bluuebunny/crossref_metadata_2025 huggingface dataset:
P34 Changing patterns of skin diseases in patients attending a tertiary private hospital in central Uganda
Authors: Namutebi Fiona
Published: June 2023

There is scarce epidemiological data available on the prevalence of skin disorders in Uganda, a tropical country in sub-Saharan Africa. Skin diseases are a common occurrence in sub-Saharan Africa but receive less attention because they are perceived to cause less mortality. Previous studies have reported a skin disorder prevalence of 30–70% in the general population of developing countries. The majority of studies in sub-Saharan Africa reveal skin infections as the most common dermatology disorder, with a few studies reporting a changing trend toward eczematous disorders. The aim was to investigate the spectrum of skin disorders at a tertiary private healthcare hospital in central Uganda. A cross-sectional study was conducted from September 2021 to August 2022. All patients attending the outpatient skin clinic were recruited. Initial visits were considered. The diagnosis was made clinically and, where necessary, additional investigations were carried out. Data analysis was done with SPSS version 21 (IBM) and Microsoft Excel. A total of 1097 diagnoses were made in 913 patients, of whom 72.9% were adults and 27.1% were under 18 years of age. There were 488 (53.4%) females and a mean (SD) age of 28.9 (16.2) years. The top-five diagnoses were eczematous disorders (n = 223; 20.3%), adnexal disorders (n = 195; 17.8%), infections (n = 182; 16.6%), papulosquamous disorders (n = 93; 8.5%) and pigmentary disorders (n = 59; 5.4%). The results of this research show changing patterns of skin disease presentation from infections in 2016 to eczematous disorders in 2022 as the leading cause of outpatient dermatology consultation at a tertiary private hospital in central Uganda. This calls for proper replanning and budget allocation given the changing spectrum of skin disease presentation at this facility.
P48 Clinical update: ivermectin access for scabies improved through the pharmaceutical benefits scheme change in Australia
Authors: Cox Victoria , Dowden Michelle , Stephens Mike , Currie Bart
Published: June 2023

Scabies is estimated to affect 455 million people worldwide and is a neglected tropical disease that is endemic in northern Australia. Indigenous communities in northern Australia are reported to have some of the highest rates of scabies in the Pacific, with recent prevalence reported to be as high as 25%, with secondary complications including rheumatic heart disease and poststreptococcal glomerulonephritis. However, the high burden of skin disease is still under-reported and under-represented on the national policy agenda. It is essential to consider public health strategies and therapeutic opportunities to address the high burden of disease and minimize serious secondary complications that contribute to the health and life expectancy gap that exists between aboriginal and Torres Strait Islander people and other Australians. Considering the clinical need of the patient population, the management of scabies in northern Australia highlights the impracticality and tolerability issues experienced, with topical permethrin 5% cream as the sole first-line treatment option and use of ivermectin only as a second-line treatment after treatment failure with permethrin cream. This is especially important as poor medication access and treatment failure are well known to perpetuate a cycle of health inequity in northern Australia. A multistakeholder submission to the Pharmaceutical Benefits Advisory Committee was developed to support a change to the listing of ivermectin on the Pharmaceutical Benefits Scheme (PBS) in Australia, and to recommend ivermectin as a first-line treatment option for the management of scabies in aboriginal and Torres Strait Islander people in Australia. The PBS listing was successfully changed after 9 months of review, and from 1 April 2022, oral ivermectin has been listed as a first-line treatment for the management of scabies in aboriginal and Torres Strait Islander people in Australia. It is anticipated that the change in national prescribing will have a large impact on a small number of Australians who face significant disadvantages and health inequity and will improve access of treatment to manage effectively a condition that disproportionately affects First Nations people in Australia. The successful PBS listing change occurred ahead of the release of the new edition of the Australian clinical guidelines, which at the time of writing are scheduled to be released in early 2023. The updated guidelines will include ivermectin as a co-first-line treatment alongside topical permethrin 5% cream for the management of scabies, in addition to considering an ivermectin-based mass-drug administration programme for endemic regions in which the burden of scabies is estimated to be > 10%, as recommended by the World Health Organization.
P58 Sustained improvement in malignant melanoma prognosis at a consultant-delivered multidisciplinary pigmented lesion clinic: a follow-up study
Authors: McDonnell Róisín , Kiely Lisa , Brennan Aline , McDevitt Joe , Murray Deirdre , Murphy Michelle , Bennett Mary , Bourke John
Published: June 2023

