bluuebunny/crossref_metadata_2025_split huggingface dataset:
PA06 Impact of clinical pharmacist-led isotretinoin management and acne care education at a multispecialty clinic
Authors: Tran Huynh Wynn , Le Phuong 
Published: June 2023

Abstract
Isotretinoin, an oral retinoid derived from vitamin A, is one of the most effective medications in the treatment of severe acne vulgaris. However, it carries serious risks due to the teratogenic nature of the drug. The iPledge national programme in the USA has been founded to reduce and prevent adverse isotretinoin reactions and complications by ensuring its safe use by patients. Owing to the strict monitoring parameters of isotretinoin, an increased workload is placed on prescribing physicians for accurate and timely iPledge authorization, comprehensive counselling, laboratory testing and many patient follow-up visits. The most commonly reported reasons for delay were iPledge-related, including issues navigating the iPledge website, missed pick-up windows, and missed or delayed doctor appointments or laboratory tests. To remedy the difficulties with isotretinoin monitoring, physicians can look to clinical pharmacists as a valuable asset to their practice. Because clinical pharmacists are well trained in isotretinoin dispensing and iPledge regulation and consultation, they can play a crucial role in the management and education of patients with severe acne vulgaris. This retrospective, longitudinal case study takes place at a multispeciality clinic with internal medicine, rheumatology and dermatology. At this multispecialty clinic, a clinical pharmacist will be integrated into the practice with complete patient appointments and isotretinoin follow-ups under the training and direction of a dermatological specialist. The initial evaluation, diagnosis and prescribing of isotretinoin will be completed by the physician. The clinical pharmacist will follow-up the patients in subsequent visits in conjunction with the physician. The study population is approximately 50 patients with existing diagnoses of severe acne vulgaris. The inclusion criteria will include patients with acne who complete a clinical pharmacist visit for isotretinoin acne treatment from September 2022 to March 2023. At each follow-up clinical pharmacist visit, patients receive management and counselling on medications, a review of laboratory values, iPledge verification, and monitoring of symptoms and quality of life. Primary outcomes are patient symptoms and quality of life, acne grade and severity and side-effects; secondary outcomes are navigation of the iPledge website, number of misses in pick-up windows and doctor appointment adherence. The preliminary results showed significant improvement in primary and secondary outcomes. Patient acne grade decreased, while satisfaction increased; there was also a decreased risk of missed pick-up windows and improved doctor appointment adherence compared with the national standard. In addition, a clinical pharmacist-led iPledge programme can substantially reduce the time required for administrative work on managing isotretinoin while maintaining the same quality of care for patients with severe acne.
PA10 You and Your Skin: developing and trialling a new history-taking aid for psychological wellbeing in children and young people with skin conditions
Authors: Ali Rukshana , Choong Cheryl , Ravenscroft Jane , McPherson Tess , Baron Susannah 
Published: June 2023

Abstract
The mental health burden in children and young people (CYP) living with skin conditions is profound and well documented. There is a lack of integrated psychology/psychiatry provision within paediatric dermatology departments, and dermatologists can struggle to explore psychological wellbeing as part of their clinic consultations due to limited time and/or a lack of confidence and/or training. The British Society for Paediatric and Adolescent Dermatology formed a working group to address the mental health needs of CYP presenting with skin conditions. A multidisciplinary consensus group was formed to assess evidence and develop appropriate guidance. A systematic review found that no single psychological measure or patient-related outcome measure (PROM) adequately captures the psychological burden and impact of living with a skin condition. Patient feedback was clear that PROMs can be less useful than an appropriate clinical consultation. This identified a need for an appropriate clinical tool to help explore CYP psychological wellbeing as part of dermatology consultations: You and Your Skin (YAYS) was developed as a self-completed history-taking aid for CYP &gt; 8 years old and a parent/carer for those &lt; 8 years old. The YAYS was created iteratively using adapted validated and clinically relevant domains from the Children’s Dermatology Quality of Life Questionnaire, the Teenage Quality of Life Questionnaire, the British Association of Dermatologists Acne 3 questions and the Patient Health Questionnaire-9, including self-harm and risk. Questions explore how CYP feel about their skin today and how their skin condition has affected their daily life, caused embarrassment and bullying, and impacted on sleep and mood, and whether they had any thoughts of life not being worth living. The YAYS was co-developed with patients, parents/carers and an external expert stakeholder consensus group (dermatologists, psychologists, psychiatrists, general practitioners, nurse specialists and patient support groups) with revisions done according to feedback on the usability, accessibility and appropriateness of questions. This process has involved 12 revisions of YAYS for CYP and eight revisions of the YAYS parent/carer form, and has been trialled in three paediatric dermatology centres in London, Oxford and Nottingham with 354 CYP and 29 parent/carer forms completed to date. The YAYS development process, findings from YAYS and feedback will be presented. The purpose of YAYS is to enable CYP to raise issues and guide clinicians in evaluation and discussion of mental health concerns in CYP, collaboratively as part of their clinic appointment. Feedback has been very positive from CYP who feel empowered that the impact of their skin disease on all areas of wellbeing can be addressed.
