bluuebunny/crossref_metadata_2025 huggingface dataset:
P036 Dupilumab treatment normalizes skin barrier function in children aged 6–11 years with moderate-to-severe atopic dermatitis
Authors: Cork Michael J , Leung Donald , Ong Peck , Danby Simon , Ramirez-Gama Marco , Garcia Shannon , Patricia Taylor , Byers Rob , Matcher Stephen , Zahn Joseph , Bologna Gabriel , Zhang Annie
Published: June 2024

Atopic dermatitis (AD) is associated with significant disruption in skin barrier function, mediated by the type 2 inflammatory cytokines interleukin (IL)-4 and IL-13. This study reports the effect of dupilumab treatment in paediatric patients (aged 6–11 years) with moderate-to-severe AD on skin barrier function and clinician- and patient-reported outcomes. PELISTAD (NCT04718870) was an open-label, exploratory study on skin barrier function in children aged 6–11 years with moderate-to-severe AD treated with dupilumab for 16 weeks based on baseline patient weight (300 mg every 4 weeks: ≥ 15 kg to < 30 kg; 200 mg every 2 weeks: ≥ 30 kg to < 60 kg) and matched healthy volunteers. Transepidermal water loss (TEWL) was assessed longitudinally after skin tape stripping from lesional and nonlesional skin of patients with AD treated with dupilumab, and from healthy skin. Eczema Area and Severity Index (EASI) and skin pain numerical rating scale (NRS) were assessed during the same periods. Twenty-three dupilumab-treated patients and 18 healthy volunteers were included in the study. After 16 weeks of dupilumab treatment, median (95% confidence interval) TEWL after five skin tape strips significantly decreased in the lesional skin of patients with AD (33.8, 25.2–42.3) compared with baseline (63.7, 44.1–83.3; P < 0.001). Least squares mean (standard error) TEWL after five skin tape strips in lesional skin (30.7, 3.5) was comparable with that observed in healthy skin (27.8, 4.4) at week 16 (P = 0.62). Similar improvement was observed in nonlesional skin (25.5, 2.1), which did not significantly differ from healthy skin (24.6, 2.6; P = 0.79). Mean (SD) EASI decreased from 34.8 (11.9) at baseline to 11.3 (11.0) at week 16. Improvement was also observed in skin pain NRS, which decreased from mean (SD) 6.8 (2.5) at baseline to 2.3 (1.4) at week 16. Overall safety was consistent with the known dupilumab safety profile. Dupilumab treatment normalizes skin barrier function and improves clinician- and patient-reported outcomes in paediatric patients aged 6–11 years with moderate-to-severe AD. This research was sponsored by Sanofi and Regeneron Pharmaceuticals Inc. ClinicalTrials.gov identifier: NCT04718870.
P054 National incidence and survival of extramammary Paget disease 2013–2020 in England: reporting a total of 740 cases
Authors: Karkara Shweta , Khan Farishta , Van Bodegraven Birgitta , Venables Zoe C
Published: June 2024

Extramammary Paget disease (EMPD) is a rare disease affecting the rich apocrine gland-bearing areas commonly involving the genital and perianal skin. It was first described in 1889 by Radcliffe Crocker. Clinical features of EMPD are insidious and nonspecific, frequently resulting in delayed diagnosis. Diagnosis of EMPD is important due to its association with an underlying malignancy, most commonly urothelial or anorectal, but it may also be cervical, prostatic, ovarian or endometrial in 10–30% cases. Until recently, there were limited national epidemiological data on incidence and survival. National data on EMPD incidence and 5-year survival in England from 2013 to 2020 were extracted from report-level data published openly by the ‘Get Data Out’ National Disease Registration Service programme. Crude incidence rates are reported per 100 000 person-years (PY). Survival is reported as overall Kaplan–Meier survival and net survival with associated 95% confidence intervals (CIs). Data for 2020 vs. 2019 incidence rate ratios with 95% CIs were calculated using the exact Poisson method. In total, 740 new cases of EMPD were diagnosed between 2013 and 2020, with a mean of 93 cases per year. The average crude incidence rate of EMPD per year was 0.17 per 100 000 PY (95% CI 0.14–0.21). The crude incidence rate in 2013 was 0.16 per 100 000 PY (95% CI 0.13–0.20), which increased nonsignificantly in 2019 to 0.20 per 100 000 (95% CI 0.16–0.24). During 2020, the first year of the COVID-19 pandemic, incidence decreased to 0.14 per 100 000 (95% CI 0.11–0.18). The incidence rate ratio between 2020 and 2019 was 0.73 (95% CI 0.54–0.98), with a P-value of 0.03. The 5-year overall Kaplan–Meier survival was 74.1% (95% CI 63.4–82.1) for EMPD diagnosed in 2013. Important limitations include lack of published data on site, age group, depth of invasion and associated comorbidities including malignancies due to small numbers. The reported net survival for EMPD is comparable with that for melanoma and prostate and breast cancer, presumably a result of its association with other underlying malignancies. The increase in crude incidence rates likely represents the ageing population, and one might expect age-standardized rates to be relatively static. The apparent decrease in the crude incidence rate of EMPD during 2020 demonstrates the impact of the pandemic on skin cancer services, as also shown for melanoma and other cancers. A study in the Netherlands assessed 178 cases of invasive and 48 cases of in situ EMPD and found an overall relative 5-year survival of 72% for invasive tumours and an incidence rate of 0.11 per 100 000 PY for EMPD. These data are important in better understanding the trends and outcomes of this rare and poorly understood tumour.
P076 Risk of psoriatic arthritis and uveitis among patients with psoriasis after COVID-19 infection: a nationwide cohort study
Authors: Chen Tai-Li , Wu Chen-Yi , Chang Wei-Chuen , Chen Chih-Chiang
Published: June 2024

