bluuebunny/crossref_metadata_2025_split huggingface dataset:
O10 A national retrospective cohort study of the incidence and trends of 3061 cases of cutaneous T-cell lymphoma in England between 2013 and 2020
Authors: Sheern Charlotte , Levell Nick J , Jeffrey Polly , Mistry Khaylen , Scorer Matthew J , Venables Zoe C 
Published: June 2024

Abstract
There are limited epidemiological data available for cutaneous T-cell lymphoma (CTCL). This retrospective, national cohort study reported CTCL incidence from 2013 to 2020, including the early impact of the COVID-19 pandemic. National data were extracted from the ‘Get Data Out’ National Disease Registration Service, haematological malignancies dataset. Crude incidence rates (CIRs) were reported per 100 000 person-years (PY) from 2013 to 2020. Joinpoint regression was used to assess trends and annual percentage change from 2013 to 2019. Incidence rate ratios with Poisson regression were used to assess the early impact of COVID-19 on CIR in 2020 vs. 2019. In total, 3061 new cases of CTCL were diagnosed in England between 2013 and 2020 (mean 383 new diagnoses per year). The CIR was 0.69 PY over the whole period, 0.75 PY [95% confidence interval (CI) 0.68–0.83] in 2013, and 0.60 PY (95% CI 0.54–0.67) in 2020. Over the 8 years analysed, 38% of patients (1153 of 3061) were female and 62% (1908 of 3061) were male (ratio 1 : 1.7). Overall, 61% of patients (1858 of 3061) were aged &lt; 70 years and 39% (1203 of 3061) were aged ≥ 70 years. The annual percentage change from 2013 to 2019 was −1.21% (95% CI −3.52 to 1.15) overall, −2.36% (95% CI −9.65 to 5.30) in those aged &lt; 70 years, and −0.96% (95% CI −4.03 to 2.40) in those aged ≥ 70 years. During the early COVID-19 pandemic, the CIR fell significantly, by 15%, from 0.71 PY (95% CI 0.64–0.78) in 2019 to 0.60 PY (95% CI 0.54–0.67) in 2020 (P = 0.02, incidence rate ratio = 0.85). This is the first and largest national dataset to report the incidence of CTCL in England. The number of diagnoses per year reduced over the period, with a greater reduction seen in patients aged &lt; 70 years; however, these trends were not significant. A fall in incidence was observed during the early COVID-19 pandemic. The time from first presentation to diagnosis of CTCL is approximately 6 years. The data were limited by the complex variety of clinical, histological and molecular features of CTCL, which make diagnosis challenging. Higher incidences have been described internationally, with a CIR of 0.86 PY reported in the USA, and 0.96 PY in France. Cases that did not have a histological diagnosis or dataset completed by a multidisciplinary team meeting may have been missed. Limitations of the study include a lack of data on ethnic groups and CTCL subtypes. These data are essential in expanding our knowledge of the changes in national CTCL epidemiology, and supporting ongoing research.
CPC05 A case of adult-onset en coup de sabre responsive to abatacept
Authors: Diong Sophie , Wynne Bairbre , McMenamin Mairin 
Published: June 2024

