IQoL-32: a new ichthyosis-specific measure of quality of life.

Background: Inherited ichthyoses are associated with impaired quality of life (QoL).

Objectives: The aim of this study was to create and validate a QoL questionnaire specifically dedicated to patients with ichthyosis.

Methods: A prequestionnaire was drawn after selecting items from a verbatim transcript.

Therapeutic patient education in children with atopic dermatitis: position paper on objectives and recommendations.

Poor adherence is frequent in patients with atopic dermatitis (AD), leading to therapeutic failure. Therapeutic patient education (TPE) helps patients with chronic disease to acquire or maintain the skills they need to manage their chronic disease. After a review of the literature, a group of multispecialty physicians, nurses, psychologists, and patients worked together during two international workshops to develop common recommendations for TPE in AD.

Therapeutic patient education in atopic dermatitis: worldwide experiences.

Therapeutic patient education (TPE) has proven effective in increasing treatment adherence and improving quality of life (QoL) for patients with numerous chronic diseases, especially atopic dermatitis (AD). This study was undertaken to identify worldwide TPE experiences in AD treatment. Experts from 23 hospitals, located in 11 countries, responded to a questionnaire on 10 major items.

[Schnitzler's syndrome].

Schnitzler syndrome (SS) is a rare clinical entity, which belongs to the spectrum of monoclonal gammapathy-associated systemic disorders. Its pathophysiology remains elusive, even if it is tempting to consider it as a late onset and probably acquired auto-inflammatory syndrome. SS mainly occurs in the fifth and sixth decade, and present with an urticariform rash with periodic fever and/or osteoarticular pain.

Large congenital melanocytic nevi: therapeutic management and melanoma risk: a systematic review.

Background: Large congenital melanocytic nevi (LCMN) are defined as congenital melanocytic nevi having a projected adult size exceeding 20 cm in diameter. They occur in 1 in 20,000 to 1 in 500,000 newborns. Therapeutic management of LCMN remains controversial and is often decided according to melanoma risk.

Clinical characteristics predicting internal neurofibromas in 357 children with neurofibromatosis-1: results from a cross-selectional study.

Objective: To identify clinical characteristics associated with internal neurofibromas in children with NF1, as a means of ensuring the early identification of patients at high risk for malignant peripheral nerve-sheath tumors developed from preexisting internal neurofibromas.

Patients And Methods: We used data from two NF1 populations, in France and North America, respectively. The French database comprised 1083 patients meeting NIH diagnostic criteria for NF1 and the Neurofibromatosis Institute Database of North America comprised 703 patients.

[Skin manifestations of new targeted treatments].

Many cutaneous adverse events have been identified with recently developed targeted treatments. Some of them are common and specific, like paradoxical psoriasiform eruptions with anti-TNFα, papulopustular eruptions and paronychias with EGFR inhibitors and peculiar hand-foot skin reactions with multitargeted kinase inhibitors sorefenib and sunitinib. Patients treated with these recently available biologics need a careful monitoring.

[Non adherence and topical steroids].

Compliance raises very specific questions in dermatology related to the frequent use of local treatments: creams or ointments, including topical corticosteroids. The adherence in dermatology is a complex issue. It is difficult to quantify objectively because of the patient subjectivity, the constant adaptation to changes in the course of the disease, and due to the lack of adapted device.

An α-lactalbumin-enriched and symbiotic-supplemented v. a standard infant formula: a multicentre, double-blind, randomised trial.

The aim of the present study was to evaluate the safety, tolerance and preventive effect on atopic dermatitis of an experimental α-lactalbumin-enriched and symbiotic-supplemented infant formula. A total of ninety-seven non-breastfed term neonates were enrolled into a double-blind, multicentre, randomised controlled trial in which they received experimental (n 48) or standard formula (n 49) for 6 months. The primary outcome was weight at 6 months of age.

