Systemic treatments in paediatric psoriasis: a systematic evidence-based update.

In 2008, a systematic review revealed that evidence-based data on efficacy and safety of treatments in paediatric psoriasis are scarce and with low level of evidence. In recent years, publications on this topic have increased exponentially. To present a systematic, evidence-based update on the efficacy and safety of systemic treatments in paediatric psoriasis and to provide treatment recommendations, an update of the previous review was performed. PubMed, EMBASE and the Cochrane Controlled Clinical Trial Register were searched between January 2007 and March 2014 for all available literature on efficacy and safety of all systemic treatments in paediatric psoriasis. The levels of evidence were determined on the Oxford Centre for Evidence-based Medicine Levels of Evidence. The newly retrieved evidence was combined with the evidence available in the former review. Fifty-two studies were included: 36 from the former review, plus 16 new articles. New evidence on induction therapy was mainly available on fumaric acid esters (FAEs), which are shown to be effective in a subgroup of patients. Long-term (96 weeks) safety and efficacy data on etanercept were found. Prospective studies are scarce. Most conclusions are formulated on studies with low level of evidence. Of the conventional systemic treatments, methotrexate still has the most evidence albeit in a low number of patients and with a low level of evidence. FAEs seem to be effective in a subgroup of patients, with gastro-intestinal complaints, flushes and temporary shifts in leucocyte counts and liver enzymes being the main side-effects. Etanercept has still accumulated most evidence of the available systematic treatments, with a large efficacy and reassuring safety profile in a 96-week follow-up.

The influence of treatments in daily clinical practice on the Children's Dermatology Life Quality Index in juvenile psoriasis: a longitudinal study from the Child-CAPTURE patient registry.

BACKGROUND: Juvenile psoriasis has a negative effect on the quality of life (QoL). The influence of treatments on QoL of these children has never been investigated before in a prospective observational study. OBJECTIVES: To assess the Children's Dermatology Life Quality Index (CDLQI) in a cohort of patients with juvenile psoriasis and to evaluate the influence of treatments in daily clinical practice on CDLQI. METHODS: We conducted a prospective observational study of children with psoriasis from a registry containing daily clinical practice data. Before and after treatment, QoL was assessed by the CDLQI and disease severity was documented by the Psoriasis Area and Severity Index (PASI). Three clusters of treatments were analysed: topical, dithranol and systemic therapy. RESULTS: In total, 125 patients were enrolled in the registry. Cross-sectionally, a mean ± SD CDLQI score of 7·5 ± 5·0 and a mean ± SD PASI of 7·0 ± 5·8 were recorded. Itching and problems with treatment had the highest impact on the children's QoL. Longitudinally, 85 patients were analysed with a total of 137 treatment episodes. All treatments contributed to a significant decline in total CDLQI score, with the largest decrease seen in dithranol and systemic treatments. A significant correlation was found between ΔCDLQI and ΔPASI for all treatment modalities. The highest positive impact of treatments was found in a decline of itch and sleep disturbance. CONCLUSIONS: In this first prospective observational study on CDLQI in juvenile psoriasis, a positive influence of treatments in daily clinical practice on QoL was demonstrated.

Paediatric-onset psoriasis is associated with ERAP1 and IL23R loci, LCE3C_LCE3B deletion and HLA-C*06.

BACKGROUND: Recent genome-wide association studies have identified several genetic risk factors for psoriasis, but data on their association with age at onset are lacking. OBJECTIVES: To compare the association between known risk alleles and psoriasis in well-defined cohorts with paediatric- and adult-onset psoriasis. METHODS: Based on previous studies we selected seven genes and loci associated with psoriasis. Patients with paediatric-onset (< 18 years) and adult-onset psoriasis (≥ 18 years) and controls were genotyped. Genotype frequencies were compared between controls (n = 450) and all cases (n = 217), and between controls and cases stratified for confirmed age at onset (paediatric onset n = 80, adult onset n = 85). RESULTS: Paediatric-onset psoriasis showed a significant association with single nucleotide polymorphisms in the ERAP1 (P = 0.042) and IL23R loci (P = 0.042), LCE3C_LCE3B-del (P = 0.003) and HLA-C*06 (P = 1.72 × 10(-19)) when compared with the control group. A significant association of these four genes was also demonstrated when all psoriasis cases were compared with controls. In adult-onset psoriasis a significant association was found for HLA-C*06 (P = 5.11 × 10(-6)) and for LCE3C_LCE3B-del (P = 0.042). No associations were found for the IFIH1, IL12B and TRAF3IP2 loci. CONCLUSIONS: Notwithstanding the small cohort sizes, we demonstrated an association with established and recently discovered genetic risk factors in paediatric-onset psoriasis including genes involved in epidermal barrier function and adaptive immunity. Our data suggest that heritable factors may play a more important role in paediatric-onset psoriasis than in adult-onset psoriasis.