Rapid-access clinics are designed to improve the early detection and prognosis of melanoma, but there is little hard evidence in the literature of their effectiveness. We established a pigmented lesion clinic (PLC) in 2002 and have previously demonstrated a subsequent reduction in melanoma thickness at our hospital. We undertook a retrospective cohort study to assess the long-term effects. Data for all melanomas diagnosed in the Republic of Ireland between 1 January 1998 and 31 December 2019 were provided by the National Cancer Registry Ireland (n = 15 619). We excluded T0 and Tis and unknown T melanomas. Our PLC was found to have a higher proportion of tumours diagnosed at an earlier stage of T1 (51.6%) vs. the region/county where the PLC is based (37.8%) and Ireland (40.3%). Following the introduction of the PLC, from 2003 to 2007, a significant difference was noted between our PLC and the rest of Ireland, with a higher proportion of T1 tumours diagnosed (41.6% vs. 29.7%; P < 0.001). For the region, the percentage of T1 tumours was lower than for the rest of the country, but this difference fell short of significance (34.5%; P = 0.057). In the two 5-year periods following this (2008–2013 and 2014–2019), our PLC had significantly more early tumours (44.4% and 62.1%, respectively) than the rest of Ireland (35.9% and 52.2%, respectively; P < 0.001 for both time periods). More PLCs have been established across the country, probably accounting for the higher proportion of early-stage melanomas diagnosed in 2014–2019. The figures for the region were also significantly better than those for the rest of the country [40.7% and 55.7% (P = 0.008 and P = 0.039), respectively], suggesting that the PLC has had an effect on prognosis, resulting in a greater proportion of early melanomas in the region vs. Ireland. Preliminary analysis found a hazard ratio (HR) of 0.78, or a 22% reduced risk death, at 5 years (unadjusted Cox proportional hazard regression on all nonmetastatic melanoma with known T diagnosed 1998–2019, n = 16,489) for those attending the PLC vs. the rest of Ireland. Patients in the rest of the region were found to have a higher HR (1.15) vs. the rest of Ireland. The PLC appears to have contributed to improved prognosis in the region in the years following its establishment. This study supports the long-term benefits of a PLC over the course of more than 20 years.
P59 Results of sentinel lymph node biopsies in thin melanomas (Breslow thickness 0.8–1.0 mm) and correlation with histopathological features
Authors: Kenny Bernard Anton , Durack Alana
Published: June 2023

In July 2022, the National Institute for Health and Care Excellence (NICE) melanoma guidelines were updated to include recommendation for a sentinel lymph node (SLN) biopsy for those with a Breslow thickness of 0.8–1 mm and at least one of the following: ulceration, lymphovascular invasion and/or a mitotic index of ≥ 2 mm². SLN biopsy is a staging procedure and therefore has an impact on prognosis. However, SLN biopsy is an invasive procedure with associated morbidity. A 5-year retrospective review of melanoma data in the south east of Ireland was undertaken to establish the correlation between positive sentinel lymph node biopsies in 0.8–1.0-mm-thick melanomas and the risk factors outlined above. Melanoma outcomes, histopathology reports and outpatient clinic letters were assessed and data were extracted from 1 January 2018 to 31 December 2022 inclusive. A total of 52 patients were identified with a melanoma of Breslow thickness of 0.8–1 mm from the predetermined timeframe. Eight per cent (n = 4) of lesions were ulcerated. Thirteen per cent (n = 7) had a mitotic index of ≥ 2 mm². One lesion was both ulcerated and had a mitotic index of ≥ 2 mm². No lesions demonstrated lymphovascular invasion. Nineteen (36%) patients underwent SLN biopsy. All patients who underwent SLN biopsy were negative. One patient with an ulcerated melanoma did not undergo SLN biopsy and five with a mitotic index of ≥ 2 did not undergo SLN biopsy. There was no evidence of disease recurrence within the follow-up period (2 months–5 years). This population showed a 0% positivity rate on SLN biopsy. No correlation with mitotic index or ulceration and SLN biopsy was identified. No patients have developed local recurrence or metastatic disease within the follow-up period. This study supports the updated NICE guidance recommending SLN biopsies for thicker melanomas (i.e. Breslow thickness > 1.0 mm) and SLN biopsy in thinner melanomas (Breslow thickness 0.8–1 mm) only if concerning features are present on histology (i.e. ulceration, lymphovascular invasion and/or a mitotic index of ≥ 2).
P63 Effectiveness of an image-analysing artificial intelligence-based digital health technology to diagnose nonmelanoma skin cancer and benign skin lesions
Authors: Marsden Helen , Palamaras Ioulios , Kemos Polychronis , Greenhalgh Jack
Published: June 2023

Squamous cell carcinoma (SCC) and basal cell carcinoma (BCC) are common types of nonmelanoma skin cancer (NMSC). The DERM-003 study was a prospective, multicentre, single-arm, masked study that aimed to demonstrate the effectiveness of an artificial intelligence-based digital health technology (AI-DHT) to identify SCC, BCC and premalignant conditions in dermoscopic images of suspicious skin lesions. Patients with at least one suspicious skin lesion that was suitable for photography were eligible. Each lesion was photographed with three smartphone cameras (iPhone 6S, iPhone 11 and Samsung 10) with a dermoscopic lens attached. Each image was assessed by the AI-DHT. A clinical diagnosis was made by the dermatologist, and histopathology results were obtained for biopsied lesions. The AI-DHT output was compared with the histopathology diagnosis, and the area under the receiver operating characteristic curve (AUROC) was calculated. Secondary endpoints included other diagnostic measures and assessment of premalignant lesions. Altogether, 572 patients (49.5% female, mean age 68.5 years, 96.9% Fitzpatrick skin types I–III) were recruited from four UK National Health Service trusts, providing images of 611 suspicious lesions. There were no exclusion criteria relating to skin type. In total, 592 lesions had images from all three cameras available; only one lesion had no images available. Altogether, 395 lesions had a histopathology result. Forty-seven biopsied lesions were diagnosed as SCC and 184 as BCC. The AUROCs for images taken by the iPhone 6S were 0.88 [95% confidence interval (CI) 0.83–0.93] for SCC and 0.87 (95% CI 0.84–0.91) for BCC. For Samsung 10, the AUROCs were 0.85 (95% CI 0.79–0.90) and 0.87 (95% CI 0.83–0.90), and for iPhone 11, they were 0.88 (95% CI 0.84–0.93) and 0.89 (95% CI 0.86–0.92) for SCC and BCC, respectively. Using images taken on iPhone 6S of biopsied-only lesions, the sensitivity rates in detecting SCC and BCC were 98% (95% CI 88–100) and 94% (95% CI 90–97), respectively; the specificity rates were 38% (95% CI 33–44) and 28% (95% CI 21–35), respectively; the positive predictive values were 17% (95% CI 16–19) and 59% (95% CI 56–61), respectively; and the NPVs were 99% (95% CI 95–100) and 81% (95% CI 70–89), respectively. All 12 lesions diagnosed as Bowen disease were classified correctly by the AI-DHT. Of the 61 lesions diagnosed as actinic keratosis, 52 were correctly classified of the 22 lesions diagnosed as dysplastic and 18 were correctly classified by the AI-DHT. All 16 lesions diagnosed as melanoma were classified as such by the AI-DHT. The AI-DHT has the potential to support the diagnosis of NMSC and premalignant lesions.
P67 Overall lifetime risk for alopecia areata is 2.1% and is higher in women, non-White ethnic groups and the most deprived: a UK population-based cohort study
Authors: Thompson Andrew R , Tziotzios Christos , Nesnas John , Randall Rowena , Czachorowski Maciej , Messenger Andrew
Published: June 2023

