Multidisciplinary educational programme for caregivers of children with atopic dermatitis- in South East Norway - an observational study.

BACKGROUND: Educational programmes for caregivers of children with atopic dermatitis (AD) are reported to reduce the severity of AD and improve quality of life (QOL). Oslo University Hospital (OUH) in Norway offers a multidisciplinary educational programme for caregivers of children with AD. We aimed to evaluate the AD educational programme by assessing QOL of the family, the severity of the disease and caregiver's fear of topical corticosteroid (TCS) before and after attending the programme. METHODS: This was a small observational prospective cohort study including 41 caregiver-child pairs. The children (mean age 3.4 years) had doctors' diagnosed AD with a difficult to treat eczema. The children's caregivers were referred from physicians to attend the AD educational programme at our hospital. At inclusion and at a 3 months follow-up QOL was assessed by Dermatitis Family Impact (DFI), the eczema severity by Patient-Orientated - SCORing Atopic Dermatitis (PO-SCORAD) and caregivers fear of TCS was recorded by asking a dichotomous "yes" or "no" question: "Are you worried about using TCS on your child?" RESULTS: Three months after caregivers attending the educational programme there was an improvement in QOL by reduced mean DFI from 9.6 (SD 6.3) to 6.8 (SD 5.4), the mean PO-SCORAD was reduced from 38.5 (SD 15.1) to 24.6 (SD13.6), the number of caregivers reporting fear of TCS use was reduced from 33/46 (72%) to 12/41 (29%). All results p < 0.001. CONCLUSION: Our study suggests beneficial effects by improving QOL of the family, the severity of the eczema and in reducing the fear of TCS when caregivers of children with difficult to treat AD attend an AD multidisciplinary educational programme. Lack of control group makes it difficult to draw definite conclusions.

Management of congenital ichthyoses: European guidelines of care, part two.

These guidelines for the management of congenital ichthyoses have been developed by a multidisciplinary group of European experts following a systematic review of the current literature, an expert conference held in Toulouse in 2016, and a consensus on the discussions. These guidelines summarize evidence and expert-based recommendations and intend to help clinicians with the management of these rare and often complex diseases. These guidelines comprise two sections. This is part two, covering the management of complications and the particularities of some forms of congenital ichthyosis.

Management of congenital ichthyoses: European guidelines of care, part one.

These guidelines for the management of congenital ichthyoses have been developed by a multidisciplinary group of European experts following a systematic review of the current literature, an expert conference held in Toulouse in 2016 and a consensus on the discussions. They summarize evidence and expert-based recommendations and are intended to help clinicians with the management of these rare and often complex diseases. These guidelines comprise two sections. This is part one, covering topical therapies, systemic therapies, psychosocial management, communicating the diagnosis and genetic counselling.

Whole-exome sequencing for diagnosis of hereditary ichthyosis.

BACKGROUND: Hereditary ichthyosis constitutes a diverse group of cornification disorders. Identification of the molecular cause facilitates optimal patient care. OBJECTIVE: We wanted to estimate the diagnostic yield of applying whole-exome sequencing (WES) in the routine genetic workup of inherited ichthyosis. METHODS: During a 3-year-period, all ichthyosis patients, except X-linked and mild vulgar ichthyosis, consecutively admitted to a university hospital clinic were offered WES with subsequent analysis of ichthyosis-related genes as a first-line genetic investigation. Clinical and molecular data have been collected retrospectively. RESULTS: Genetic variants causative for the ichthyosis were identified in 27 of 34 investigated patients (79.4%). In all, 31 causative mutations across 13 genes were disclosed, including 12 novel variants. TGM1 was the most frequently mutated gene, accounting for 43.7% of patients suffering from autosomal recessive congenital ichthyosis (ARCI). CONCLUSION: Whole-exome sequencing appears an effective tool in disclosing the molecular cause of patients with hereditary ichthyosis seen in clinical practice and should be considered a first-tier genetic test in these patients.

Narrowband UVB therapy for vitiligo: does the repigmentation last?

BACKGROUND: Since 1997, a number of trials have shown promising results in treating generalized vitiligo with narrowband ultraviolet B (UVB) both in adults and children. However, there is little knowledge concerning the duration and permanency of the treatment-induced repigmentation. OBJECTIVE: Our main objective was to perform a follow-up trial of successfully treated patients receiving narrowband UVB for generalized vitiligo. METHODS: We have investigated to what degree the treatment-induced repigmentation remains stable for up to 2 years post-treatment. We performed an initial open trial including 31 patients with generalized vitiligo. They received narrowband UVB thrice weekly for up to 12 months. Patients experiencing > 75% repigmentation were defined responders and were included in the follow-up trial. Responders were followed every 6 months for up to 2 years after cessation of treatment. We observed the pigmentation status and registered any changes indicating loss of pigmentation and relapse. RESULTS: Eleven of the 31 treated patients were included in the follow-up trial. Six patients had relapse and five patients had stable response 24 months after cessation of treatment. Four out of six relapses were within 6 months post-treatment. CONCLUSION: In our study population of 31 patients with generalized vitiligo, five patients (16%) experienced > 75% stable repigmentation 2 years after cessation of a treatment programme of up to 1 years narrowband UVB therapy.