Comparison of the Simple Patient-Centric Atopic Dermatitis Scoring System PEST with SCORAD in Young Children Using a Ceramide Dominant Therapeutic Moisturizer.

INTRODUCTION: Patient eczema severity time (PEST) is a new atopic dermatitis (AD) scoring system based on patients' own perception of their disease. Conventional scales such as SCORing of atopic dermatitis (SCORAD) reflect the clinician's observations during the clinic visit. Instead, the PEST score captures eczema severity, relapse and recovery as experienced by the patient or caregiver on a daily basis, promoting patient engagement, compliance with treatment and improved outcomes. This study aims to determine the correlation between carer-assessed PEST and clinician-assessed SCORAD in paediatric AD patients after 12 weeks of treatment using a ceramide-dominant therapeutic moisturizer. METHODS: Prospective, open-label, observational, multi-centre study in which children with AD aged 6 months to 6 years were treated with a ceramide dominant therapeutic moisturizer twice daily for 12 weeks; 58 children with mild-to-moderate AD were included. Correlation between the 7-day averaged PEST and SCORAD scores for assessment of AD severity was measured within a general linear model. PEST and SCORAD were compared in week 4 and week 12. RESULTS: At week 12, a moderate correlation was found between the SCORAD and PEST scores (r = 0.51). The mean change in SCORAD and PEST scores from baseline to week 12 was -11.46 [95% confidence interval (CI) -14.99 to -7.92, p < 0.0001] and -1.33 (95% CI -0.71 to -0.10, p < 0.0001) respectively. PEST demonstrated greater responsiveness to change (33.3% of scale) compared to SCORAD (13.8% of scale). CONCLUSION: The PEST score correlates well with the SCORAD score and may have improved sensitivity when detecting changes in the severity of AD. The ceramide-dominant therapeutic moisturizer used was safe and effective in the management of AD in young children

Identification of a large rearrangement in CYLD as a cause of familial cylindromatosis

Pathogenic mutations in CYLD can be identified in patients affected with Brooke-Spiegler syndrome, (Familial) Cylindromatosis or multiple familial trichoepithelioma. To date, only technologies which are able to identify small point mutations in CYLD, such as sequence and WAVE analysis, were used. Here we describe the identification of a larger rearrangement identified by Quantitative PCR analysis of CYLD, indicating that a combination of these technologies is necessary when searching for pathogenic mutations in CYLD

Trichotillomania and related disorders in children and adolescents

Eleven chronic hair pullers, 11 subjects with obsessive-compulsive disorder (OCD), and 11 subjects with a non-OCD anxiety disorder were assessed with structured interviews and the Child Behavior Checklist (CBCL). Only 4 hair pullers (36%) reported both rising tension and relief with hair pulling. Each group had significantly more internalizing than externalizing symptoms on the CBCL. Seven hair pullers (64%) had a lifetime history of at least one other axis I diagnosis. The results provide further evidence that trichotillomania in referred children and adolescents is usually a chronic disorder often associated with internalizing symptoms and psychiatric comorbidity. Rising tension followed by relief with hair pulling may be an unnecessary restriction in the diagnosis of childhood trichotillomania.Peer Reviewedhttp://deepblue.lib.umich.edu/bitstream/2027.42/43954/1/10578_2006_Article_BF02353354.pd

Periungual hyperpigmentation in newborns.

Hyperpigmentation of the distal phalanx of both hands and feet is usually a prominent feature of dark-skinned newborns, but recently it has been described also in fair-skinned ones as a coincidental finding and classified as a transient benign disorder of infancy. We report our experience, having observed a group of at-term newborns and a group of premature newborns. The group of premature newborns did not show any hyperpigmentation. On the contrary, 7 out of 40 fair-skinned at-term newborns had hyperpigmentation in the distal phalanx of the fingers which was not related to any dermatologic disorder and started to fade away after 2 years of age, i.e., longer than previously reported. The reason why some fair-skinned newborns display this hyperpigmentation is not known. Although we can speculate that it can be reactive to maternal pigment hormones, its persistence is not completely in agreement with this possibility