Effectiveness and safety of dupilumab in the treatment of atopic dermatitis in children (6-11 years): data from a French multicentre retrospective cohort in daily practice.

BACKGROUND: Dupilumab is the first biotherapy available for the treatment of moderate-to-severe childhood atopic dermatitis (AD). OBJECTIVE: The aim of this study was to evaluate the effectiveness and safety of dupilumab in daily practice. METHODS: Patients aged 6-11, who had received a first dose of dupilumab, were included in this multicentre retrospective cohort study. The primary endpoint was change in SCORAD after 3 months of treatment. Secondary endpoints were change in IGA score at 3 months, proportion of patients with SCORAD50 and SCORAD75, description of adverse events and proportion of children in our cohort who would be excluded from pivotal phase 3 clinical trial. RESULTS: Eighty patients were included. After 3 months of treatment, there was a significant decrease in SCORAD (mean: 21.8 ± 13.8 vs 53.9 ± 18.5; P < 0.0001) and IGA (1.3 ± 0.8 vs 3.5 ± 0.7; P < 0.0001). Conjunctivitis was observed in 11.3% (n = 9/80); three patients experienced dupilumab facial redness (DFR); 17.5% (n = 14/80) reported injection site reactions; 6.3% (n = 5/80) discontinued treatment. 61.2% (n = 49/80) children were ineligible in the phase 3 trial. LIMITATIONS: There is no control group. Because it was a real life study based on information from patient medical records in a French multicentre cohort, we cannot rule out the presence of reporting bias generated by the use of patient reported characteristics and missing information. CONCLUSION: These real-life data confirm the efficacy and safety of dupilumab in children with moderate to severe AD extended to dyshidrosis and atopic prurigo, but it also revealed a lower frequency of DFR and conjunctivitis. However, administration in injectable form may be a barrier in this age group.

Evidence-based recommendations to assess psoriasis severity: systematic literature review and expert opinion of a panel of dermatologists.

BACKGROUND: Severity of psoriasis appears to be multidimensional and its assessment in everyday clinical practice requires a complex holistic approach. OBJECTIVES: To develop evidence-based recommendations to assess severity of plaque-type psoriasis in adult patients in everyday clinical practice. METHODS: A scientific committee (10 members identified on the basis of their expertise in psoriasis) using Delphi methodology selected eight questions in three domains: severity, health-related quality of life (HR-QoL) and comorbidities. Three systematic literature reviews (one per domain) of all studies published between January 1980 and June 2009 were performed based on Pub-Med, Cochrane and Embase database. Selected articles were systematically reviewed and evidence appraised according to the Oxford Levels of Evidence. On June 2009, a group of 44 French dermatologists both hospital and office based participated in a meeting including three separate rounds of discussions, plenary sessions, and modified Delphi technique votes. Recommendations for clinical practice based on systematic review and clinical experience were formulated by the group. Subsequently, agreements among the participants regarding these recommendations as well as potential impact on clinical practice were evaluated. RESULTS: A total of 10 642 references were identified, of which 154 articles were analysed. Ten key recommendations on the assessment of psoriasis severity were formulated: three recommendations relating to severity assessment, three recommendations relating to HR-QoL (including the use of the Dermatology Life Quality Index [DLQI] in clinical practice) and four recommendations relating to comorbidities (including systematic screening for peripheral or axial inflammatory joint damage, regardless of psoriasis severity). CONCLUSIONS: Ten recommendations to assess the severity of plaque-type psoriasis in adult patients in daily practice were developed. The recommendations are based on systematic appraisal of available evidence. They were developed and supported by a panel of dermatologists, which enhances their validity and practical relevance.

Evaluation of a novel 308-nm monochromatic excimer light delivery system in dermatology: a pilot study in different chronic localized dermatoses.

