British Journal of Dermatology最新文献

Background: Generalised pustular psoriasis (GPP) is a chronic, systemic, neutrophilic inflammatory disease. A previous Delphi panel established areas of consensus on GPP, although patient perspectives were not included, and aspects of treatment goals remain unclear.

Objectives: To identify and achieve consensus on refined, specific treatment goals for GPP treatment via a Delphi panel with patient participation.

Methods: Statements were generated based on a systematic literature review and revised by a Steering Committee. Statements were categorised into overarching principles, short-term treatment goals and long-term treatment goals. A global panel of 30 dermatologists and three patient representatives voted in agreement or disagreement with each statement. Consensus was defined as ≥80% approval by panellists.

Results: Consensus was reached in the first round of voting and ≥90% agreement was reached for 23/26 statements. In summary, GPP requires a timely, tailored treatment plan, co-developed by patients and physicians, that involves a multidisciplinary approach and addresses the complexity, heterogeneity and chronicity of the disease. Short-term treatment goals should include pustule clearance within 7 days and prevention of pustule recurrence, reduction of cutaneous symptom burden (≥ -4 points on the Itch and Skin Pain Numeric Rating Scale), improvement in systemic symptoms (e.g. resolution of fever within 3 days of treatment initiation and reduced fatigue), prevention of life-threatening complications, and progressive improvement of inflammatory biomarkers. In patients with comorbid psoriatic diseases, treatment decisions should prioritise GPP. Long-term treatment goals should include minimising disease activity through flare prevention and symptom control between flares, sustained disease control, management of comorbidities and improvement in quality of life (QoL). Small differences in perception between patients and physicians regarding the importance of certain treatment goals, e.g. avoiding hair and/or nail loss to improve QoL, reflect the complexity of assessing treatment goals and emphasise the need for a patient-centred approach.

Conclusions: In the first global Delphi panel in GPP to include patient perspectives, consensus between dermatologists and patients was achieved on overarching principles of treatment, short-term and long-term treatment goals for GPP. These findings provide valuable insights for developing guidelines that consider the perspectives of both patients and physicians in the treatment of GPP.

The ability to grow long scalp hair is a distinct human characteristic. It probably originally evolved to aid in cooling the sun-exposed head, although the genetic determinants of long hair are largely unknown. Despite ancestral variations in hair growth, long scalp hair is common to all extant human populations, which suggests its emergence before or concurrently with the emergence of anatomically modern humans (AMHs), approximately 300 000 years ago. Long scalp hair in AMHs was also a trait that was selected because it conveyed essential signals related to an individual's age, sexual maturity, health and social status. Biologically, hair length is primarily determined by the amount of time that a hair follicle spends in the active growth phase (anagen). While anagen duration is typically tightly regulated in most mammals, the inherent ability of a hair follicle to continuously recruit new dividing progenitors to its base, where hair fibre is generated, theoretically removes limits on maximal anagen duration. We propose a model wherein hair cycle progression into and out of anagen is regulated by evolutionary malleable molecular checkpoints. Several animal species and domesticated animal breeds display long body hair, which suggests that extremely long scalp hair in humans emerged via attenuation of an existing out-of-anagen checkpoint mechanism rather than via a newly evolved molecular programme. Studying congenital and somatic mosaicism conditions featuring altered hair length could potentially unveil the currently unknown molecular basis underlying this human trait.

Melanoma is the fifth most common skin cancer in the UK, accounting for 4% of all new cancer cases, with a predicted 7% increase in incidence between 2014-35. In parallel, since the initial publication of the Melanoma NICE Guidelines in 2015, there has been a paradigm shift in the management of the disease, with the introduction of effective systemic therapies. These innovations have reshaped the management of melanoma throughout the patient journey, and improved clinical outcomes. Surgical management has evolved, with the role of sentinel node biopsy in staging and management of regional lymph nodes becoming clearly defined, and a reduction in the need and indications for morbid block dissections. In advanced disease, effective therapies have allowed a de-escalation of surgery, changing the role and sequencing of local therapies. Regional therapies for in-transit disease have expanded and are effective in controlling this pattern of disease as part of multidisciplinary care. These advances have undoubtedly improved the care for people with melanoma, but have also increased the complexity of management. In this context, this article seeks to summarizes the most relevant of the recent updates to the NICE guidelines.

Background: Epidermolysis bullosa (EB) is a group of rare, severe, genetic disorders characterised by persistent skin fragility and open wounds. EB manifests as cutaneous and mucosal blistering, erosions and impaired wound healing.