PA12 A case of pustular psoriasis in epidermolytic ichthyosis: coincidence or common pathway?
Authors: Lynch Fiona , Sadlier Muriel , Irvine Alan 
Published: June 2023

Abstract
An 8-year-old boy with epidermolytic ichthyosis (EI) due to a heterozygous KRT1 mutation presented with a striking bullous skin eruption. Microbiological swabs were sent, and oral antibiotics were prescribed for suspected bullous impetigo. He deteriorated clinically, developing a fever and more extensive pustular rash reminiscent of generalized pustular psoriasis (GPP). Skin biopsies from two distinct cutaneous sites revealed histology strongly supportive of pustular psoriasis. Oral ciclosporin was commenced in addition to intensive topical emollient therapy. Following widespread desquamation, the pustular eruption fully resolved. Recurrence upon weaning of ciclosporin necessitated the introduction of ustekinumab. The rash remains controlled on this therapy. The EI is caused by heterozygous mutations in KRT1 or KRT10, and presents at birth with erythroderma and widespread skin denudation. The erythema and skin fragility abate in early childhood, giving way to prominent hyperkeratosis. Severe palmoplantar keratoderma is seen in those with KRT1 mutations. The GPP is a rare but life-threatening condition characterized by sterile pustules with or without systemic involvement. Interleukin (IL)-36-mediated inflammatory signalling plays a pathogenic role, as evidenced by loss of function mutations in IL36RN, which encodes an IL-36 receptor antagonist (Marrakchi S, Guigue P, Renshaw BR et al. Interleukin-36-receptor antagonist deficiency and generalized pustular psoriasis. N Engl J Med 2011; 365:620–8), and the efficacy of IL-36 receptor inhibitor spesolimab in a recent phase II trial. Both EI and GPP are rare, and the confluence of these entities in this case is striking. Four other cases in the literature describe a GPP-like eruption in patients with congenital ichthyoses. The coexistence of these conditions might be explained by their immunological profiles. Studies of the skin of patients with congenital ichthyoses have shown immune dysregulation with T helper 17/IL-23 skewing, and upregulation of associated inflammatory cytokines, including IL-36γ. The latter encodes for an IL-36 receptor agonist (Paller AS, Renert-Yuval Y, Suprun M et al. An IL-17-dominant immune profile is shared across the major orphan forms of ichthyosis. J Allergy Clin Immunol 2017; 139:152–65). Thus, increased IL-36 receptor agonist activity in congenital ichthyosis could theoretically lead to GPP in genetically predisposed individuals with already upregulated IL-36 receptor-mediated signalling.