Alopecia areata (AA) is an immune-mediated nonscarring alopecia often associated with substantial morbidity. However, there are limited population-based data on potential disparities in the burden of AA, including across different ethnicities and sociodemographic classifications. We aimed to provide the first large-scale, population-based evaluation of lifetime risk of AA across sociodemographic subgroups. This study used routinely collected data from a large UK population-based database: the Oxford-Royal College of General Practitioners (RCGP) Research and Surveillance Centre (RSC). All children and adults registered with general practices contributing to the RCGP RSC network in the period 2009–2018 without a prior diagnosis of AA were included. People were considered to develop AA if they had an AA diagnosis and, in the subsequent 365 days, no code for an alternative diagnosis (e.g. traction alopecia and scarring alopecia). The cumulative lifetime risk of AA was estimated at age 80 years using survival models, with age as the timescale and accounting for the competing risk of death. Lifetime risk was calculated for the whole population and by sociodemographic subgroups comprising sex, major UK ethnicity groups and socioeconomic deprivation. In a total population of 4 052 231, 6961 developed new-onset AA. The lifetime risk in the overall population was 2.11% [95% confidence interval (CI) 2.06–2.16]. There was substantial difference in lifetime risk of AA by ethnicity; lifetime risk was 1.74% (95% CI 1.68–1.80) in the White subgroup, 5.87% (95% CI 5.51–6.24) in Asian, 3.03% (95% CI 2.63–3.42) in Black, 4.44% (95% CI 3.50–5.37) in mixed and 4.47% (95% CI 3.63–5.31) in other ethnic groups. Females had a higher lifetime risk of AA than males: 2.35% (95% CI 2.28–2.43) vs. 1.88% (95% CI 1.81–1.94). Lifetime risk was greater with increasing socioeconomic deprivation: least-deprived 1.68% (95% CI 1.59–1.78) vs. most-deprived quintile 2.92% (95% CI 2.77–3.07). Our findings demonstrate that AA affects nearly 1 in 50 people over a lifetime. Lifetime risk is substantially greater in people of non-White ethnicity and is highest in those of Asian ethnicity (one in 17 people). The lifetime risk is also higher in females and those with the greatest socioeconomic deprivation. Clinicians should be aware of the disproportionate distribution of AA across the population and support the provision of healthcare resource to groups with the highest burden.

Funding sources: this research was sponsored by Pfizer. Project management, medical writing and statistical support were provided by Momentum Data, UK, and were funded by Pfizer.
P79 A multidisciplinary expert consensus on the assessment of and support for mental health in children and young people living with skin conditions
Authors: Moledina Zahra , McPherson Tess , Ravenscroft Jane , Ali Rukhsana , Baron Susannah
Published: June 2023

Psychological and mental health concerns are common in children and young people (CYP) living with skin conditions and can have a profound impact on wellbeing. However, there is limited guidance on how best to assess and support the mental health of this population, who are at risk of poor health outcomes. The British Society for Paediatric and Adolescent Dermatology (BSPAD) formed a working group to address the mental health needs of CYP with skin conditions, including hair and nails. A systematic review, appraisal of literature and a national survey were carried out, and a multidisciplinary consensus guideline group was convened, comprising dermatologists with expertise in paediatric dermatology and psychodermatology, psychologists, psychiatrists, general practitioners, paediatricians and specialist nurses. Two multidisciplinary virtual panel meetings took place: (i) an initial meeting to discuss the scope, review the current evidence and identify areas for development and (ii) a second meeting to agree the content and wording of the recommendations. The primary objective was to provide consensus-based guidance on the assessment and monitoring of, and support for, mental health in CYP with skin conditions. Secondary objectives were to address important practical clinical questions relating to primary guidance objectives and provide audit and research recommendations. The expert panel achieved consensus on 11 recommendations for health workers managing CYP with skin conditions. A new patient-completed history-taking aid called ‘You and Your Skin’ was developed and is being piloted. The recommendations focus on improved mental health assessments for CYP presenting with a skin condition, with clinical guidance and suggested screening measures included. Information is given regarding accessing psychological support for CYP when required, and recommendations were provided for training in mental health and neurodiversity for staff. The methodology and recommendations will be presented in full. Embedding a psychosocial approach within services treating CYP with skin disease should ensure that CYP with psychological needs are able to be identified, listened to, supported and treated. This is likely to improve health outcomes.
P87 Therapeutic outcomes in xanthoma disseminatum with cladribine: a long-term experience of six patients from a tertiary care institute
Authors: Taneja Neha , Sharma Ananya , Sharma Ritu
Published: June 2023