BACKGROUND: Recently, units have been developed that are capable of delivering large fluences of narrowband ultraviolet (UV) B selectively to cutaneous lesions within a reasonable time. OBJECTIVES: To analyse the efficacy of a novel nonlaser 308-nm monochromatic excimer light (MEL) delivery system in various dermatoses usually treated by narrowband UVB phototherapy. METHODS: Fifty-four patients with chronic and resistant localized dermatoses were enrolled in a prospective study: 17 with palmoplantar pustular psoriasis, seven with plaque-type psoriasis, four with nail psoriasis, eight with chronic atopic dermatitis of the hands, 10 with chronic nonatopic dermatitis of the hands and eight with alopecia areata. The 308-nm xenon chloride MEL delivery system (Excilite; DEKA, Florence, Italy) was used to produce an average incident dose rate of 50 mW cm(-2) at a tube-to-skin distance of 15 cm and with a maximum irradiating area of 512 cm2. The initial dose was based on multiples of a predetermined minimal erythema dose (MED), and subsequent doses were based on the response to treatment. Treatments were scheduled weekly for a maximum of 10 weeks. Clinical responses were evaluated using photographic documentation and (except for alopecia areata) clinical score. RESULTS: The MED ranged from 250 to 350 mJ cm(-2) (mean +/- SD 318.2 +/- 28.4). MEL at 308 nm was the most effective for palmoplantar pustular psoriasis with a mean improvement of 79% after a mean of 5.3 treatments and a mean dose of 11.8 MED per treatment. Plaque-type psoriasis was significantly less sensitive to treatment and nail psoriasis demonstrated no benefit from treatment. Chronic palmar atopic dermatitis was cleared in two patients and the mean improvement was 54% as compared with 46% in patients with chronic nonatopic dermatitis of the hands. Four complete regrowths among the eight patients with alopecia were observed after a mean of 5.1 treatments. The percentages of improvement had significantly decreased at the 6-month visit, and only four patients (24%) with palmoplantar pustular psoriasis still demonstrated a significant improvement. Common side-effects included intense erythema and, more rarely, blisters, but these were well tolerated. CONCLUSIONS: Our preliminary results confirm the efficacy of this novel 308-nm MEL delivery system, which appears to be effective and safe for palmoplantar pustular psoriasis. To a lesser extent, plaque-type psoriasis, chronic atopic and nonatopic dermatitis of the hands and alopecia may also benefit from this treatment.

[Iatrogenic acrodermatitis enteropathica-like syndrome in leucinosis]. / Pseudo-acrodermatite entéropathique per carence alimentaire en isoleucine chez un nourrisson atteint de leucinose.

INTRODUCTION: Leucinosis (maple syrup urine disease) is a metabolic disorder caused by an enzymatic deficiency involved in the degradative pathways of the three branched-chain amino acids. We report an observation of acrodermatitis enteropathica-like syndrome induced by essential amino acid deficiency in a child with leucinosis. CASE REPORT: A child with leucinosis was referred to our hospital for exfoliative dermatitis of the perioral and anogenital regions associated with diarrhea and pancytopenia. The diagnosis of iatrogenic acrodermatitis enteropathica-like syndrome was confirmed after screening showing isoleucine deficiency. Rapid response was observed after adequate isoleucine supplementation. DISCUSSION: The acrodermatitis enteropathica-like eruption in our patient was due to an iatrogenic amino acid nutritional imbalance. Our observation underlines the risk of using a branched-chain amino acid-free formula without adequate supplementation of deficient amino acids. In addition, dietary insufficiency of isoleucine, associated with the treatment of organic aciduria should be added to the causes of acrodermatitis enteropathica-like syndrome.

Genital squamous cell carcinoma in men treated by photochemotherapy. A cancer registry-based study from 1978 to 1998.

BACKGROUND: One single report from the U.S. 16-centre-trial indicated that psoralen and ultraviolet A radiation (PUVA) therapy may induce an increased risk of genital tumours in men, and protection of the genital area is, therefore, recommended. OBJECTIVES: To evaluate the relevance of this risk in routine clinical practice. METHODS: Two groups of patients were included in a 1978-98 retrospective study. Case records of men with genital squamous cell carcinoma (SCC) identified from the Cancer Registry of the Doubs area of France were examined for a history of PUVA therapy, topical tar treatment, psoriasis, human papillomavirus infection or genital dermatitis. In addition, all the dermatologists of the Doubs area (in public and private practice) using PUVA therapy were asked to provide information on the number of patients having received PUVA therapy and whether the genital area was exposed during treatment. RESULTS: Between 1978 and 1998, among the 48 men who had developed a genital SCC in the Doubs area, only one had a history of intensive PUVA therapy. About 150,000 treatments with PUVA therapy had been performed by 15 dermatologists in the Doubs area for 5400 patients since 1978. No case of genital SCC had been reported, despite the fact that the genital area had not been protected during UVA exposure. CONCLUSIONS: Although retrospective, our study demonstrates that the occurrence of genital SCC in men treated with PUVA therapy is a very rare event in common dermatological practice.