tbuckley/cabot-search huggingface dataset:
BI17 Impact of an annual multidisciplinary clinic on patient care and experience in cutaneous T-cell lymphoma
Authors: Huw Greenish, Harry Heath, Rainy Marsh, Michelle Furtado, Giles Dunnill, Claire Bailey
Journal: British Journal of Dermatology (2024)

Abstract Cutaneous T-cell lymphomas (CTCLs) are a group of rare conditions with diagnostic and management challenges and reduced patient quality of life (QoL). Optimal management requires haematology, dermatology and referral to a local skin specialist multidisciplinary team (MDT), with further referrals to a regional supranetwork skin MDT for some patients. An annual CTCL clinic day was established to bring the MDT to the patient and address the challenges of CTCL. Objectives included establishing a local database of patients with CTCL including staging, adjusting treatment as appropriate, upskilling local clinicians, educating patients and initiating annual QoL assessments. Currently two annual clinics have taken place with a third planned. Patients with both known and suspected CTCL were invited. The clinics included nurse-led patient education and a 30-min dermatology-led consultation with access to a specialist haematology consultant and regional CTCL expert. On-the-day blood tests and diagnostic biopsies were performed if needed. Patients completed pre- and postclinic questionnaires for feedback, itch visual analogue scale and Dermatology Life Quality Index and mycosis fungoides (MF)-QoL assessments, and clinician feedback was sought. A total of 31 patients attended one (n = 17) or both (n = 14) clinics following an average 28% late-cancellation/no-show rate. Across the two clinics, it was determined that a total of eight patients did not have CTCL. All patients with CTCL were appropriately staged. Of those attending both annual clinics only 1 patient had a stage progression (IA to IB) and the majority had stable or improved QoL (9 of 13, MF-QoL). Four patients had unplanned hospital visits between clinics, all due to symptom or disease progression. Of the 23 patients with CTCL, 10 had their treatment type changed at one or both of the clinics, 3 had the frequency changed, 3 stopped treatment and 7 had no change in treatment. Patient feedback regarding the clinic was positive with 93% rating the outcome of the clinic as excellent or very good, and 97% responding that they would attend a similar clinic in the future. Some patients retained knowledge from the first clinic education session, whereas others did not and these patients benefited from repeat education. Clinician feedback also reported high value. The outcome of the first two annual CTCL MDT clinic days demonstrated the value patients placed on the service and the need to tailor education frequency to individual needs. In summary, the annual clinic efficiently delivers patient-centred management and care, and education to both patients and clinical teams, and ensures close working relationships between departments within the Trust. This project was a joint working partnership between the Trust and Recordati Rare Diseases UK.
CPC14 Winning streak: a case of congenital ichthyosiform erythroderma
Authors: Cathal O’Connor, Neidín Bussmann, Cynthia Heffron, Sarah Ní Mhaolcatha, Sally O’Shea
Journal: British Journal of Dermatology (2024)

Abstract A first-born female infant was delivered at term with widespread redness and scale. There was no family history of genodermatoses or inflammatory dermatoses. There was no parental consanguinity and no history of miscarriages. On examination there was erythroderma, generalized thick adherent scale in a feathery pattern following lines of Blaschko, and shiny red whorls on the dorsolateral feet. There was no skin peeling or skin fragility. Several fingernails were hypoplastic. The nasal bridge was flat and the neck was short. Red reflex was present bilaterally but both eyes were noted to be small. The head and trunk were large relative to the limbs and the proximal limb segments were proportionately short. Given the constellation of signs, a specific syndromic ichthyosis was suspected. Skin biopsies from linear scaly streaks showed orthohyperkeratosis and numerous dilated follicular ostia with keratin plugs. Foci of calcification were seen in the corneocytes of the stratum corneum and hair follicles, highlighted with a von Kossa stain. Skeletal survey showed symmetric punctate calcification and stippling of the proximal femoral epiphysis and ankle bilaterally, and of the right humeral epiphysis and right carpus. These findings were in keeping with chondrodysplasia punctata, although no gross rhizomelia was appreciated radiologically. Ophthalmology review did not identify congenital cataracts. Bloods tests showed normal haematological, renal and bone parameters, and a hyperbilirubinaemia meeting the phototherapy threshold. The erythema was noted to improve markedly following 12 h of phototherapy. Urea 10% cream was helpful in reducing the ichthyosis. Genetic testing revealed a pathogenic variant in the ­emopamil-binding protein (EBP) gene (c.184C&amp;gt;T; p.Arg62Trp), confirming the diagnosis of Conradi–Hünermann–Happle syndrome. Conradi–Hünermann–Happle syndrome is a rare type of chondrodysplasia punctata associated with X-linked dominant mutations in the EBP gene, which impair cholesterol biosynthesis. Affected unborn males are not expected to survive to birth. Conradi–Hünermann–Happle syndrome is characterized by the triad of congenital ichthyosiform erythroderma, skeletal dysplasia and congenital cataracts. The cutaneous phenotype is variable, due to the effects of X-inactivation in female infants. Dystrophic calcifications within keratotic infundibular follicular plugs, especially in the stratum corneum, are a unique but under-recognized histopathological feature of newborns with Conradi–Hünermann–Happle syndrome. It is important to perform the skin biopsy early, as the dystrophic calcifications typically resolve spontaneously after the first few weeks of life. This case highlights a typical dramatic presentation of Conradi–Hünermann–Happle syndrome, and emphasizes the importance of early skin biopsy for diagnostic clues.
DE02 Mentoring tomorrow’s dermatologists: establishing a mentorship scheme for junior doctors interested in dermatology
Authors: Sarah Winter, Sally Ashton
Journal: British Journal of Dermatology (2024)