Psoriasis is a chronic, inflammatory, immune-mediated dermatosis associated with multiple comorbidities, such as psoriatic arthritis and uveitis. Systemic inflammation contributes to the pathophysiology of psoriatic complications. Nevertheless, whether patients with psoriasis have a higher risk of developing psoriatic arthritis and uveitis after COVID-19 infection, a disease known to boost inflammatory cytokines, was largely unknown. Therefore, we aimed to investigate the risk of incident psoriatic arthritis and uveitis among patients with psoriasis after COVID-19 infection using real-world data. In this retrospective nationwide study, we analysed data from the US Collaborative Network of the TriNetX Research Database. Adults aged ≥ 18 years diagnosed with psoriasis were eligible for inclusion. Two study cohorts were established: a COVID-19 cohort and a non-COVID-19 cohort. Patients with a diagnosis of COVID-19 between 2020 and 2022 contributed to the COVID-19 cohort. Patients without COVID-19 were matched to construct the non-COVID-19 cohort using 1 : 1 propensity score matching. Multiple covariates, such as age, sex, race, ethnicity, comorbidities, baseline steroid use, systemic immunosuppressant use, and use of biologics, were included to calculate the propensity score. We excluded participants with a history of psoriatic arthritis or uveitis. A Cox regression model was used to estimate hazard ratios (HRs) and 95% confidence intervals (CIs) for psoriatic arthritis and uveitis. Stratified analyses according to age, sex, race and ethnicity were also performed. In total 21 186 participants were included. Overall, 1083 patients with COVID-19 and 495 control patients developed psoriatic arthritis during the follow-up. Patients with psoriasis after COVID-19 infection had an increased risk of incident psoriatic arthritis (HR 2.16, 95% CI 2.07–2.25) compared with those without COVID-19. Moreover, 748 patients with COVID-19 and 401 control patients developed uveitis. Patients with psoriasis with COVID-19 had an increased risk of incident uveitis (HR 1.83, 95% CI 1.70–1.97) compared with those without COVID-19. The increased risk of psoriatic arthritis and uveitis remained significant among men, White patients, and those aged > 45 years. In conclusion, patients with psoriasis after COVID-19 infection have an associated increased risk of psoriatic arthritis and uveitis. Long-term follow-up of complications such as psoriatic arthritis and uveitis may be considered for patients with psoriasis after COVID-19 infection. Future research is warranted to elucidate the pathophysiology linking psoriasis, COVID-19 and psoriatic comorbidities.
P087 The British Hair and Nail Society present a review of our national grand round of challenging hair disorders and the benefits to the wider British Association of Dermatologists membership
Authors: Sharma Sonia , Bryden Alyson , Clayton Nicola , Farrant Paul , Mowbray Megan , Holmes Susan , Therianou Anastasia , Al-Nuaimi Yusur , Meah Nekma , Asfour Leila , Takwale Anita
Published: June 2024

The British Hair and Nail Society was formed in 2011. We currently have 133 members and invite them to submit challenging cases of hair disorders to our grand round (with relevant history, histopathology and photographs) for review by our expert hair panel. This comprises consultants with hair expertise from around the UK, and together we aim to address the referrers’ queries relating to diagnosis, treatment or both. We appreciate the complexity of hair disorders and the limited number of specialist hair clinics around the country, and therefore we have recently offered the opportunity for all British Association of Dermatologists (BAD) members to submit cases once a year. The aim of our review is to present the types of complex hair disorders that have been referred to our dedicated national hair-related grand round and showcase some of the most educational cases. The hair disorders grand round began in 2018 and since then a total of 26 hair cases (10 children and 16 adults) have been submitted from various regions. The main aims of referral include diagnostic expertise (n = 4), aid with treatment plans (n = 8) or both (n = 14). The median age of patients was 33 years (range 4–75). There were 14 female and 12 male cases. The regions of referral include Oxford, Brighton, Manchester and Scotland. There were equal numbers of scarring alopecia (n = 13) and nonscarring (n = 13) cases. The most common nonscarring hair condition was alopecia areata (n = 6), with the possibility of other concurrent hair conditions (n = 1) or an underlying genetic disorder (n = 3). The most common diagnosis of scarring alopecia was folliculitis decalvans (n = 6). The diagnosis was altered in 10 cases (38%). Examples of some of our rare diagnoses include keratosis follicularis spinulosa decalvans, genetic hypotrichosis and scalp dermatomyositis. We reviewed cases of recalcitrant disease such as folliculitis decalvans, and novel therapeutics were suggested such as the use of naltrexone, utilizing photodynamic therapy after removing crusts, and even the use of manuka honey and cannabinoid oil. Our grand round has recommended independent funding requests in three cases and supports applications from clinicians for their patients with complex conditions. We hope our presentation showcases to the wider BAD membership the value of our national hair disorders grand round in supporting complex diagnostic and therapeutic decisions, as well as offering support for clinicians in the form of individual funding request submissions or trialling novel therapies, which may have limited evidence. We welcome any future submissions.
P098 Association between air pollution and incidence of psoriasis: a nationwide population-based case–control study
Authors: Li Chia-Lun , Ma Sheng-Hsiang , Wu Chun-Ying , Lyu Ying-Syuan , Chang Yun-Ting , Ao Chon-Kit , Wu Chen-Yi
Published: June 2024