PA15 Becker naevus syndrome associated with a mosaic pathogenic variant in ACTB
Authors: Bali Radhika , Hussain Khawar , Roberts Nerys , Laguda Bisola 
Published: June 2023

Abstract
A 15-year-old girl presented with an 8-year history of a gradually enlarging, irregular, nonconfluent hyperpigmented patch on her left flank. The lesion demonstrated an area of increased hair growth, did not cross the midline and was mildly pruritic but otherwise asymptomatic. Another pigmented patch, present since birth, was also noted on her right lower back and was consistent with a café-au-lait macule. Medical history included a marfanoid phenotype with musculoskeletal complications including scoliosis and joint hypermobility, for which she was under the care of the orthopaedic team for bilateral patellar instability associated with recurrent dislocation. She was also noted to have increased skin laxity, mild pectus excavatum, mitral valve prolapse and symptomatic tachyarrhythmias. She was born at term, to nonconsanguineous parents, with no postnatal developmental concerns. There was a family history of an older brother with significant scoliosis and hip dysplasia requiring hip replacements, developing from the age of 14 years. Genetic testing was negative for FBN1 and five other genes associated with Marfan syndrome. Thyroid function and bone profile were normal. Menarche occurred at 11 years of age, and a full hormone profile was normal except for a mildly raised prolactin level, deemed to be a normal variant in puberty. Abdominal and pelvic ultrasounds were normal. Skin biopsy from the left flank revealed orthokeratosis, irregular elongation of rete ridges and sparse perivascular chronic inflammation in the superficial dermis. Next-generation sequencing and droplet digital polymerase chain reaction from DNA extracted from skin revealed de novo mosaic pathogenic variants in ACTB, which encodes β-actin, an intracellular protein involved in cell migration, signalling and proliferation. Recurrent mosaic variants in ACTB have previously been associated with 61% of Becker naevi and one case of Becker naevus syndrome in one study (Cai ED, Sun BK, Chian A et al. Postzygotic mutations in beta-actin are associated with Becker’s nevus and Becker’s Nevus syndrome. J Invest Dermatol 2017; 137:1795–8), as well as congenital smooth muscle hamartomas and odontomaxillary dysplasia. Our patient had cutaneous and extracutaneous features suggestive of Becker naevus syndrome. This rare syndrome predominantly involves skeletal and soft tissue abnormalities, with breast hypoplasia most frequently reported. Notable features here include a café-au-lait macule, scoliosis and pectus excavatum. This case highlights the value of DNA analysis from skin in confirming a diagnosis, after 9 years of presentation to paediatric services. Given the heterogeneous extracutaneous phenotypes, there are also important implications for counselling and screening of associated conditions.
PA26 Paediatric atopic dermatitis: correlation between Eczema Area Severity Index, quality of life and visual analogue scale severity scores reported by children and their parents, and in different age groups
Authors: Sambi Pavan , Warburton Katharine , Montgomery Rachel , Thompson Mia , Majeethia Parisha , Clark Sheila 
Published: June 2023

Abstract
Atopic dermatitis (AD) often has a significant impact on the quality of life (QoL) of those affected and their families. Validated QoL and visual analogue scales (VAS) are widely used in dermatology to measure the impact of this common disease. As part of a larger prospective study, we subanalysed data to look at the correlation between Eczema Area and Severity Index (EASI) score, three different QoL scores and parental/child VAS scores for overall severity, itch and sleep disturbance. Data were analysed from 68 paediatric patients with AD (42 males, 26 females), aged 4–16 years (mean 10 years, 0 months). Patient age groups were subdivided into 4–8 years (n = 17), 8–12 years (n = 25) and 12–16 years (n = 26). Scores for EASI, Children’s Dermatology Life Quality Index (CDLQI), Patient Oriented Eczema Measure (POEM) and Dermatitis Family Impact questionnaire (DFI) were measured, and VAS scores (0–10) were assessed for overall severity, itch and sleep loss. The mean EASI score for the cohort was 18.3, and comparable for all age groups: 4–8 years (17.6), 8–12 years (18.1) and 12–16 years (19.0). The CDLQI correlated with DFI (r = 0.72), POEM (r = 0.66) and overall VAS severity scores (r = 0.61). Further analysis by age group showed CDLQI correlated with POEM and DFI most strongly in younger age groups (4–8 years: POEM r = 0.86; DFI r = 0.77; 8–12 years: POEM r = 0.72; DFI r = 0.81) compared with older children aged (12–16 years: POEM r = 0.41; DFI r = 0.53). The CDLQI and VAS overall severity scores correlated consistently across age groups (4–8 years, r = 0.59; 8–12 years, r = 0.67; 12–16 years, r = 0.66). By comparing EASI with CDLQI scores, moderate correlation was found between the age groups (4–8 years, r = 0.39; 8–12 years, r = 0.53; 12–16 years, r = 0.46). Comparing EASI with the three VAS severity scores, we found poor correlation between EASI score and overall severity score (r = 0.24). This was most marked in the 4–8 years group (n = 17, r = 0.04) vs. the 8–12 years group (n = 25, r = 0.27) and 12–16 years group (n = 22, r = 0.33). Similarly, there was poor correlation between EASI and sleep loss score (r = 0.21) and itch score (r = 0.35) across all age groups. In our study, all of the existing validated QoL scores above correlated well with each other and across different age groups. The CDLQI and VAS severity scores also correlated consistently at all ages. There was poor correlation between EASI and VAS severity scores. This was more apparent in younger age groups, implying a high degree of caregiver distress due to eczema in younger children. This may indicate that as children mature their own perception of eczema may diverge from the perceived parental disease severity/impact on the family.