Abstract
En coup de sabre (ECDS) is a rare subtype of linear morphoea, located on the forehead or frontoparietal scalp. It is typically seen in children, with &lt; 100 cases of adult-onset ECDS reported. The name derives from the characteristic scar that indents the skin and underlying structures. It can involve the dermis, subcutaneous tissue, muscle, bone and underlying structures, with resulting extracutaneous manifestations including neurological, ocular and oral complications. A 70-year-old female patient presented with a progressive, year-long history of forehead linear atrophy and induration manifesting along a decade-long vertical rhytid. Examination revealed a right paramedian linear atrophic depression with a violaceous rim. This extended posteriorly into her frontal scalp, with associated linear cicatricial alopecia, and inferiorly towards her orbital rim. Histopathology highlighted scarring alongside lymphocytic inflammation and focal subtle vacuolar interface change. Given both the clinical and pathological features, a diagnosis of ECDS was made. Magnetic resonance imaging of her brain revealed no skeletal or parenchymal abnormalities. Ophthalmology examination was normal. Given a history of progression, and the clinical appearance of active disease, immunosuppressive therapy was warranted. Abatacept 125 mg subcutaneously weekly was chosen over methotrexate given the presence of comorbid liver disease. An 8-month course was completed, improving the appearance of the lesion. No further activity has been noted 1 year off treatment. ECDS is a chronic autoimmune fibrosing condition of the skin and underlying tissue with the potential for significant disease-associated morbidity. There is currently no universal diagnostic test for ECDS and no pathological marker that correlates with disease activity. Histology often highlights a perivascular and periappendageal lymphocytic infiltrate, with vacuolar interface changes. All patients should undergo brain imaging to determine the extent of disease. While its aetiopathogenesis remains unknown, there is evidence to suggest that the early inflammatory stages are driven by the T helper (Th)1/Th17 pathway, while the Th2 pathway may be responsible for the fibrosis and damage observed later in the disease. This concept underpins the efficacy seen with abatacept, which inhibits T-lymphocyte activation. Abatacept is a lesser-recognized but important therapeutic option in our armamentarium for managing ECDS. Studies have shown a latency period of &gt; 2 years between symptom onset and diagnosis, likely due to the rarity of this condition and reduced knowledge of adult-onset ECDS. Given the cutaneous and extracutaneous manifestation of this condition, this can have disastrous consequences. Increased awareness of adult-onset ECDS is important to allow for prompt diagnosis, investigation and management.
P012 A delayed diagnosis of neurofibromatosis type 1 during pregnancy
Authors: Dyer Alice , Papadakou Elena , Shah Anjnee 
Published: June 2024

Abstract
Neurofibromatosis type 1 (NF1) is a multisystem autosomal dominant disorder with a prevalence of 1 in 3500 live births. It is caused by mutation of a tumour suppressor gene NF1, located in the long arm of chromosome 17 (17q11.2). There is a 1 : 1 ratio for spontaneous vs. inherited mutations. Mutations resulting in the loss of function of neurofibromin leave cells susceptible to uncontrolled proliferation via overstimulation of protein kinase pathways by unchecked Ras proteins. The common cutaneous manifestations of NF1 – café au lait macules, neurofibromas and intertriginous freckling, appear in most patients by the age of 20 years. Neurofibromas typically first appear during puberty, resulting in questions regarding the role of steroid hormones. To support the hypothesis that steroid hormones contribute to neurofibroma development, we present a new diagnosis of NF1 following the eruption of neurofibromas in a 32-year-old woman during her first trimester of pregnancy. A patient presented in the third trimester of her first pregnancy, with a long history of four unchanged cutaneous papules affecting the right breast. During the first trimester these had increased in number, size and extent of distribution. The patient had no previous history of skin abnormalities, scoliosis, malignancy or developmental delay. There was no family history of NF1. Examination revealed over 50 skin-coloured papules in a segmental distribution involving the entire right arm, right chest and right thigh, as well as five café au lait macules involving the trunk. Histological analysis confirmed neurofibromas, supporting the NF1 clinical diagnosis, which was positive on genetic testing. The pregnancy progressed uneventfully. The patient’s mother subsequently tested positive for NF1 mutation without having any symptoms or signs. In vitro studies have demonstrated that up to 86% of cutaneous neurofibromas possess progesterone receptors; they have also found high levels of heterogeneity when examining the proliferation of NF1 cell cultures in response to steroid hormones. It is unclear why levels of steroid hormones at birth or during puberty are enough to trigger formation of neurofibromas in some patients but not others. This represents an important area for research regarding whether further tumour factors, such as epigenetics, may alter the response to environmental and genetic triggers. Consideration of NF1 as a diagnosis in people of all ages is essential, particularly in view of the increased risks it presents during pregnancy. Patients with NF1 are more likely to develop gestational hypertension, pre-eclampsia and intrauterine growth restriction. Appropriate prenatal counselling and imaging for detection of spinal soft tissue tumours prior to epidural use are additional important aspects of patient management. These were not possible for our patient due to her late diagnosis.
P020 Lentigo maligna: a vanishing act
Authors: Murphy Lisa , Hackett Caitriona 
Published: June 2024

