (log-rank P value = 0.689). Long-term survival was achieved in 32.5% of patients (42.3% vemurafenib, 57.7% ipilimumab). Negative predictors of long-term survival in the vemurafenib group were brain and liver metastases, as well as elevated LDH, S100s and liver enzymes. For ipilimumab, an increase in lymphocytes and eosinophils during course of treatment correlated with long-term survival. Our real-life experience shows that long-term survival is possible with using both therapeutic agents, vemurafenib and ipilimumab. Pattern of metastases and laboratory values might be of interest in decision making for a specific therapeutic approach. Combination of drugs and observational studies in larger patient cohorts are necessary to further validate our findings.

It has been known for decades that the immune system is able to detect and destroy tumor cells. In the past, this knowledge - mostly acquired through animal experiments - could not be used to benefit our patients, because immuno-oncological therapeutic approaches in humans had constantly failed over recent decades. With the exception of adjuvant interferon therapy, none of these approaches had found its way into everyday clinical practice, and only very few patients were able to enjoy long-term survival associated with good quality of life. With the advent of novel immunological approaches, the meaning of long-term survival as well as quality of life has been redefined for oncological patients. For the first time, a significant percentage of patients responds to treatment with immune checkpoint inhibitors, showing long-term remission and even cure. It has already become apparent that immunotherapy will in the future be one of the therapeutic mainstays in the treatment of metastatic melanoma as well as many other tumor types. The present review article presents the most important new treatment modalities, their mechanism of action, clinical data regarding treatment response, and adverse events to be expected.

Seit Jahrzehnten ist bekannt, dass Tumoren vom Immunsystem erkannt und zerstört werden können. Diese, vor allem in Tierversuchen gewonnene Erkenntnis konnte jedoch in der Vergangenheit nicht zum Nutzen unserer Patienten umgesetzt werden, da immunonkologische Therapieansätze in den letzten Jahrzehnten in der Anwendung beim Menschen stets versagt haben. Daher hat, mit Ausnahme der adjuvanten Interferontherapie, keines dieser Verfahren den Einzug in die klinische Versorgung gefunden. Langzeitüberleben unter guter Lebensqualität war dabei sehr wenigen Patienten vorbehalten. Mit den neuen immunologischen Therapieansätzen wird jedoch sowohl das Langzeitüberleben als auch die Lebensqualität onkologischer Patienten neu definiert. Auf die neuen "Immun-Checkpoint-Inhibitoren" spricht erstmals ein relevanter Teil der behandelten Patienten an und diese zeigen in der Regel langandauernde Remissionen bis hin zur Heilung. Schon jetzt ist klar, dass die Immuntherapie in Zukunft eine der wesentlichen Therapiesäulen bei der Behandlung des metastasierten Melanoms und auch vieler anderer fortgeschrittener Tumoren bilden wird. In dieser Übersicht werden die wichtigsten neuen Therapiemodalitäten besprochen und sowohl deren Wirkprinzip als auch klinische Daten zum Therapieansprechen und zu erwartenden Nebenwirkungen der Therapie referiert.

e21615 Fear of progression in patients with low-risk malignant melanoma Fear of cancer progression (FoP) is one of the problems most commonly reported by cancer patients and is among the most prevalent cancer-related worries in cancer survivors. FCR is highly variable in different cancer entities. Background: This study aimed to elicit the prevalence and significance of FCR in melanoma patients with low risk tumors and further to assess psychosocial and demographic factors predicting severity of FoP as well as to determine the impact of FoP on quality of life (QoL). Methods: In total, 133 patients with low-risk melanoma (pT1a) completed the short version of the Fear of Progression Questionnaire (FoP-Q-SF), the Hospital Anxiety and Depression Scale (HADS), and the EQ-5D questionnaire, as well as self-constructed questions on the socio-demographic background. Results: MeanFoP-Q-SF sum score was 30.1 points (± 8.4 points SD) and the median 29 points. Two thirds of patients deviated not more than 1 SD from the mean. Risk factors for elevated FoP were female gender (p < 0.01), young age (p = 0.04) and working (p = 0.02) patients and patients with severe comorbidities (p = 0.03). Cancer in closely related persons predicted higher FoP (p < 0.01). HADS anxiety scale (r = 0.49, p < 0.01) and depression scale (r = 0.26, p < 0.01) correlated with FoP. A negative correlation with the EQ-5D index was found (r = -0.34, p < 0.01). Conclusions: As in many other malignancies,FoP is prevalent in low-risk melanoma patients and is associated with reduced QoL. Severe comorbidities, cancer in related persons, gender and income could be identified as predictors of FoP. Considerable high levels of FoP even in patients with low-risk malignancies underline the need of psychosocial support and intervention for special subgroups of melanoma patients to enable the patient to manage FoP more successfully.