Air pollution (AP) is an alarming public health crisis worldwide. Common ambient pollutants include sulfur dioxide (SO2), carbon monoxide (CO), ozone (O3), coarse particulate matter (PM10), fine particulate matter (PM2.5), and nitrogen dioxide (NO2). Although previous studies have shown that AP has been associated with various cardiovascular, respiratory and inflammatory diseases, the relationship between AP and psoriasis remains unknown. This nationwide, population-based case–control study aims to explore the relationship between AP and incident psoriasis, using data from the National Health Insurance Research Database of Taiwan between 2012 and 2018. Patients with newly diagnosed psoriasis were included, which was confirmed by board-certified dermatologists or rheumatologists on at least three occasions. Exclusion criteria included patients with a diagnosis of psoriasis before 2012, patients under 20 years of age in 2012, or incomplete AP data. For each patient with psoriasis, four controls were matched considering age, sex, urbanization level, index date and comorbidities. AP data were retrieved from 73 general air quality monitoring stations operated by the Taiwan Environmental Protection Administration. Exposure to air pollutants (SO2, CO, O3, PM10, PM2.5 and NO2) was analysed in the 5 years preceding psoriasis diagnosis and in controls. The mean pollution levels were further divided into four quartiles (1 = lowest and 4 = highest) to assess the dose–response effect. A conditional logistic regression model was used to assess the association. Our study included 45 093 patients with psoriasis and 180 372 matched controls. No significant intergroup difference was found in age, sex, urbanization level or comorbidities. After adjusting for age, sex, urbanization, index date and comorbidities, the second and third concentration quartiles of CO exposure were associated with a decreased psoriasis incidence [adjusted odds ratio (aOR) 0.94, 95% confidence interval (CI) 0.91–0.97; and aOR 0.91, 95% CI 0.89–0.94, respectively), while the third concentration quartile of PM10 (aOR 1.05, 95% CI 1.02–1.08) and the second concentration quartile of PM2.5 (aOR 1.08, 95% CI 1.04–1.11) were associated with a slightly increased incidence of psoriasis. Using a model incorporating all air pollutants, we found that the levels of PM10 (quartile 4: aOR 1.09, 95% CI 1.03–1.16), PM2.5 (quartile 2: aOR 1.08, 95% CI 1.04–1.13) and NO2 (quartile 4: aOR 1.14, 95% CI 1.05–1.23) were associated with a slight increase in the incidence of psoriasis. Our study shows that although exposure to certain PM10, PM2.5 and NO2 levels was significantly associated with incident psoriasis, the increased risks were trivial, with no dose–response relationship.
P109 Association between air quality index and the risk of rosacea: a nationwide population-based cohort study
Authors: Ma Sheng-Hsiang , Wu Chun-Ying , Li Meng-Chieh , Ho Hsiu J , Ao Chon-Kit , Wu Chen-Yi
Published: June 2024

Air pollution is associated with several inflammatory skin disorders. However, the association between air quality and rosacea remains unclear. Our objective was to investigate the association between air quality index (AQI) and the incidence of rosacea. Overall, 21 709 479 participants without rosacea before 2008 were recruited from the Taiwan National Health Insurance Research Database. The long-term average AQI value for each participant was acquired from the Taiwan Air Quality Monitoring System Network and was calculated from 1 January 2008 until the diagnosis of rosacea, withdrawal from National Health Insurance, or 31 December 2018. We observed a significant association between AQI and the incidence of rosacea, with each unit elevation in AQI increasing the risk of rosacea by 5%. Compared with the quartile 1 group, the quartile 2, 3 and 4 cohorts exhibited 1.82-fold, 4.48-fold and 7.22-fold increased risks of rosacea, respectively. Additionally, exposure to PM2.5, SO2 and CO increased the risk of rosacea, whereas exposure to PM10 was associated with a lower risk. Limitations of this study include its retrospective design and involvement of participants mainly from Taiwan. Additionally, some potential confounding factors may not have been accessible in the database. This study supported a significant dose–response relationship between AQI and the incidence of rosacea.
P111 A retrospective analysis of cutaneous sarcoidosis
Authors: Hiranput Siwaporn , McAllister Lucy , Shing Stacey Li Hi , Damani Namiz , Abdalla Doaa , Yesudian Paul Devakar
Published: June 2024