Objectives: To determine the long-term efficacy, tolerability and safety of Oleogel-S10 (birch bark extract) in dystrophic (DEB) and junctional (JEB) EB in the 24-months open-label phase (OLP) of the EASE study.

Methods: EASE was a double-blind, randomised, controlled, phase III study consisting of two phases: a 90-day double-blind phase (DBP), and a 24-month OLP. Patients from both former treatment groups in the DBP entered the single-arm OLP (n = 205). Patients received Oleogel-S10 on all EB partial thickness wounds. OLP endpoints included: incidence, severity/relatedness of adverse events (AEs), wound infection maximum severity, changes in body surface area percentage (BSAP) of wounds, EB Disease Activity and Scarring Index (EBDASI), pain, itch, disease severity and quality of life outcomes.

Results: The OLP data demonstrated Oleogel-S10 target wound treatment adherence was >99% and mean treatment duration was 584.7 days (±246.1 days). 71.7% of patients in the OLP were aged <18 years and 86.8% had DEB; recessive DEB predominated (78.0%). AEs were reported in 77.1% of patients and were typically mild-to-moderate. Severe and serious AEs were observed in 18.0% and 24.4% of patients, respectively. AEs resulted in the withdrawal of 7.8% of patients (n = 16), including three with treatment-related AEs. Nine deaths were reported: none attributable to treatment. Incidence of target wound infections was low (n = 7); five were mild-to-moderate and two severe. In patients treated with Oleogel-S10 throughout, mean (SD) BSAP changes from DBP baseline at 3, 12 and 24 months were -4.3% (8.1), P < 0.0001; -5.9% (8.6), P < 0.0001; -3.7% (9.0), P = 0.0026, respectively. Similarly, significant changes in EBDASI skin activity score from DBP baseline were observed: -3.9 (8.3), P < 0.0001; -5.1 (8.2), P < 0.0001; -3.0 (8.3), P = 0.0068, at 3, 12 and 24 months, respectively.

Conclusions: These data support an encouraging long-term safety profile of Oleogel-S10, and a sustained reduction in wound burden over at least 24 months of Oleogel-S10 treatment.

Background: The lack of attention to Chronic Hand Eczema (CHE) and the lack of a specific International Classification of Diseases code for CHE may have limited the assessment of CHE prevalence. To date, prevalence estimates have primarily been derived from (partly small) single-country studies.

Objectives: To estimate the annual prevalence of self-reported physician-diagnosed CHE across socio-demographic characteristics among adults in Canada, France, Germany, Italy, Spain, and the United Kingdom (UK).

Methods: In this observational Chronic Hand Eczema epidemiology, Care, and Knowledge of real-life burden (CHECK) study, a questionnaire was administered to adults between 18 and 69 years old in the general population, recruited through online panels. Quotas and minor weighting adjustments were performed to ensure that the participants were representative of the general population regarding sex, age, region, employment status, urban/rural setting, and, in the UK only, ethnicity. Additional weights were applied to account for population size differences when aggregating country results. Information on self-reported physician-diagnosed CHE was collected. CHE was defined, in accordance with the European Society of Contact Dermatitis, as having hand eczema continuously for three months or more or at least two flares in the past 12 months. CHE annual prevalence with 95% confidence intervals (CIs) was determined for each country, and by subgroups of sex, age, employment, and urban/rural.

Results: Among 60,131 participants, 2,847 self-reported physician-diagnosed CHE, yielding an annual prevalence of 4.7% (CI: 4.6-4.9). Subgroup analyses revealed the CHE prevalence was significantly higher in females than males (5.6% [5.4-5.9] vs. 3.8% [3.6-4.1]; P<0.001), in employed versus unemployed participants (5.3% [5.1-5.6] vs. 3.3% [3.1-3.6]; P<0.001), and in urban versus rural residents (5.0% [4.8-5.2] vs. 3.7% [3.4-4.1]; P<0.001). The prevalence was highest among those aged 30-39 years (6.5% [6.0-7.0]) and lowest in those aged 60-69 years (2.6% [2.3-3.0]).

Conclusions: This large multi-national study is the first to assess CHE prevalence in Europe and Canada using a consistent definition across a broad geographical population. This study reveals that CHE is a common skin disease with annual prevalence of 4.7%, with higher prevalence among females, individuals aged 30-39, those employed, and those living in urban areas.