Factors influencing quality of life in patients with inherited ichthyosis: a qualitative study in adults using focus groups.

Background: There is limited information regarding quality of life in patients with inherited ichthyosis.

Objectives: To identify factors influencing quality of life in patients with inherited ichthyosis.

Methods: The study used focus groups and involved adult patients suffering from inherited ichthyosis from three French hospital centres.

At-risk phenotype of neurofibromatose-1 patients: a multicentre case-control study.

Objectives: To assess associations between subcutaneous neurofibromas (SC-NFs) and internal neurofibromas in patients with neurofibromatosis type 1 (NF-1) and to determine whether the association between SC-NFs and peripheral neuropathy was ascribable to internal neurofibromas.

Patients And Methods: Prospective multicentre case-control study. Between 2005 and 2008, 110 NF-1 adults having two or more SC-NFs were individually matched for age, sex and hospital with 110 controls who had no SC-NF.

Short- and medium-term efficacy of specific hydrotherapy in inherited ichthyosis.

Background: Management of inherited ichthyoses is symptomatic. Despite treatment, skin symptoms have a major impact on patients' quality of life (QoL).

Objectives: To assess the short- and medium-term efficacy of hydrotherapy on QoL and clinical symptoms of patients with inherited ichthyosis.

[Labor epidural analgesia for a woman with a pityriasis versicolor in the lumbar region].

Epidural analgesia is usually contraindicated in case of infection at the site of needle insertion. Tinea versicolor is a benign superficial cutaneous fungal infection caused by the proliferation of a skin commensal yeast of low pathogenicity. We report the case of a pregnant woman with a tinea versicolor in the lumbar region, who benefited from a labor epidural analgesia, realised with reinforced antiseptic measures.

Topical corticosteroid phobia in atopic dermatitis: a study of its nature, origins and frequency.

Background: Topical corticosteroids remain the mainstay of atopic dermatitis therapy. Many atopic dermatitis therapeutic failures appear to be attributable to poor adherence to treatment due to topical corticosteroid phobia.

Objectives: To assess the facets, origins and frequency of fear of topical corticosteroid use among patients with atopic dermatitis.

[Amicrobial pustulosis of the skin folds and autoimmune erythroblastopenia].

Introduction: Amicrobial pustulosis of the skin folds represents a new entity within the spectrum of neutrophilic dermatoses. This disease is characterized by acute onset of pustular lesions in the skin folds, association with an autoimmune disorder, and improvement under systemic corticosteroids.

Observation: A 24-year-old woman had been presenting pustular dermatosis for several months involving the inguinal folds and the scalp.

Efficacy and tolerability of methotrexate in severe childhood alopecia areata.

Background: Alopecia areata (AA) occurring in childhood is associated with a poorer prognosis than adult AA and may severely affect quality of life. The efficacy of methotrexate (MTX) was reported in adults with AA but there is little information about its use in children.

Objectives: We aimed to assess the efficacy and safety of MTX in severe childhood AA.

[A rare congenital lesion: bronchogenic cyst. Case report and review of the literature].

Cutaneous bronchogenic cysts are very rare congenital lesions. Scapular localization is frequently found in the literature. Only the histological analysis can confirm the diagnosis, because no clinical characteristic is pathognomonic.

Patient-Oriented SCORAD (PO-SCORAD): a new self-assessment scale in atopic dermatitis validated in Europe.

Background: Patient-oriented medicine is an emerging concept, encouraged by the World Health Organization, to greater involvement of the patient in the management of chronic diseases. The Patient-Oriented SCORing Atopic Dermatitis (PO-SCORAD) index is a self-assessment score allowing the patient to comprehensively evaluate the actual course of atopic dermatitis (AD), using subjective and objective criteria derived mainly from the SCORAD, a validated AD severity clinical assessment tool.

Objectives: To validate the PO-SCORAD index in a large European population of patients exhibiting all forms of AD severity by assessing its correlation with the SCORAD index.