Abstract
The abscopal effect is a phenomenon seen in the treatment of metastatic cancer, whereby the treatment of a tumour with radiotherapy results in the shrinkage of another tumour elsewhere in the body. We report the case of a biopsy-proven lentigo maligna that self-resolved in the setting of radiotherapy for a preceding malignant melanoma on the left chest. Our patient was a 57-year-old man who was originally referred to the clinic with a lesion on his left chest wall. His history was significant for sun exposure, as he had previously worked in the army, and had completed numerous army tours in Lebanon. He was reviewed in clinic, and the lesion was deemed clinically suspicious for melanoma. He was diagnosed with a superficial spreading malignant melanoma on his left chest wall in March 2019 (Breslow thickness 3.6 mm). Wide local excision was negative. A sentinel lymph node biopsy was also performed and this was positive (T3N1M0). He proceeded to have an axillary lymph node dissection. He completed 20 fractions of radiotherapy to his left axillary region in May 2021 and he was commenced on pembrolizumab with the oncology team in July 2021. On routine surveillance review in January 2022, he was noted to have an irregular-shaped lentigo on his right infraorbital cheek. Pigment clods and rhomboidal structures were seen, suggestive of lentigo maligna. An incisional biopsy was arranged, and histology was consistent with a lentigo maligna. He was referred to have this lesion excised. A mapping biopsy was performed in June 2022, and this did not show any evidence of lentigo maligna. He completed his immunotherapy treatment with pembrolizumab in May 2022. At a further follow-up review, the pigmented lesion was no longer clinically evident. It was agreed that an excisional biopsy should be performed to assess for any histological evidence of lentigo maligna. Once more there was no further evidence seen of lentigo maligna on histology. He was reviewed in clinic subsequent to this again, and the site was clear. It is our hypothesis that the lentigo maligna may have resolved due to the abscopal effect, as a result of his radiation treatment for the melanoma on his chest. We suspect that this response would also have been primed by the immunotherapeutic agent pembrolizumab, to help mediate this antitumour response.
P021 The diagnosis and management of pemphigus vegetans in pregnancy
Authors: Clancy Cornelius , Hussain Fawad , Pappa Elena 
Published: June 2024

Abstract
A 31-year-old woman, 16 weeks pregnant at presentation and with no prior medical history, presented to hospital with 2 months of progressive vegetative plaques of the perioral and nasal mucosa, and genital pruritus. After review by medical teams and initiation of treatment for pelvic inflammatory disease, her genital pruritus resolved. However, the patient re-presented to the emergency department 1 week later due to persistence of her facial lesions. Initial diagnoses considered included exuberant granulation tissue in response to infection, nasal papilloma prolapse, pyodermatitis–­pyostomatitis vegetans, and pemphigus vegetans. Due to concerns of facial disfigurement, a biopsy from the lower lip was completed. This revealed mild acantholysis, further confirmed by direct immunofluorescence, with intercellular deposition of IgG and C3 within the intraepidermal space, consistent with a diagnosis of pemphigus vegetans [Messersmith L, Krauland K. Pemphigus vegetans. In: StatPearls. Available at: https://www.ncbi.nlm.nih.gov/books/NBK545229 (last accessed 1 February 2024)]. Published case reports of pemphigus vegetans treatments were reviewed and the options discussed with the patient. Systemic treatment was declined due to associated risks with pregnancy, and instead topical management was initiated with a trial of triamcinolone injections over the course of several months, which significantly improved the patient’s physical appearance. Systemic treatment was initiated postpartum with oral corticosteroids, allowing disease remission with ongoing patient follow-up. This case highlights the diagnostic challenge in diagnosing rare conditions such as pemphigus vegetans in pregnancy, treating severe immunobullous disease during pregnancy, and the psychological impact of immunobullous disease.
P025 Community lesion imaging clinics for tele-triage of suspected skin cancer: implementation of a low-cost model in an NHS trust
Authors: Morgan Hannah , Greenish Huw , Shirwac Hafsa Faarax , Fleming Colin , Anderson Alexander D G 
Published: June 2024