Juvenile psoriasis in European and Asian children: similarities and differences.

BACKGROUND: The first manifestations of psoriasis begin in childhood in more than one-third of patients. However, epidemiological data of juvenile psoriasis are lacking. OBJECTIVES: To compare Dutch (NL group) and Singaporean (SG group) children with psoriasis with the aim of studying the characteristics of juvenile psoriasis and to highlight similarities and differences between these different ethnic groups. METHODS: Data were collected from 207 patients younger than 18 years diagnosed with psoriasis from Radboud University Nijmegen Medical Centre, Nijmegen, the Netherlands and the National Skin Centre, Singapore. RESULTS: A striking difference in familial distribution was found, with more Dutch children having an affected family member (73·3% vs. 13·6%). Presence of itch and triggering factors were more common among Dutch children (80% vs. 14·2% and 33·3% vs. 7·4%, respectively). However, both groups shared similar triggering factors like stress and infections. Other similarities included mean age at presentation (NL group 11·3 years; SG group 14·1 years) and gender ratio (NL group, M/F 1 : 1·1; SG group, M/F 1 : 1·4). Plaque psoriasis was the most common type in both cohorts while guttate and pustular psoriasis were rare. In both groups, the head, followed by the limbs, was the most common site involved. Similar proportions of children in both countries had nail involvement and psoriatic arthritis was rare. CONCLUSIONS: The disparity in familial distribution may point to genetic differences between the two groups. Further studies to evaluate this difference in familial distribution may contribute to the understanding of the pathogenesis of psoriasis.

An intrapatient comparison of quality of life in psoriasis in childhood and adulthood.

BACKGROUND: Chronic diseases can have a great influence on health-related quality of life. Nevertheless, only little research has been carried out on childhood psoriasis. The perception of quality of life by adults with psoriasis of their childhood psoriasis has never been investigated. OBJECTIVES: The aims of this study were to (i) investigate retrospectively the influence of psoriasis as experienced in childhood as compared with the current quality of life in adulthood; (ii) assess retrospectively the impact of childhood psoriasis on daily life; and (iii) compare the current quality of life in patients with childhood onset psoriasis (COP) and adult onset psoriasis (AOP). METHODS: A survey was performed among all members of the Dutch Psoriasis Society. Validated questionnaires on quality of life, impact on daily life and clinical severity were used. RESULTS: Questionnaires of 1762 patients were suitable for analysis. Adults with an onset of psoriasis before the age of 18 years retrospectively rate their quality of life during childhood much less as compared with their current quality of life (intrapatient comparison). Influence of psoriasis in childhood particularly had a high degree of limitations on recreational and social activities in 15-30% of patients. Quality of life in adulthood is not determined by age of onset of psoriasis. CONCLUSIONS: In childhood, the quality of life is greatly influenced by psoriasis. The social development domain, which is one of the developmental milestones in a child, is particularly impaired. The current quality of life of patients with COP is equal to that of patients with AOP.

A cross-sectional study using the Children's Dermatology Life Quality Index (CDLQI) in childhood psoriasis: negative effect on quality of life and moderate correlation of CDLQI with severity scores.

BACKGROUND: Juvenile psoriasis is a chronic and incurable skin disease that affects approximately 0·7% of children. OBJECTIVES: To achieve more insight into the quality of life (QoL) in childhood psoriasis and to investigate whether disease severity scores correlate with QoL scores. METHODS: All consecutive patients with juvenile plaque psoriasis (≤ 18 years old) who visited our outpatient department were included. At baseline, the Children's Dermatology Life Quality Index (CDLQI) questionnaire was completed and disease severity was assessed by the Psoriasis Area and Severity Index (PASI) and the Physician Global Assessment (PGA). RESULTS: Thirty-nine patients were included in the study. A median CDLQI of 6 [interquartile range (IQR) 5­9] was reported. Median PASI was 6·3 (IQR 3·3­8·2) and median PGA was 2 (IQR 1­3). The correlation coefficient between PASI and CDLQI was 0·47 (P = 0·003), whereas the correlation coefficient between PGA and CDLQI was 0·51 (P = 0·001). CONCLUSIONS: The negative effect on QoL in juvenile psoriasis was confirmed in the largest cohort presented up to now. The correlation between disease severity scores and disease-related QoL in children with psoriasis is only moderate. Therefore, both clinical outcome parameters (PASI, PGA) and measures of QoL (CDLQI) should be included in adequate, patient-oriented clinical decision making.

Dithranol therapy in childhood psoriasis: unjustifiably on the verge of falling into oblivion.