PD02 Home daylight photodynamic therapy
Authors: O’Reilly Marese , Goodman Carol , Yule Susan , Lesar Andrea , Eadie Ewan , Ibbotson Sally 
Published: June 2023

Abstract
Actinic keratoses (AK) on the face and scalp are often extensive and are a major cause of morbidity and associated with increased risk of skin cancer. Field-directed treatment approaches, such as daylight photodynamic therapy (DPDT) are required, and this has been shown to be effective, well-tolerated and feasible in the UK between April and September. We have used DPDT since 2013, with good outcomes. Patient engagement indicated that convenience and tolerability were rated as highly as efficacy, and we therefore developed an entirely home-based approach ‘home DPDT (H-DPDT)’ in 2021. Working with an art-and-design student, we developed a fully recyclable home kit to enable patients to self-treat with H-DPDT entirely in their home environment. Patients were selected by a dermatologist and then received nurse-led training prior to treatment. In-person review after the second treatment was undertaken to assess response and decide if further treatment was needed, and telephone follow-up was undertaken after the first and third treatments. Final clinician review was undertaken 3–6 months after the last treatment. We reported on implementation in 2021 and introduced this into our routine PDT service options, and we now wish to report our findings from 2021–22. Seventeen patients (82% male) were treated in 2021–2022 for AK on the head and neck. Each patient completed an average of four (range 2–5) treatments. Clinical response was good (≥ 75% response) in nine of 15 (60%) patients assessed. Response was moderate in one (7%) patient (50–75% response) and the remaining five (33%) had a poor response (&lt; 50% response). Treatment success correlated with grade of disease. Patients with more hyperkeratotic disease had poorer outcomes. In 2022, five patients responded to the routine daylight PDT questionnaire. Of these, two rated the H-DPDT as Excellent and the other three rated it as Good. Four patients stated that they had tried other AK treatments, two of which thought that H-PDT was better. One patient felt alternative treatments were more effective but they tolerated H-PDT better. Three of the five patients expressed a preference for H-DPDT for repeat treatment and would recommend it. This innovative H-DPDT service enables patients to self-treat in their home environment, and the initial results showed high rates of patient satisfaction. Treatment outcomes were better for those with milder disease, highlighting the need for careful patient selection and good surface preparation. As a proof of concept, H-DPDT is a feasible therapeutic option and empowers patients, but it may have more of a place in the treatment of milder AK disease, and further work to optimize this approach is indicated.