Xanthoma disseminatum is a rare, non-Langerhans-cell histiocytosis with systemic involvement that has limited treatment options and response. Cladribine has been previously reported in few case reports and a single case series to induce long-term remission of cutaneous lesions. The aim was to study the treatment outcomes in a long-term follow-up of patients with xanthoma disseminatum, treated with cladribine. This was an ambispective case series of six patients diagnosed with xanthoma disseminatum on the basis of clinical and histopathological features. All patients were treated with monthly cycles of cladribine (2-chlorodeoxyadenosine) 0.14 mg kg−1 daily for 5 days a month, ranging from two to eight cycles. The follow-up period ranged from 2 months to 4.75 years. There were four males and two females in our series, with mean (SD) age of 25.3 (13.1) years (range 9–48). Disease duration at presentation ranged from 1.5 to 8 years. All patients presented with skin-coloured to yellowish-brown papules in a periorbital, perioral and flexural distribution. Four of six (67%) patients had mucosal involvement. All but one patient had associated diabetes insipidus. Half the patients had bone involvement, and two showed extensive infiltrates in the brain causing neurological symptoms. Patients receiving at least three cycles (n = 5) reported a mean improvement of 72% in cutaneous lesions at the end of the follow-up period, allowing for the avoidance of tracheostomy in a patient with laryngeal involvement. Imaging (magnetic resonance imaging of the brain, positron emission computed tomography) repeated in four patients after 3–6 cycles showed evidence of significant improvement in extracutaneous disease. Adverse effects were noted in four (67%) patients, including transient leukopenia in three, post-infusion fever in one and proximal renal tubular acidosis in one. All side-effects were resolved after the cessation of therapy and/or with supportive treatment; no serious infections were reported. Cladribine is a purine analogue that has an antiproliferative effect on histiocytes and lymphocytes, as well as an immunomodulatory action. It has emerged as an effective therapeutic modality for patients with xanthoma disseminatum that necessitates further evaluation for dosage and safety.
P96 Nutrition in dermatology practice: an unmet need for both patients and healthcare professionals
Authors: Lockwood Katie , Dhariwal Sukhjit , Hall Wendy , Gibson Rachel , Griffiths Christopher , Ahmed Alia , Maruthappu Thiviyani
Published: June 2023

The role of diet and nutrition in the management of chronic inflammatory skin conditions such as eczema, psoriasis and acne is one of the commonest questions asked by patients. Despite this, there is little guidance or training to help healthcare professionals provide disease-specific evidence-based recommendations to patients. Furthermore, growing evidence supports the role of diet and nutrition in the management of skin conditions such as psoriasis and atopic eczema. We sought to explore dermatology professionals’ experiences of patients’ dietary habits in an outpatient setting. We circulated a questionnaire to members of the British Dermatological Nursing Group and the British Association of Dermatologists. We collected demographic data, along with information on how frequently professionals were asked about diet by patients, how confident they felt responding to questions and concerns expressed regarding dietary restrictions, and whether dietitian input or nutrition training would be of benefit to their practice. Three of the authors received funding from the Psoriasis Association. We received 95 responses from dermatology nurses and 64 from doctors. Over 60% of respondents held senior positions with over 10 years of experience. Almost 100% of respondents reported being asked about diet by patients and 73.1% did not feel confident when answering these questions. Patients were most commonly asked about nutrition in relation to inflammatory conditions such as eczema (95.3%), acne (95.3%) and psoriasis (76.6%). All doctors and 97.8% of nurses reported having experienced patients restricting their diet without supervision, and the majority expressed concerns about such dietary modifications. Dairy, gluten and refined sugar were the most commonly excluded foods. Professionals also reported that patients admitted to following restrictive diets and undertaking ‘food intolerance testing’ that they had encountered online. Over 90% of respondents felt that additional nutrition training and access to specialist dietician support would be of benefit to dermatology practice. Dermatology professionals are frequently asked about nutrition and the role of diet yet often lack confidence in responding to these enquiries. Dermatology patients appear vulnerable to dietary misinformation presented online regarding ‘intolerance tests’, and this has the potential to worsen with ongoing delayed access to secondary care. There are also concerns regarding unsupervised restrictive diets, which can negatively impact patients’ mental health and predispose to eating disorders and nutritional deficiencies. Specialist nutrition training for dermatology professionals and the support of dietitians in the clinical setting would help address this unmet need and provide holistic patient care.
P104 Use of intravenous antibiotics in severe hidradenitis suppurativa: a multicentre retrospective study
Authors: Tan Xiang Li , Aziz Sally , Rashidghamat Ellie , Hemsley Carolyn , Goldenberg Simon , Lamb Ruth
Published: June 2023