Sarcoidosis is a multisystem granulomatous disorder of uncertain aetiology, manifesting with cutaneous features in one-third of affected individuals. We retrospectively reviewed medical records of patients with a clinical diagnosis of cutaneous sarcoidosis in dermatology departments within our region. Sixty-five patients were identified. The mean age at presentation was 49 years (range 28–75), with increased prevalence in women (44, 68%). Twenty-six patients (40%) had localized disease affecting a single anatomical location, three of which were within tattoos. Thirty-nine (60%) had involvement of at least two anatomical locations. Limbs were the most frequently affected site (41). Other affected areas were the face (32), torso (25), scalp (9) and neck (5). Plaques were the most common morphology, seen in 26 cases, followed by papules (23), nodules (23), patches (6), erythema nodosum (4), annular (3), ulceration (3) and subcutaneous nodules (3). The colours of lesions were erythematous (37), yellow–orange–brown (22), violaceous (9) and skin coloured (5). All patients had biopsy-proven noncaseating granulomas; 60 patients had this confirmed on skin biopsy and five patients from other organs. In total, 41 patients (63%) had systemic sarcoidosis. Twenty-four of those with systemic sarcoid osis (59%) had cutaneous signs as their first presentation of sarcoid osis. Pulmonary sarcoidosis was diagnosed in 36 (55%), with relevant findings on chest X-ray, computed tomography of the thorax and/or pulmonary function tests. Of these, 22 patients did not report respiratory symptoms. Other organs involved were eye (6), joint (6), extrapulmonary lymph nodes (5), liver (4), cardiac (3), central nervous system (2) and renal (2). Lymphopenia was detected in 14 (22%) and anaemia in six (9%) patients. Twenty-three (35%) had elevated serum angiotensin-converting enzyme (ACE) levels. Four patients with elevated serum ACE levels did not have systemic sarcoidosis. Twenty-two of 41 patients with systemic sarcoidosis did not have elevated serum ACE levels. Complete resolution of skin lesions was observed in 32 patients. Management included watchful monitoring (8), potent or superpotent topical steroids (11), short courses of prednisolone (3), antimalarials (3), intralesional steroids (2), methotrexate (1), tofacitinib (1), and treatments initiated for systemic sarcoidosis including long-term prednisolone (3) and adalimumab (1). Our study highlights a lack of correlation between clinical features of cutaneous disease and systemic involvement. We found no association between biochemical profile and severity of sarcoidosis, or disease progression. It underscores the importance of maintaining a high index of suspicion and conducting thorough investigations for early detection of extracutaneous disease, as patients with extensive systemic sarcoidosis may be asymptomatic.
BH09 Mycophenolate mofetil in the management of lichen planus pigmentosus in a woman with frontal fibrosing alopecia
Authors: Htwe Su , Holmes Susan
Published: June 2024

A 50-year-old woman of south Asian descent presented with a 4-year history of hair loss, itching and follicular prominence at her frontal hairline, in association with increasingly sparse eyebrows and loss of body hair. She was otherwise well and on no medication. Clinical examination revealed typical features of frontal fibrosing alopecia (FFA), with associated papules on the forehead. In addition, striking hyperpigmentation was evident over the forehead, face and neck. This was felt to be in keeping with FFA-associated dyspigmentation–lichen planus (LP) pigmentosus. Initial management was lymecycline 408 mg once daily, tacrolimus 0.1% ointment twice daily plus mometasone furoate 0.1% two times weekly to the neck, and pimecrolimus cream twice a day to the face, as well as a sun protection factor 50 sunscreen. Hydroxychloroquine was avoided initially due to the potential side-effect of altered skin pigmentation with this drug. While the hairline recession remained unchanged, the pigmentary changes worsened despite treatment and affected areas became increasingly itchy. Lymecycline was stopped and hydroxychloroquine 200 mg once daily was started. After 12 months, the cutaneous pigmentation continued to worsen and was extending onto the arms and torso. A trial of low-dose isotretinoin (20 mg once daily) was considered as this has been reported to be of benefit in LP pigmentosus. However, after 6 months, there was no improvement. A trial of mycophenolate mofetil 500 mg twice daily was then commenced. After 3 months of treatment, skin itching had settled and no new areas of hyperpigmentation were noted. After 9 months, there had been a significant improvement in skin pigmentation, which has been maintained. The extent of frontal hairline recession has remained stable. While FFA was initially described exclusively in postmenopausal White women, it has since been reported in Hispanic, African, Afro-Caribbean and Asian individuals, premenopausal women, and men. Dyspigmentation associated with FFA has largely been a feature observed in patients with darker skin types, and it is not clear whether this pigmentation is part of the spectrum of FFA in individuals with darker skin or whether LP pigmentosus is occurring coincidently with FFA in patients with darker skin types. Low-dose isotretinoin has been reported as being of benefit in LP pigmentosus but was ineffective in this case. Mycophenolate mofetil is also reported to be of benefit in the management of LP pigmentosus, and led to a significant improvement in both pigmentation and itch in this case. This case highlights the therapeutic benefit of mycophenolate mofetil in in LP pigmentosus associated with FFA.
PD08 Normal phototests in five patients with Bloom syndrome
Authors: Bakr Farrah , Duncan Mhairi , Naik Harsha , Sarkany Robert , Lehmann Alan , Mohammed Shehla , Fassihi Hiva
Published: June 2024