Abstract
Across the NHS there has been a significant and sustained rise in referrals for suspected skin cancer, resulting in a capacity crisis in many NHS trusts. Teledermatology has been successfully used to triage suspected skin cancer referrals nationally, but often involves dedicated medical photographers and expensive equipment. The aim of this study was to report the initial results of tele-triage based on a low-cost community imaging lesion clinic (CLIC) service using dermatology staff and readily available equipment. CLICs were provided in a community hospital setting and staffed by band 3 healthcare assistants. A structured questionnaire was completed and data entered directly into a cloud-based software platform (Islacare, London, UK) using an iPad. The same iPad was used to capture a localizing shot, a close-up macro shot using a readily available clip-on macro lens and a dermoscopic image using a Dermlite DL4. Patients under 18 years of age, those referred with more than three lesions, and patients with genital lesions were excluded. Referral letters, questionnaire data and images were reviewed by a senior dermatologist within 48 h on the Isla platform. In total, 438 patients were reviewed (489 lesions) between August and November 2023. The male-to-female ratio was 1.08, with a median age of 72 years (interquartile range 62–80). Overall, 122 of 438 patients (27.9%) were discharged directly from triage, with advice returned to the referrer. An additional 91 of 438 (20.8%) were identified as suitable for direct listing for biopsy or simple excision. Four of 438 (0.9%) were referred to another specialty following triage, one of 438 (0.2%) had a delayed face-to-face assessment organized, one of 438 (0.2%) declined to participate in tele-triage and requested a face-to-face appointment, one of 438 (0.2%) experienced technical problems, and in one of 438 (0.2%) the site for review was not clear. A potential total of 218 of 438 (49.8%) face-to-face assessments were avoided. The average time for clinician review was 2 min 55 s per patient. Tele-triage using a low-cost CLIC was well tolerated, feasible and effective and resulted in a capacity increase of almost 50%. A significant proportion of patients were managed in a community setting without the need for travel to hospital. One further advantage was the creation of a consecutive series of images and metadata suitable for future study of diagnostic artificial intelligence algorithms or submission to a national dataset.
P030 Implementing the Edmonton Obesity Staging System: a cross-sectional study of the prevalence and management of obesity in a systemic therapies clinic
Authors: Tummon Olga , Gill Rebecca , Murphy Michelle 
Published: June 2024

Abstract
Registry studies from the UK and Ireland report the incidence of obesity among patients on systemic treatment for psoriasis as 40%. This is almost double the background population prevalence of obesity in the UK (26%) and Ireland (23%). Patients with higher body mass index (BMI) have poorer responses to systemic therapies. This study aimed to investigate the prevalence and management of obesity among consecutive patients attending dermatology systemic clinics. Weight and height were recorded, and a patient questionnaire was distributed. Patients identified as living with obesity were classified using the Edmonton Obesity Staging System (EOSS). The EOSS categorizes patients living with obesity into five stages based not solely on BMI but on the presence of obesity-related comorbidities; studies have shown the staging system correlates with clinical outcomes. The study included 113 participants. The median age was 49 years (range 19–79), and 57% (64) of participants were female. The most common diagnosis was psoriasis (77%, 88), followed by atopic dermatitis (16%, 19) and hidradenitis suppurativa (HS) (0.4%, five). Defining a BMI of ≥ 25 kg m−2 as overweight and ≥ 30 kg m−2 as obese, 38% (43) of patients were overweight and 45% (51) were living with obesity. The mean BMI was 31.4 kg m−2, with an SD of 7.3 (range 19.4–53.5). The mean BMI in patients with psoriasis was 31 kg m−2, with eczema 28 kg m−2 and HS 41 kg m−2. Of those living with obesity, 31% (16) fulfilled the requirements for EOSS stage 3, and 32% (37) met the criteria for EOSS stage 2. Hypertension at 31% (36) and mental health issues related to weight at 33% (38) were the two most common self-reported comorbidities. In total, 23% (26) of patients were being prescribed a biologic agent at an increased dose or frequency, and 92% (24) of these patients were either overweight or obese, reflecting the challenges of treating this patient cohort. Overall, 80% (41) of patients in the study classified as living with obesity expressed an interest in accessing medical management for their obesity, while 10% (n = 9) reported that a doctor had discussed obesity management options with them. This is the first study to implement an obesity staging system to examine patients with dermatological diagnoses. The results show that our patients on systemic therapies do not simply have high BMIs but also have physical and psychological consequences. New medical options for the management of obesity have the potential to help many of our patients and, in turn, improve their coexisting dermatological diseases. The EOSS is a valuable tool in time-poor environments to assess obesity and facilitate discussion of management options.
P033 Do suspected cancer pathway referrals to dermatology meet the NICE criteria for referral?
Authors: O’Neill Harriet , Sharma Maulina , Shahidullah Hossain 
Published: June 2024