BACKGROUND: In childhood psoriasis, physicians aim for an effective and safe treatment such as with dithranol. This study presents the largest study of dithranol-treated patients described in the literature. OBJECTIVE: The aim of the study was to determine the position of dithranol in the treatment strategy for psoriasis. METHODS: All juvenile patients receiving dithranol treatment at our center were evaluated retrospectively. RESULTS: Sixty patients (with 82 treatment episodes in total) were included. The mean age at the start of dithranol treatment was 11.1 years (range: 3.7-17.9 years). The result of the treatment was: excellent (3.7%), good (69.5%), moderate (8.5%), reasonable (13.4%) or disappointing (4.9%). Mild irritation was seen in 39%, and severe irritation in 63% of the patients. CONCLUSIONS: Dithranol can be regarded as an efficacious and safe topical therapy for the treatment of childhood psoriasis. It is a valuable alternative topical treatment which should not be disregarded in the treatment regimen for childhood psoriasis and should be commenced before ultraviolet or systemic treatments are initiated.

Genital psoriasis: a questionnaire-based survey on a concealed skin disease in the Netherlands.

BACKGROUND: Psoriatic lesions may involve nearly all sites of the body. Involvement of the genital skin is frequently classified as part of intertriginous psoriasis without special awareness and treatment for this presentation of the disease. Gaining knowledge about the frequency of the involvement of genital skin in these patients will improve the overall care for patients with psoriasis. OBJECTIVES: We studied the prevalence of genital psoriasis in the Netherlands and epidemiological characteristics of this specific presentation of the disease. Furthermore, we studied the relation between flexural and genital psoriasis. PATIENTS/METHODS: A self-administered questionnaire was sent to all 5300 members of the Dutch Psoriasis Society. Sociodemographic patient characteristics and disease-related data (such as localization of psoriatic lesions, involvement of the genitalia, age at onset of genital psoriasis and severity of genital psoriatic lesions) were collected and analysed. RESULTS: A response rate of 37% was achieved. Almost 46% of the responding patients with psoriasis, that is 16.5% of all potential responders (n = 5300), report genital involvement at some time during the course of their disease. The genitalia can become affected at any age. Many patients with current genital involvement (38%) do not have the flexural skin affected. CONCLUSIONS: A large part of patients with psoriasis suffer from genital psoriasis, which was not associated with flexural involvement in at least one third of them. More attention to the genital region is required in the current standard treatment of both male and female psoriatic patients at any age.

No evidence found that childhood onset of psoriasis influences disease severity, future body mass index or type of treatments used.

BACKGROUND: In more than one-third of the psoriatic population, the first manifestations occur in childhood. Whether the age of onset of psoriasis influences the march of psoriasis is not known. OBJECTIVES: To describe the epidemiology and clinical features as well as prescribed treatments and familial distribution in psoriasis depending on the age of onset of the disease. METHODS: A structured questionnaire was sent to 5300 adult psoriatic patients. Respondents were divided into two groups: patients who experienced an onset of disease before the age of 18 [childhood onset psoriasis (COP)] and patients with an onset of disease from the age of 18 [adult onset psoriasis (AOP)]. RESULTS: Questionnaires of 1926 (36.3%) patients were suitable for analysis. In 37.1% of patients, first signs of the disease occurred before the age of 18. COP occurs predominantly in females, has a longer delay in diagnosis and a higher frequency of familial distribution. The development of guttate and erythrodermic psoriasis in adulthood is more frequently seen in COP. In contrast to common belief, type of psoriasis in COP often remains the same from childhood to adulthood. There was no evidence found that getting psoriasis before the age of 18 years influences development of high body mass index in adulthood, disease severity in later life or type of treatments used. CONCLUSIONS: The age of onset of psoriasis essentially does not influence the subsequent course of the disease in adulthood.

[The seated position in patent foramen ovale?]. / Sitzende Position bei offenem Foramen ovale?

Anesthetic management of patients presenting for posterior cranial fossa surgery in the seated position includes detection and treatment of venous air embolism. Atrial positioning of a central venous (cv) line may be verified by either X-ray or an atrial ECG tracing. We report a case where a chest X-ray film proved superior. A 26-year-old white female was scheduled for posterior cranial fossa exploration. A cv line was inserted via the left antecubital vein; the chest X-ray film documented correct positioning of the catheter tip within the atrium but an aberrant course of the superior vena cava. Echocardiography was performed in the induction room and indicated a patent foramen ovale. In view of the risk of paradoxical air embolism, surgery was postponed. Subsequent cardiologic and radiologic examinations revealed a patent foramen ovale and a persisting left superior vena cava draining into a dilated coronary sinus. Surgery was rescheduled and carried out uneventfully in the prone position. This case demonstrates: 1) an advantage of a thoracic-X-ray film compared to atrial ECG tracing as not only the catheter tip position, but also the course of the catheter can be identified; and 2) the usefulness of preoperative screening for a patent foramen ovale in patients scheduled for surgery in the seated position.