Bloom syndrome (BS) is a rare autosomal recessive disorder characterized by abnormal growth, skin changes, immunodeficiency, insulin resistance and diabetes, impaired fertility, and a significantly increased risk of cancer from an early age, commonly leukaemia. It is caused by variants in the BLM gene, encoding the DNA repair enzyme RecQL3 helicase, resulting in chromosome instability, excessive homologous recombination, and a greatly increased number of sister chromatid exchanges (SCE), pathognomonic of the syndrome. In the literature, patients with BS are frequently reported to be photosensitive. Although the skin is typically normal at birth, there are reports of marked and recurrent erythema following sun exposure, resulting in progressive facial telangiectasia and poikiloderma. The facial rash is commonly in a butterfly distribution across the nose and cheeks and is thought to be further exacerbated by sun exposure. Over time, the rash can also appear on the dorsum of the hands and forearms. In order to better characterize the photosensitivity reported in the literature, all patients with BS in a specialist unit were assessed by a photodermatologist and underwent phototesting. There were five children (three male, two female; age range 7–15 years) with a confirmed diagnosis of BS based on abnormal SCE and detection of pathogenic variants in the BLM gene. Two of the children had skin phototype I–II (ethnicity: Scotland, Poland) and three had phototype IV (ethnicity: one from Pakistan, two from India). None of the children described severe and exaggerated sunburn reactions despite sunny holidays abroad. On examination, there were no facial skin changes, specifically no evidence of telangiectasia or poikiloderma. Following monochromator and solar simulated irradiation, all five had normal immediate and 24-h responses. There were no delayed reactions. In summary, we present five cases of BS without the characteristic finding of photosensitivity, supported by normal phototesting. This is unlike the severe and exaggerated sunburn reactions following minimal sun exposure seen in other DNA repair disorders such as subtypes of xeroderma pigmentosum and trichothiodystrophy. This is the first time that photosensitivity in BS has been objectively assessed, and these phototest findings call into question the idea that photosensitivity is a cardinal feature of this disease entity. Nevertheless, in view of the known increased risk of nonmelanoma skin cancer in patients with BS from their early 30s, regular skin surveillance and sensible sun protection measures remain the mainstay of the dermatological management of these patients.
PD13 A UK single-centre prospective audit and clinical experience of developing an ultraviolet B phototherapy service using 308-nm excimer pulsed light for inflammatory skin disease
Authors: Mohammed Haya , Vilenchik Victoria , Banner Alexandra , Keith Daniel
Published: June 2024

Phototherapy is commonly recognized as a convenient treatment modality for inflammatory skin diseases (ISDs). It has seen significant technological advancements over the last 2 decades. With variable success, high running costs and demand for trained professionals, treatment outcomes in ISDs using cabinet phototherapy are limited. Due to its high efficacy and safety profile, 308-nm excimer pulsed light (EPL) represents an exciting and practical option in moderate-to-severe ISDs that are treatment resistant and difficult to reach, where only affected areas are targeted and overall fewer patient visits are required for clearance than with conventional phototherapy. A prospective single-centre cohort study was conducted on 36 patients with ISDs treated with EPL, including psoriasis (n = 14), palmoplantar pustular psoriasis (n = 6), vitiligo (n = 2), necrobiosis lipoidica (n = 3), vitiligo (n = 2) and dermatosis (n = 9). The number of sessions required ranged from 4 to 18, with a maximum fluence between 250 and 850 mJ cm−2 among patients. The primary outcome involved assessing lesions post-EPL treatment using Physician’s Global Assessment (PGA) scores including ‘much better’, ‘better’, ‘no difference’ or ‘worsened’. Secondary outcomes included occurrence of complications and comparison of Dermatology Life Quality Index (DLQI) scores before and after EPL. At the end of each completed EPL treatment course, lasting an average of six sessions in total, and requiring an average maximum fluence of 350 mJ cm−2, 72% (n = 26) of patients achieved clearance and were found to have ‘much better’ or ‘better’ lesion outcomes when reviewing PGA scores. A significant reduction in post-EPL DLQI in 28% (n = 10) of patients was also found, with an average score reduction by 4. No complications were reported in 94% of patients (n = 34), with minor post-EPL burn found in 6% of patients (n = 2) but high overall patient satisfaction. In conclusion, our study contributes further supporting evidence to the scarce dermatological literature surrounding the successful use of EPL as a powerful treatment modality in inflammatory skin disease, especially if recalcitrant. Large studies including randomized controlled trials are necessary to help validate these promising results and determine optimal dosing regimens, long-term efficacy and patient satisfaction.
DS03 Survey of dermatological surgical training in the UK
Authors: Webber Lucy , Barlow Richard , Bray Adam
Published: June 2024

Skin cancer incidence is rising annually in the UK, creating a substantial surgical burden. The dermatology training curriculum stipulates the need for regular supervised surgical lists with increasing independence throughout training to help address this growing demand. We distributed an electronic survey to dermatology trainees via the British Association of Dermatologists and British Society for Dermatological Surgery to evaluate current dermatological surgery training in the UK. The survey focused on training experience, satisfaction, and procedural competence to identify areas for improvement. Overall, 79 out of approximately 280 trainees responded (28.2%), of whom 64 (82%) were based in England, 8 in Wales (10%), 5 in Scotland (6%) and 1 in Northern Ireland (1%). Twelve deaneries within England were represented. The majority worked full time (56 trainees; 71%), and 76% of respondents were female. Twelve respondents were CESR trainees (15%) and three were clinical fellows (4%), with the remainder distributed from ST3 to ST6. In total, 59 respondents (76%) had surgical exposure within dermatology or allied surgical specialties before entering the training programme. Fifteen respondents (19%) reported having a supervised surgical list prior to starting training, defined as a consultant, senior dermatologist or equivalent observing their list. Around one-half of trainees (45, 53%) first received surgical supervision during their ST3 year, and 20.3% (16 trainees) had never received direct surgical supervision. Of these respondents, more than one-third (38%, six trainees) were at ST5/ST6 level. Overall, 65% (50 trainees) received intermittent direct supervision during their training, influenced by factors such as departmental staffing and prioritization of service provision; 68% of respondents (54 trainees) reported seeking additional hands-on surgical opportunities to fill gaps in their training. In total, 20% (16 trainees) felt that the procedural curriculum was unachievable within their training programme, and 29% of ST6 trainees (6 of 21) reported an inability to perform excision biopsies on the head and neck unsupervised. Dissatisfaction with surgical training was expressed by 35% (27 trainees). While our response rate limits the ability to draw definitive conclusions, our survey highlights significant intra- and interdeanery disparities in dermatological surgical training. A striking 86% of trainees (68) expressed the need for more consistent supervision to maintain and develop their surgical skills. Trainees call for clearer curricular guidance on surgical exposure and supervision, and national standardization of training to enhance surgical proficiency among the future consultant workforce.
DP05 (P061) Squamoid eccrine ductal carcinomas: challenges in diagnosis and management
Authors: Cunliffe Amy , Elsheikh Somaia , Odedra Sunita , Varma Sandeep
Published: June 2024