Hidradenitis suppurativa (HS) is a chronic inflammatory condition, characterized by recurrent abscesses and sinus tracts in intertriginous areas. Intravenous (IV) antibiotics are used in cases of severe HS after the failure of conventional therapies, or to optimize patients prior to initiation of biologics or surgery. However, there is limited evidence on the efficacy of IV antibiotics [Join-Lambert O, Coignard-Biehler H, Jais J-P et al. Efficacy of ertapenem in severe hidradenitis suppurativa: a pilot study in a cohort of 30 consecutive patients. J Antimicrob Chemother 2016; 71:513–20; Ingram JR, Collier F, Brown D et al. British Association of Dermatologists guidelines for the management of hidradenitis suppurativa (acne inversa) 2018. Br J Dermatol 2019; 180:1009–17]. Our aim was to determine the immediate and long-term impact of IV antibiotics in severe HS and to develop a pre- and post-IV antibiotics assessment and care pathway. We conducted a multicentre retrospective review of patients with HS treated with IV antibiotics at two tertiary HS centres between 2013 and 2022. In total, we included 30 patients (14 females and 16 males), with a mean (SD) age of 42.6 (12.8) years (range 15–66) and a mean (SD) body mass index of 29.96 (7.31) kg m−2. All patients were classified as Hurley stage 3. The majority of patients received ertapenem (n = 24); other antibiotics included ceftriaxone (n = 5) and meropenem (n = 1). Patients had an average of 38.7 days on treatment (range 14–56). There were statistically significant improvements at the completion of antibiotics in biochemical and haematological parameters, including C-reactive protein −27.5 mg L−1 [95% confidence interval (CI) −11 to −57; P < 0.003], white cell counts −1.75 × 109 cells L−1 (95% CI −0.95 to −2.80; P < 0.001) and haemoglobin +7 g L−1 (95% CI 2.5–13; P < 0.005). Dermatology Life Quality Index (out of 30) also improved significantly at completion (−5, 95% CI −1 to −10; P < 0.008). These were all maintained 12 months postcompletion. Clinical severity of HS assessed through pain visual analogue score (VAS), discharge VAS, DERMVAS, overall VAS, and HS Physician Global Assessment showed improvement but did not reach statistical significance. Improvement in discharge VAS [out of 10; −2, 95% CI −1.5 to −4.5 (P < 0.066)] achieved borderline significance. To our knowledge, this is the largest analysis of the efficacy of IV antibiotics in patients with the most severe form of HS (i.e. Hurley stage 3), where we show improvement in clinical parameters and quality of life. The findings of this study should encourage a randomized controlled study to determine conclusive evidence to guide the evidence-based management of severe HS and have informed the development of a more formal assessment pathway.
BG09 RASA1 exon deletion in a neonate with vascular malformations
Authors: Mann Nina , Solman Lea , Searle Claire , Srinivasan Jothsana , Ravenscroft Jane C
Published: June 2023

A full-term infant was noted to have a large, macular, pale-pink vascular birthmark across the left side of the chest, extending to the upper arm. Three smaller pale-pink irregular birthmarks on the elbow, thigh and flank were noted, as was an enlarged left upper arm. He had an oxygen dependency shortly after birth for which he required admission to the neonatal unit. A detailed history revealed that his father, paternal grandmother and aunt had similar birthmarks. His paternal aunt had an episode of bleeding from a spinal arteriovenous malformation (AVM) in her teens, which had been investigated with genetic testing by Sanger sequencing, but this did not identify a variant in RASA1. Following involvement from the dermatology, neonatology, genetics and radiology teams, investigations were organized. Findings included a high-flow vascular malformation with arteriovenous shunting on magnetic resonance imaging (MRI) of the left arm, normal MRI head and spine, and normal cardiac anatomy. Genetic testing by next-generation sequencing detected a heterozygous exon 21–23 deletion in RASA1. A diagnosis of capillary malformation–AVM syndrome (CM–AVM) was confirmed. Results of genetic testing of the father are awaited. Referral to a tertiary centre for consideration of management of AVM is underway. CM–AVM is a rare autosomal dominant condition. Pathogenic variants in RASA1 result in a lack of functional p120-RasGAP protein, which leads to abnormalities in angiogenesis, although this pathway is not well understood (Wooderchak-Donahue W, Johnson P, McDonald J et al. Expanding the clinical and molecular findings in RASA1 capillary malformation-arteriovenous malformation. Eur J Hum Genet 2018; 26:1521–36). Cutaneous vascular malformations, classically pink–red with a pale halo, are the most consistent cutaneous manifestation, but they may be subtle and can mimic other vascular birthmarks such as segmental infantile haemangioma, salmon patches or port wine stain. In our patient, pale halos were not apparent until 4 months of age. Identification is important as life-threatening complications from cutaneous vascular arteriovenous fistulas can occur (Wooderchak-Donahue et al.). This case highlights the importance of clarifying a family history, classifying vascular birthmarks and working with the multidisciplinary team. Genetic testing should be considered, even with previous negative analysis, as test sensitivity has improved and will now include analysis of EPHB4, which has also been associated with CM–AVM.
BG10 A case of pachydermoperiostosis (primary hypertrophic osteoarthropathy)
Authors: Brindley Luke , Hunjan Manrup , Lewis Helen , Lovgren Marie-Louise , Titheradge Hannah
Published: June 2023