Abstract
Suspected cancer pathway referrals to dermatology are increasing to an extent that is overwhelming services, yet only 6% of these receive a diagnosis of melanoma or squamous cell carcinoma [Cancer Waiting Times (CWT) urgent suspected cancer referrals: referral, conversion and detection rates. Available at: https://www.cancerdata.nhs.uk/cwt_conversion_and_detection (last accessed 5 February 2024)]. There is an urgent need to identify factors that might reduce inappropriate referrals and improve this conversion rate. We analysed 100 urgent suspected skin cancer referrals from primary care, received consecutively from 1 October. Data were extracted for referring healthcare practitioner, suspected diagnosis and whether the referral was judged to meet the NICE criteria for referral of suspected skin cancers. Only 68% (n = 68) of referrals met the NICE referral criteria for urgent suspected skin cancer. In total, 97% (n = 31) of the referrals that did not meet these criteria had an eventual diagnosis of a benign lesion or nonsignificant basal cell carcinoma (BCC); one referral judged inappropriate had the clinical outcome of a BCC on the nasal tip, which would have met the NICE criteria. Overall, 17% (n = 17) of referrals came from non-general practitioners (GPs) such as physician associates or advanced nurse practitioners. These referrals were less likely to be judged to meet the NICE referral criteria than those from GPs: 41% (n = 7) of non-GP referrals were deemed appropriate vs. 73% (n = 61) of referrals from GPs. Adherence to the NICE criteria for referring suspected skin cancers to dermatology is disappointing. Crucially, less than half of referrals from non-GPs met these criteria, compared with nearly three-quarters of those from qualified GPs. This raises the case for only accepting referrals for suspected skin cancer from qualified GPs. Our evidence suggests that such a stance could potentially lead to a 5% reduction in referrals overall. It is also significant that not one of the referrals deemed to be inappropriate on paper led to an eventual diagnosis of melanoma or squamous cell carcinoma. This suggests that triaging referrals at the pre-appointment stage with the option of querying or even rejecting referrals that do not meet NICE criteria would be low risk. This could potentially reduce urgent suspected skin cancer referrals by 32%.
P069 Familial malignant melanoma: CDKN2A mutation-positive family tree
Authors: Winter Sarah , Ashton Sally , Davies Hannah , Mughal Avad 
Published: June 2024

Abstract
The most common cause of inherited melanoma is mutations in the CDKN2A gene, which is a gene regulator of cell division. The risk of melanoma in carriers of this gene is 30% by 50 years and 67% by 80 years (Goldstein AM, Fraser MC, Struewing JP et al. Increased risk of pancreatic cancer in melanoma-prone kindreds with p16INK4 mutations. N Engl J Med 1995; 333: 970–5). Fortunately, germline mutation in the CDKN2A gene is believed to be rare in the population (Harland M, Cust AE, Badenas C et al. Prevalence and predictors of germline CDKN2A mutations for melanoma cases from Australia, Spain and the United Kingdom. Hered Cancer Clin Pract 2014; 12: 20). Those positive with the CDKN2A gene have associated risks of developing other malignancies such as lung, breast, brain and, in particular, pancreatic carcinoma. Pancreatic cancer is associated with smoking and alcohol-induced pancreatitis. To the best of our knowledge no study has mapped out an extensive CDKN2A mutation-positive family tree including second-degree relatives. We have mapped a family tree including first- and second-degree relatives, stemming from our index patient. She was the first in her family to be diagnosed with malignant melanoma, at the age of 33 years. She tested positive for the CDKN2A gene mutation, BRAF wildtype. Our patient had 10 first-degree relatives. To date, four of these relatives have been diagnosed with malignant melanoma. Her son was diagnosed with malignant melanoma at the age of 12 years and was also found to be positive for the CDKN2A mutation. Four of her other first-degree relatives have been diagnosed with other malignancies: lung adenocarcinoma, squamous cell carcinoma, breast cancer and chordoma. No diagnosis of pancreatic carcinoma has been made in a first-degree relative. There are 21 second-degree relatives, with only one confirmed malignant melanoma. Not all family members have been tested for the CDKN2A mutation, and confounding factors include lifestyle and sun exposure. However, this family tree clearly shows the spread of the condition through a family, with the mutation present in a non-Mendelian pattern. More cohorts need to be collected to accurately predict the risk of malignancy and correction of preventable factors. Being genetically tested for the CDKN2A mutation is beneficial for patients and their families as appropriate counselling can be provided. Patients would be better informed in continued skin self-examination, sun protection and risk of other malignancies.