Squamoid eccrine ductal carcinomas (SEDCs) are rare adnexal carcinomas displaying both squamous and duct differentiation. We wish to remind clinicians of these recently described cancers, which can have aggressive behaviour (van der Horst MPJ, Garcia-Herrera A, Markiewicz D et al. Squamoid eccrine ductal carcinoma. Am J Surg Pathol 2016; 40: 755–60). We identified five cases in our department: four female patients and one male. All lesions developed on the face, in patients aged 69–83 years. The majority developed over one to several years, apart from one rapidly growing lesion, and measured between 5 and 40 mm. Clinical examination suggested squamous cell carcinoma (SCC) in two cases and basal cell carcinoma in three cases due to features such as puckering, rolled borders, telangiectasia, shine, ulceration and crust. Punch biopsies were performed in all five lesions; however, histological diagnosis of SEDC was suggested in only two. One of these patients underwent Mohs micrographic surgery (MMS), which successfully cleared the lesion after two stages. The second patient, diagnosed on biopsy, had a large 30-mm lesion with invasion into the lacrimal sac and left nasal bone. The patient opted for palliative radiotherapy rather than extensive surgery and subsequently developed a left-cheek metastasis and symptoms suggestive of metastatic disease. Three of our cases were diagnosed after wide local excision (WLE) based on the histological and immunological features. The punch biopsies had suggested either SCC or adenosquamous/adnexal infiltrative carcinoma. One of these patients required adjuvant radiotherapy due to perineural involvement (< 0.1 mm). The other two cases were incompletely excised on WLE and the patients were offered further surgery or radiotherapy. Histologically these lesions were poorly defined infiltrative neoplasms consisting of atypical squamoid cells extending into the deep dermis or subcutaneous tissue with ductal differentiation, pronounced cytological atypia and associated desmoplastic stroma. Tumour thickness measured 3–10.3 mm. Immunohistochemistry was positive for CK7, AE1/AE3, CK5/6, p63, CAM5.2, carcinoembryonic antigen and epithelial membrane antigen. These five cases highlight the challenges faced in the diagnosis of SEDC for both the dermatologist and the dermatopathologist. MMS is recommended (Lim MM, Macdonald JA. Squamoid eccrine ductal carcinoma: treatment and outcomes. Am J Dermatopathol 2022; 44: 249–53) and lesions misdiagnosed as SCC may receive less effective treatment; our three patients who underwent WLE have required further interventions. We also report one patient with locally aggressive and likely metastatic disease. Patients with SEDC benefit from early diagnosis, MMS and close follow-up to reduce the risk of recurrence and improve patient outcomes.
DP06 (P001) A descriptive, observational and cross-sectional study of clinicoepidemiological, dermoscopic and histopathological aspects of primary localized cutaneous amyloidosis and its effect on the quality of life of the patient
Authors: Rane Meghana , Mahajan Sunanda
Published: June 2024

The aims of this study were to examine the clinicoepidemiological factors related to primary localized cutaneous amyloidosis; to investigate its dermoscopic and histopathological findings; to determine the clinicohistopathological and dermoscopic correlation; and to study its effect on the quality of life of the patients (Al-Ratrout JT, Satti MB. Primary localized cutaneous amyloidosis: a clinicopathological study from Saudi Arabia. Int J Dermatol 1997; 36: 428–34; Rasi A, Khatami A, Javaheri SM. Macular amyloidosis: an assessment of prevalence, sex and age. Int J Dermatol 2004; 43: 898–9). An open, prospective, descriptive and observational study was conducted in patients with primary cutaneous localized amyloidosis suspected clinically. It was carried out for a period of 18 months, with 100 patients in the study. Only patients who gave written informed consent for the study were included. Pregnant and lactating patients were also excluded. Clinical examination with clinical photographs, skin biopsy and dermoscopy of the same lesions was done using a disposable handheld biopsy punch under local anaesthesia. The final diagnosis was then made after compiling all of the clinical, dermoscopic and histopathological features. Microsoft Excel and SPSS version 19 (IBM, Armonk, NY, USA) were used for statistical analysis. Out of the 100 patients, 56 had macular amyloidosis, 33 had lichenoid amyloidosis, eight had biphasic amyloidosis, two had dyschromic amyloidosis and one had primary amyloidosis of the concha. The most common age group was 40–60 years, and the condition was more common in women. A history of use of scrubbing and rubbing while bathing was reported in 45 patients: 33 (73%) of these had macular amyloidosis, nine (20%) had lichenoid amyloidosis, two (4%) had mixed amyloidosis and one (2%) had dyschromic amyloidosis. Pruritus was seen in 92 patients (92%). Dermoscopy of amyloidosis showed a hub-and-spoke pattern and amorphous pigmented globules; 11 patients showed nonspecific pigmented globules and seven showed scales and a central scar pattern. On histopathology of macular amyloidosis, 94 patients showed amyloid deposits stained positively with Congo red. The mean Dermatology Life Quality Index was 3 in macular amyloidosis, and 4 in lichenoid and biphasic amyloidosis. Studies using confocal laser scanning microscopy and direct immunofluorescence could further provide insight and can allow better outcomes. Primary cutaneous amyloidosis has a small-to-moderate effect on the quality of life of patients as it is pruritic and disfiguring. Dermoscopy is a valuable tool to diagnose characteristic features and thus helps to make quick and accurate diagnosis and avoid skin biopsies, as shown in this study.
DP10 Scalp disease in dermatomyositis
Authors: Connolly Aveen , Philippidou Marianna , Gordon Patrick , Wincup Chris , Worsnop Fiona , Creamer Daniel
Published: June 2024