We report a case of a 21-year-old man who was referred from the community with concern about persistent swollen and inflamed eyelids together with possible facial acne. He was known to have hypertrophic osteoarthropathy under the care of rheumatology for which he was on regular infliximab infusions. He had previously been treated with methotrexate (intolerant) azathioprine and adalimumab (lack of efficacy). He also had a long-standing history of inflammatory bowel disease and associated complications: iron-deficiency anaemia, previous choledochal cyst and biliary stricture, as well as previous deficiencies in vitamins B12, A and E. The patient was known to the genetics team, with prior genetic testing demonstrating that he is homozygous for a pathogenic SLC02A1 variant (c.1264_1265delAC). This gene encodes a prostaglandin transporter involved in mediating the uptake and clearance of prostaglandins in numerous tissues. On examination, he was found to have pronounced thickening of the facial skin, especially the forehead, alongside deep furrowing of the scalp. The eyelids were severely swollen and inflamed, with prominent straight eyelashes. There were milia present on the face predominantly on the brow and cheeks. There was clubbing of all digits on his hands and feet. The cutaneous findings were deemed all in keeping with pachydermoperiostosis. Pachydermoperiostosis, also known as primary/idiopathic hypertrophic osteoarthropathy, Touraine–Solente–Gole syndrome and Rosenfeld–Kloepfer syndrome, was first described by Friedrich in 1868. This is a rare condition, and both autosomal dominant and autosomal recessive modes of inheritance have been noted. The condition usually presents during puberty and the hallmark features are thickening and coarsening of the facial skin, periostosis and digital clubbing. Other cutaneous features can include seborrhoea with sebaceous hyperplasia, acne, hyperhidrosis and cutis verticis gyrata. Ocular manifestations include blepharoptosis, floppy eyelid syndrome, and hypertrophy of both the eyelids and palpebral conjunctiva. Crohn disease and multiple bone and joint issues are known features of the condition (Doshi D. Touraine–Solente–Gole syndrome. Orbit 2018; 37:97–101; Tabatabaei SA, Masoomi A, Soleimani M et al. Pachydermoperiostosis: a clinicopathological description. J Curr Ophthalmol 2019; 31:450–3). This reported case demonstrates both classic cutaneous and other organ system features of this rare genetic condition. It highlights the importance of being able to recognize all the associated findings when a patient presents with thickening of their facial skin and eyelids.
BI04 Cutaneous cryptococcosis: a post-transplant cat among the pigeons
Authors: Patel Majeeda , Cernova Jeva , Mabayoje Diana , Rajakariar Ravindra , Warrens Anthony , Harwood Catherine
Published: June 2023

Cryptococcosis is an infection caused by the encapsulated yeasts Cryptococcus neoformans and Cryptococcus gattii, which are opportunistic pathogens that mainly affect immunocompromised individuals. Infection is primarily by inhalation and causes pulmonary disease; haematogenous dissemination may affect other organs, particularly the central nervous system, with 10% of disseminated cryptococcosis affecting the skin. Primary cutaneous cryptococcosis due to direct skin inoculation is less common and rarely disseminates. We describe a kidney transplant recipient with disseminated cryptococcosis who presented with skin lesions in the early post-transplant period. As previously observed in a transplant patient with primary cutaneous cryptococcosis under our care, we propose that a history of close contact with urban pigeons was the source of infection. A 48-year-old man who had been transplanted in 2014 presented in May 2022 with a 3-cm-diameter, well-demarcated ulcer on his right shoulder which had been slowly enlarging over 6 weeks in close proximity to his recent COVID-19 vaccination site. Swab was positive for C. neoformans, serology was positive for cryptococcal antigen, chest X-ray showed a 14-mm nodule in the right lung and magnetic resonance imaging of the brain was normal. He was admitted by the transplant team and treated for presumed disseminated cryptococcal infection with intravenous AmBisome® and flucytosine for 2 weeks, and subsequently oral fluconazole. In view of his atypical presentation, we directly questioned him about previous skin disorders and possible sources of infection. It emerged that 6 months earlier, he had noticed multiple lesions on his legs but did not report this as they spontaneously resolved. Mobile phone images showed 0.5–1-cm umbilicated, red–purple nodules consistent with possible primary cutaneous cryptococcosis. We discovered that his balcony was covered in pigeons and their droppings, and he had unsuccessfully asked his local council to provide bird netting. This was the most likely source of his infection, although it is uncertain whether his skin involvement was primary, secondary or both, due to ongoing pigeon exposure. A 54-year-old female patient under our care who had previously presented 3 months post-transplant with extensive noduloulcerative umbilicated lesions confirmed on biopsy to be primary cutaneous cryptococcosis also had close contact with pigeons and their droppings in the immediate post-transplant period. The incidence of cryptococcosis in immunocompromised patients is increasing and cutaneous involvement may be the first manifestation of disseminated disease. The clinical spectrum of skin lesions is highly polymorphic, making diagnosis challenging. However, it should be considered in immunocompromised patients presenting with atypical skin lesions, particularly in the early post-transplant period.
BI07 Outcomes of oral squamous cell carcinoma following haematopoietic stem cell transplantation
Authors: Zaki Faaris , Shephard Martina , Danby Robert , Davies James , Pawson Rachel , Peniket Andy , Matin Rubeta
Published: June 2023