PD03 Afamelanotide reduces the ultraviolet radiation erythemal response soon after application in healthy volunteers with skin phototypes II–III
Authors: Griffin Liezel , Nucci Jessica , Bilbao Pilar , Rhodes Lesley , Farrar Mark 
Published: June 2023

Abstract
Afamelanotide is a potent α-melanocyte stimulating hormone analogue that binds to the melanocortin 1 receptor and, over time, promotes tanning, conferring systemic photoprotection. Afamelanotide also has antioxidant properties and may promote repair of ultraviolet (UV) radiation (UVR)-induced DNA damage. The objectives of this study were to examine the short-term impact of afamelanotide on DNA damage repair mechanisms, UVR erythema and melanogenic responses in healthy human skin following exposure to UVR. Healthy men and women (n = 10) volunteers aged 27–43 years (median 34) of skin phototypes II–III were recruited. Reflectance spectrophotometry was performed on six body sites (photoexposed and photoprotected) to assess skin pigmentation, and then a geometric series of erythemally weighted doses (7–80 mJ cm−2) of broadband UVB (∼60% UVB, 40% UVA; Philips TL12) was applied to unexposed upper buttock skin. After 24 h, the minimal erythema dose (MED) was assessed and erythema intensity (CIE a*) was measured at each dose site. A subcutaneous implant of afamelanotide 16 mg was administered; 5 days later, the same UVR dose series was applied to unexposed contralateral upper buttock skin. After 24 h, the MED was assessed and repeat skin reflectance measurements were performed. The overall UVR–erythema dose response (area under curve) showed a significant decrease post-afamelanotide (mean 3.5 vs. 2.7; P = 0.018). There was an apparent increase in the median MED post-afamelanotide (24.5 vs. 29.9 mJ cm−2; not statistically significant). Reflectance measurements showed skin lightness (CIE L*) significantly decreased at only one site (central forehead, from 63.0 to 61.0; P = 0.015), while determination of melanin density by reflectance at 420 and 400 nm showed a statistically significant increase in density at all sites except the right abdomen, with a mean overall increase from 2.5% to 2.9% (P = 0.001). Our results indicate that afamelanotide reduces the overall UVR–erythema dose response in healthy human skin shortly after administration, with a small increase in melanogenesis, suggesting other nonmelanogenic properties of this agent contribute to the erythema protection. Further investigation will examine whether enhanced repair of UVR-induced DNA damage plays a role.
Funding sources: research sponsored by CLINUVEL (UK) Ltd.
PD05 Investigating the course of atopic eczema up to 1 year following completion of narrowband ultraviolet B phototherapy
Authors: Jain Dimple , Bajaj Sonali , Desai Prachi , Wahie Shyamal 
Published: June 2023

Abstract
Narrowband UVB (NBUVB) is a recommended second-line treatment for moderate-to-severe atopic eczema unresponsive to adequate topical therapy, and is known to induce good short-term improvement of eczema severity (Reynolds NJ, Franklin V, Gray JC et al. NBUVB and broad-band ultraviolet A phototherapy in adult atopic eczema: a randomised controlled trial. Lancet 2001; 357:2012–16). However, it is unclear how adult patients fare with their eczema severity long after completion of a course of NBUVB. We aimed to investigate the severity of atopic eczema in adults, 1 year following the completion of NBUVB, using validated clinical activity measures. We hypothesized that, after 1 year, eczema severity would remain better than at baseline (prior to having NBUVB). We undertook a multicentre prospective observational study of adults with atopic eczema (as per Hanifin and Rajka criteria) prescribed NBUVB as part of their standard clinical care. Participants had moderate-to-severe disease, with a minimum objective SCORAD of 15 and minimum Investigator’s Global Assessment (IGA) of 3, and were resistant to at least one conventional treatment (e.g. potent topical steroid). Eczema severity was assessed by selected investigators using SCORAD and IGA. Assessments were made at baseline, at the end of NBUVB and at 4, 8 and 12 months after completion of NBUVB. We aimed to recruit 80 patients at the outset to provide us with about 25–30 patients for analysis at the 1 year post-NBUVB follow-up. Eighty patients (47 men, 33 women) with a mean age of 37 years (range 18–83) were recruited, with a baseline mean (SD) SCORAD of 39.2 (12.5). Forty-nine patients could not attend to complete NBUVB or the follow-up visits thereafter. Three patients had to stop NBUVB as their eczema flared. Mean baseline SCORAD of the 28 patients who completed the study was 41.3 (14.7), with the mean IGA being 3 (range 3–4; reflecting moderate to severe disease, respectively). Compared with baseline, mean SCORAD was significantly lower at the end of NBUVB [19.1 (13); P &lt; 0.001] and at 4 months post-NBUVB [21.9 (11.9); P &lt; 0.001]. Furthermore, compared with baseline, there was a sustained ongoing reduction of mean SCORAD at 8 months [22.6 (13.1); P &lt; 0.001] and 12 months post-NBUVB [21.1 (14.8); P &lt; 0.001]. Mean SCORAD at 1 year was not statistically significantly different to that on completion of NBUVB (P &gt; 0.5). Change in IGA matched similar sustained improvement trends with SCORAD. These findings indicate that NBUVB may provide a long-lasting effect for patients who complete a course, helping to diminish their eczema severity to a more manageable level. These data can further inform consultations with patients, when outlining the approach of NBUVB for their care.