The clinical presentation of dermatomyositis (DM) is heterogeneous, with patients expressing varying degrees of muscle and skin inflammation. Scalp inflammation is often disregarded in the clinical assessment and is seldom the subject of clinical studies. However, DM at this site contributes significantly to the symptom burden, a feature heightened by the scalp’s comparative lack of response to standard immunosuppressant therapy. As well as a sparsity of studies detailing clinical features in scalp DM, the literature on scalp histopathology is also limited. In an attempt to define the range of clinicopathological features, and to consider potential associations with myositis-specific antibodies, we undertook a prospective observational study of scalp disease in a cohort of patients with DM. Over a 10-year period, 96 consecutive adult patients with clinically and immunologically proven DM were prospectively recruited. The extent of cutaneous DM was quantified using the Cutaneous Dermatomyositis Area and Severity Index (CDASI). For the purposes of our study, the scalp-CDASI was recorded separately. In patients with scalp DM, sites of involvement were recorded: frontal, parietal, occipital and vertex. The impact of cutaneous involvement was assessed using the Dermatology Life Quality Index and a scalp itch numerical rating score (NRS). In total, 47 of 96 patients with DM had scalp involvement, and all patients had cutaneous DM at other body sites. In the scalp DM cohort, scalp-CDASI ranged from 1/9 to 8/9. The distribution of DM involvement by scalp zone demonstrated 19 of 47 global, 17 of 47 occipitoparietal, and 11 of 47 frontovertex. The mean scalp itch NRS for the scalp DM group was 4.8, 33 of 47 patients reported scalp itching and/or soreness disturbing sleep, and 38 of 47 patients reported hair loss. In the scalp DM group, myositis-specific antibodies were demonstrated in 41 of 47 patients. Overall, 17 of 41 patients were positive for TIF-1g, 11 of 41 SAE, 6 of 41 MDA5, 3 of 41 Mi-2, 3 of 41 NXP-2, and 1 of 41 Jo-1. Assessment of scalp biopsies from nine patients demonstrated a number of dermatopathological features that appear exclusive to scalp DM. Unlike other scarring alopecias, there was no typical perifollicular fibroplasia, but in these cases, there was thickening of the basement membrane around the follicles. A further consistent observation involved the dermal collagen, which showed hyalinization, homogenization and areas of necrobiosis, as well as areas of very severe elastosis. Our findings suggest that the scalp possesses a site-specific susceptibility to DM inflammation, a hypothesis that mirrors the disease tropism seen in psoriasis, lupus and other inflammatory dermatoses. The evidence collected in this study has unequivocally demonstrated that scalp DM produces a symptom burden associated with considerable morbidity. Our study is the first to examine the subjective effects of scalp involvement in DM and to quantify its impact.
DP13 First case of distant metastases from a BAP1-inactivated melanocytoma with melanoma transformation
Authors: Guckian Jonathan , Mathew Bipin , Merchant Will , Muinonen-Martin Andrew , Mitra Angana , Smith Hayley
Published: June 2024