Haematopoietic stem cell transplant (HSCT) recipients are at increased risk of malignancies, including cancers of the oral cavity, with a reported observed-to-expected ratio of 10.7, accounting for 14% of all solid tumours following HSCT. We report 10 cases of oral squamous cell carcinoma (SCC; 2016–2022) in HSCT recipients from a single tertiary centre [nine men and one woman; median age at diagnosis 52 years (range 27–66)]. Thirty per cent were ex-smokers and 10% current smokers. Prior to HSCT, 50% had reduced intensity conditioning, 40% had myeloablative conditioning and 10% had both. Eighty per cent were recipients of sibling allografts and 20% were recipients from matched unrelated donors. Ninety per cent had a history of chronic graft-versus-host disease (GVHD), with affected sites including the mouth (n = 6), skin (n = 6), eyes (n = 5), lungs (n = 4), kidneys (n = 3) and liver (n = 1). Forty per cent had active severe manifestations of oral GVHD. Treatments used for oral GVHD included topical immunosuppression (n = 5), oral immunosuppression (n = 6) and extracorporeal photopheresis (ECP; n = 2). Median latency from HSCT to oral SCC diagnosis was 10 years (range 4–22). At the time of SCC diagnosis, 20% were not taking immunosuppressants, 20% were being treated with topical corticosteroids and 50% with oral immunosuppression, and one patient was recently treated with ECP. Examination findings at SCC diagnosis included induration, ulceration, tenderness, bleeding, hyperkeratosis, speckling and lymphadenopathy (n = 1). Fifty per cent were asymptomatic. In 40% of cases, a previous biopsy showed hyperplastic or dysplastic changes. The commonest sites of SCC were the tongue (n = 4) and buccal mucosa (n = 3). The disease stage (TNM) at presentation ranged from T1N0M0 to T4N2M0. Sixty per cent of cases were T3 or T4 and 40% had nodal involvement. Management included radical surgical resection in 90% of cases; 10% received adjuvant radiotherapy and 10% chemoradiotherapy. One patient with an SCC on the lower lip underwent resection using Mohs micrographic surgery. Median duration of follow-up for the cohort was 0.8 years (range 0.4–5.7) with a 50% mortality rate. In our cohort, median survival following oral SCC diagnosis was 0.8 years (range 0.4–5.0) and SCC-specific mortality was 30% (n = 3). We report the largest UK case series, to the best of our knowledge, of oral SCC post-HSCT. The data show a long latency from HSCT to the onset of oral SCC, highlighting the importance of regular, active oral surveillance by haematologists, dermatologists, oral specialists and dentists. This follow-up is recommended irrespective of the severity of GVHD and length of iatrogenic immunosuppression. Intensive surveillance is indicated in groups at higher risk with a history of chronic oral GVHD.
BI10 ‘I never thought it would happen to me’. Photoprotection and skin cancer education: a qualitative study of the experiences of Irish postrenal transplant patients. Why the ‘personal touch’ is so important
Authors: Griffin Laoise , Laing Mary , O’Reilly Pauline , Griffin Matthew
Published: June 2023

Renal transplant recipients (RTRs) are at an increased risk of skin cancer (SC), especially squamous cell carcinoma (SCC). As the number of successful transplant recipients increases, pressure on dermatology services increases. The focus has shifted from simply screening RTRs for SC to implementing preventive measures, including SP education programmes. Sun exposure is a modifiable risk, yet RTRs are known to adopt sun-protective (SP) behaviour only partially. There is a dearth of research on why this is. We performed an in-depth qualitative study using inductive thematic analysis of 14 semistructured interviews of seven male RTRs to assess the impact of the current SP education and explore their current SP behaviour. Participants included those with SC, premalignant skin disease and no SC. Two themes were constructed. Each theme had two subthemes. The first theme was ‘Timing it Right—Skin cancer information’, with the subthemes of ‘A time of great emotion’ and ‘Providing knowledge—learning needs differ’. Choosing a suitable time to impart information on SC is important. Immediately prior to and on receiving a kidney transplant was not felt by patients to be the most appropriate time. Most felt unable to consider perceived future risks to their health during this critical period. When delivered, recipients expressed preference for an ‘in-person’ approach with supplementary information available (online and written). The second theme was ‘The person within the patient’, with the subthemes of ‘What matters to the patient’ and ‘Authentic engagement’. It is important to see the person within the patient. Each brought with them different worries and requirements, sometimes feeling guilty when expressing them as they had received a new ‘gift of life’. Participants knew what constituted a good health education experience for them. The healthcare professional who was compassionate and listened was valued and more likely to influence behaviour. Interviewees spoke about the strong bond they form with their renal teams and how they trust them. Misconceptions were also uncovered such as the belief that the Irish sun differed from that experienced abroad and that SP was just for ‘sunny days’. An overriding concern for developing melanoma rather than cutaneous SCC was evident, as was the lack of confidence in ability to self-monitor skin. SC prevention programmes are becoming increasingly well established; our study identified gaps in the knowledge of RTRs and highlighted some key areas and approaches to improve the impact of educational interventions. To maximize the impact, it is important to consider patient perspectives, cultural context and the key role our renal colleagues play in patients’ lives.