A 43-year-old man presented with a long-standing back lesion causing irritation and bleeding. A presumed pedunculated seborrhoeic keratosis was shave excised by his general practitioner; however, histology revealed a BRCA1-associated protein (BAP1)-inactivated melanocytoma (BIM) with melanoma transformation. Histologically, BIMs are identified by the presence of a dermally located expansile clone of epithelioid melanocytes, sometimes associated with a pre-existent conventional naevus. Clones have abundant amphophilic cytoplasm and nuclear polymorphism with distinct cell membranes. BAP1 immunohistochemistry showing loss of nuclear expression in altered melanocytes confirms the diagnosis. BIMs typically harbour a BRAF V600E mutation as the initial oncogenic driver, with subsequent biallelic loss of the BAP1 gene leading to BAP1 protein inactivation. They typically run a benign, indolent course, with no cases with distant metastasis previously reported. Our case had BAP1-inactivated clones with typical BIM morphology. However, there was also a dominant, aggressive, BAP1-inactivated subclone with 15 mitoses, Ki67 > 10%, ulceration, P16 loss and PRAME positivity. These features are unusual for BIMs and are considered high risk in conventional melanoma subtypes. Overlying this was a junctional component with focal architectural and cytological atypia, bordering on melanoma in situ. Next-generation sequencing showed an NRAS driver mutation. DNA methylation profiling showed more than three copy number variations: 8q12.1q24.3, 90.2-Mb gain; whole chromosome 9 loss; 9p21.3, 3-Mb biallelic loss including CDKN2A and CDKN2B (whole genes); and 11q22.3q25, 31.7-Mb loss. There was no significant family history, and germline BAP1 genetic testing was negative. The patient underwent narrow margin excision followed by wide local excision. Sentinel lymph node biopsy was not offered due to previous pulmonary emboli and obesity. After 11 months he reported persistent abdominal pain. Computed tomography showed widespread metastatic disease (right axillary lymph nodes, brain and liver). Lymph node biopsy revealed atypical naevoid to epithelioid cells with focal cytoplasmic pigmentation and mitotic activity lying within fibrous stroma. Immunohistochemistry demonstrated strong diffuse expression of SOX-10 and melan-A, with loss of BAP1 expression. We report the first case of BIM with melanoma transformation (malignant BAP1 inactivated tumour) resulting in stage IV melanoma. Our centre has identified four further lesions in three patients with similar histological presentation. Two patients with three lesions developed locoregional recurrence. Our cases demonstrate that not all BIMs run a benign to indolent course. Identifying high-risk features as seen in these cases will aid future risk stratification.
DP17 Alveolar soft part sarcoma: a not-so-common entity
Authors: Li Chia-Lun , Wu Chen-Yi
Published: June 2024

A 24-year-old man presented with an asymptomatic mass on his left thigh that had been present for 1 month. On examination, a well-defined subcutaneous nodule measuring approximately 2.1 × 2.1 cm was observed on his left thigh, with no apparent epidermal change. Magnetic resonance image showed a hyperintense lesion over the left thigh with contrast enhancement. An incisional biopsy revealed nests of eosinophilic cells displaying nuclear pleomorphism, prominent nucleoli, and positive transcription factor enhancer (TFE)3 expression. A diagnosis of alveolar soft part sarcoma (ASPS) was made. ASPS, a rare sarcoma primarily occurring in young patients, typically affects deep soft tissues of the buttock and thigh. While it shows a relatively indolent clinical progression, ASPS often indicates a poor prognosis due to late-stage metastases upon diagnosis. Microscopically, ASPS is characterized by uniform nests of polygonal tumour cells within fibrovascular septa and vascular channels, displaying distinctive cellular dyscohesion, resulting in the characteristic ‘pseudoalveolar pattern’. The cells have defined borders, abundant eosinophilic to granular cytoplasm, and an epithelioid appearance. ASPS is caused by a specific unbalanced translocation, der(17)t(X:17)(p11;p25), forming an ASPSCR1–TFE3 fusion gene. In this patient, further imaging was arranged for tumour staging, which confirmed localized disease without lymph node or distant metastasis. The patient underwent wide excision and local flap surgery and has remained recurrence free during a 4-year follow-up. This case emphasizes the importance of recognizing the clinical features of ASPS in young patients and considering skin biopsy and image studies for long-standing subcutaneous nodules, as this can provide an early diagnosis and improve patient outcomes.
Geriatric DermatologyOral presentationsBG01 The impact of menopause on eczema
Authors: Kamp Erin , Nielsen-Scott Anna , DeGiovanni Claudia
Published: June 2024

The falling oestrogen levels seen in menopause have implications on the structure and function of the skin (Duarte GV, Trigo AC, Paim de Oliveira Mde F. Skin disorders during menopause. Cutis 2016; 97: E16–23). Improvement in skin barrier function and reduction in transepidermal water loss have been seen with transepidermal oestrogen. However, further research is required to understand the role of oestrogen within the epidermis. A range of dermatological conditions have been reported in up to 64% of women attending menopause clinics (Duarte et al.). These include pruritus, xerosis and eczema (Pariath K, Nair PA. A cross-sectional study on the dermatoses in postmenopausal patients at a rural-based tertiary health care center. Indian J Dermatol 2019; 64: 360–5). Our aim was to further understand the impact of the menopause and hormone replacement therapy (HRT) on eczema severity. We sent a survey by text and/or email to patients with eczema aged ≥ 40 years. We received 12 responses from patients with an age range from 40 to 85 years. Overall, 92% patients self-reported as White (11 of 12) and 8% (1 of 12) as Asian or Asian British. The mean age of menopause was reported as 47.3 years (range 35–58). The majority of patients were diagnosed with eczema as a child (9 of 12). Interestingly, three patients were diagnosed with eczema between the ages of 49 and 53 years. Previous and current treatments included emollients, topical corticosteroids, conventional systemics and biologics. One-third of patients reported a deterioration in eczema with or before menstruation. Two-thirds (67%, 8 of 12) of patients’ eczema flared with menopause, and one patient developed eczema for the first time after menopause. None of the 12 patients noticed an improvement in eczema associated with menopause. The majority of patients did not notice a change in their eczema with HRT. Dryness, irritation, contact allergy, sensitivity, skin thinning and nonspecific rash were all noticed by patients with menopause and/or after menopause. Two-thirds of patients who completed our survey reported a flare of eczema with menopause, and we believe this is an exacerbating factor likely to be an under-recognized in the disease. It is vital that further research is carried out to further understand the role of oestrogen within the epidermis and skin barrier and the possible therapeutic benefit of HRT.