Funding: support was received from The City of Dublin Skin and Cancer Hospital Charity (registered Charity Number: 20004620).
BI11 Cutaneous presentation of Kaposi sarcoma following allogeneic haematopoietic stem cell transplantation for myeloma
Authors: McGrath Conn , Thomas Bjorn , Duncan Mhairi , Basu Tanya , Benjamin Reuben , Child Fiona
Published: June 2023

Iatrogenic Kaposi sarcoma (KS) is a relatively frequent occurrence following solid-organ transplants (0–5%), in part due to prolonged immunosuppression. However, its occurrence after haematopoietic stem cell transplantation (HSCT) is rare, with only 15 cases described in the literature. We report a case of KS presenting in the skin of a patient who had received a matched unrelated donor allogeneic HSCT for IgG kappa myeloma. A 55-year-old HIV-negative white man with myeloma underwent an allogeneic HSCT from a matched, unrelated donor following conditioning chemotherapy with fludarabine–melphalan and antithymocyte globulin. Post-transplant, he was maintained on ciclosporin as graft-versus-host disease (GVHD) prophylaxis. On day 14 post-transplant, he developed a widespread eczematous eruption that rapidly evolved into an erythroderma; a skin biopsy was consistent with acute GVHD. He was managed with superpotent topical steroids and a 5-day course of oral prednisolone. He began extracorporeal photopheresis (ECP) on day 33 post-transplant, having failed to respond adequately to systemic steroids. He also completed four cycles of rituximab. Ciclosporin was stopped by day 130 post-transplant. He had completed 14 cycles of ECP by day 139 post-transplant, with resolution of cutaneous GVHD; however, he had developed a rapidly growing solitary dusky-red nodule on his right neck that was excised due to diagnostic uncertainty. Histology revealed a dermal haemorrhagic exophytic vascular tumour composed of lobules and sheets of epithelioid cells with an eosinophilic cytoplasm and pleomorphic nuclei. Immunoperoxidase staining was negative for S100, MNF-116 and AE1/AE3. The same cells were strongly and diffusely positive for the vascular markers CD31, CD34 and ERG. The lesional cells were positive for human herpesvirus (HHV)-8 confirming a diagnosis of Kaposi sarcoma. He subsequently developed a second lesion on his left forearm, despite being off all immunosuppression. A repeat bone biopsy confirmed that he remains in morphological remission from his myeloma. Positron emission tomography–computed tomography has not identified extracutaneous disease. KS following HSCT is rare, but it can be fatal. Skin lesions are the dominant clinical presentation. HHV-8 has an important role in the pathogenesis, and transplant-related KS may occur secondary to reactivation of the virus as a result of significant immunosuppression following HSCT. However, it could also occur as primary infection or be transmitted with donor cells. The outcome of KS after HSCT is variable. A watch and wait strategy can be applied if immune recovery is expected. In our patient, it developed 5 months after HSCT; shortly after immunosuppression had been tapered, he remains well and will be closely monitored for further suspicious lesions.
BI12 The incidence and survival of Kaposi sarcoma using national cancer registry data
Authors: Quaresma Catarina , Lapin Agnieszka , Vernon Sally , Tan Agnieszka , Venables Zoe C
Published: June 2023

Kaposi sarcoma (KS) is a rare soft tissue endothelial neoplasm associated with human herpesvirus (HHV)-8 infection. Four types of KS are recognized, with the majority of cases in the West attributed to HIV-associated KS or, less frequently, to iatrogenic KS caused by systemic immunosuppression. Population-based data on KS epidemiology in England are lacking, with the last published evaluation dating back to the 1980s. More recently, data on KS epidemiology have been included in the National Cancer Registration and Analysis Service’s (NCRAS) ‘Get Data Out’ annual reports, with details published from 2013 to 2019. A nonsignificant reduction in the incidence rate of KS in England was observed from 2013 [0.271; 95% confidence interval (CI) 0.229–0.319] to 2019 (0.222, 95% CI 0.185–0.265; www.cancerdata.nhs.uk/getdataout/kaposi_sarcoma). This figure is slightly lower than the most recent reported crude incidence rates of KS in Europe from 2000 to 2007 (0.28) and significantly lower than that of the USA in 2017 (0.45) and globally in 2020 (0.439) (https://gco.iarc.fr/today/data/factsheets/cancers/19-Kaposi-sarcoma-fact-sheet.pdf). Data from NCRAS show that most cases of KS in England were diagnosed following a general practitioner referral (32.8%), outside the 2-week wait route (15.6%). A noteworthy proportion of cases (17.2%) were diagnosed following an emergency presentation to the emergency department, emergency clinician referrals or acute admissions. The 5-year net survival after the diagnosis of KS was stable at 81.3% (95% CI 73–89.5), 86.8% (95% CI 79.5–94.1) and 79.7% (95% CI 67.2–92.2) for 2013, 2014 and 2015, respectively (www.cancerdata.nhs.uk/getdataout/kaposi_sarcoma). These data provide a useful insight into the epidemiology of this rare cancer; however, it is probable that the true incidence of KS is under-reported, particularly in cases where there is no histological confirmation or reporting of KS on the death certificate, which may be more likely in patients who were HIV positive. Lack of knowledge of the underlying cause for KS is a major limitation. In conclusion, there is a paucity of published data on KS epidemiology in England. Further evaluation of KS subsets, patient demographics and comorbidity at diagnosis is needed to allow a better understanding of differences in the KS incidence in England in comparison with global figures.