Abstract Mentoring is a powerful tool for personal and professional development (Burgess A, van Diggele C, Mellis C. Mentorship in the health professions: a review. Clin Teach 2018; 15: 197–202). It is strongly advocated by the Royal College of Physicians. Several mentorship schemes have been created for doctors interested in competitive specialities. Dermatology remains one of the most competitive specialties, yet to the best of our knowledge, there are no mentoring schemes for junior doctors interested in dermatology in the UK. The aim of our project was to successfully launch a dermatology mentorship scheme to help mentees take control of their own career journey, while enabling mentors to build on their leadership skills. In 2022, a pilot dermatology scheme was created in Cardiff, matching foundation and core trainees to dermatology registrars. There were six delegates in the pilot. With the success of a pilot, where 100% of delegates felt that the scheme was beneficial to their career and progression, the mentorship scheme was expanded to include the whole of South Wales. The scheme was advertised through junior doctor groups on Facebook and through postgraduate medical education centres for mentees. In total, 23 delegates signed up to the 2023 scheme: 12 mentees and 11 mentors. A virtual mentoring workshop was held for all delegates and this was well received. Delegates were also provided with a mentoring contract to use. Initial survey results showed that 100% (n = 12) of mentees felt they had not yet been exposed to enough potential mentors at work or at medical school. Overall, 92% (n = 12) stated that they did not yet have a mentor-like figure, 67% (n = 8) of delegates stated that not having a scheme available was one of the reasons for not having a mentor, and 82% (n = 9) of the mentors wanted to develop their leadership skills through the scheme. Informal feedback has been overwhelmingly positive. Formal feedback will be welcomed in May 2024 to make improvements to the scheme for the following year. Our project shows that there is demand for mentorship in dermatology. We hope that the successful establishment of our scheme paves the way for mentorship schemes to be set up locally to help inspire and support the next generation of dermatologists.
BI22 A UK multicentre prospective study on the awareness and impact of azathioprine as a major risk factor for developing skin cancer in patients with inflammatory bowel disease: patient and clinician perspectives
Authors: Alla Altayeb, Sara Lupita O’Kelly, Lucy Howard, Eleanor M Deane, Philippa Walker-Smith, Andrew Birnie
Journal: British Journal of Dermatology (2024)

Abstract Azathioprine is an immunosuppressive drug commonly prescribed in the treatment of inflammatory bowel disease. Its immunosuppressive properties in addition to other pharmacological features unique to azathioprine are known to significantly increase the risk of nonmelanoma skin cancers, necessitating the need for close monitoring and increased ultraviolet (UV) protection practices. In this study, written surveys were distributed to patients with inflammatory bowel disease treated with azathioprine to establish their awareness of the skin cancer risk and to gain an understanding of their photoprotective behaviours, in addition to surveying their responsible gastroenterology clinicians (doctors and nurses) on their current practices and risk management. Thirty-two patient and 11 clinician surveys were completed across 3 NHS hospital trusts in the southeast of England, between July and September 2023. Of these 32 patients, 17 (53%) were male and 15 (47%) were female, two-thirds of patients (n = 21) were aged 16–49 years, and 11 patients (34%) were &amp;gt; 50 years with only 1 patient over the age of 70 years. The majority (60%) had Fitzpatrick skin type I or II. The mean duration of azathioprine therapy varied; 17 patients (53%) took azathioprine for less than 1 year and 12 patients (38%) took it for more than 3 years. The results highlighted a high level of awareness of skin cancer risk (75%), with all participants reporting some sunscreen use. However, adequate use of broad-spectrum sunscreen (with UVA protection) was poor, with the majority (78%) limiting their sunscreen use only to sunny days or when outside for extended periods. Additionally, despite the relatively young patient cohort, 16% reported a previous diagnosis of sun damage (actinic keratosis) or skin cancer, and 28% reported episodes of sunburn within the past year. Of the clinicians surveyed, six (55%) enquired about previous skin cancer and UV exposure before or during prescription of azathioprine. Ten (91%) provided written information on skin cancer risk, while seven (64%) advised on sunscreen use when prescribing azathioprine. Despite this, only one (9%) reported feeling confident advising patients on sunscreen application and the appropriate sun protection factor rating, and only two (18%) gastroenterologists reported feeling confident in undertaking a full skin check for the identification of any possible skin cancers during the monitoring phase. Although small, this study highlights the need for increased targeted preventive measures to reduce the risk of skin cancer in this population, and a need for greater support and education to the clinicians prescribing this drug.
DE04 Using an ‘umbrella clinic’ format to optimize educational experiences for trainees and clinical supervisors in dermatology
Authors: Sophie Holloran, Su Le Myo, Helen Whitley
Journal: British Journal of Dermatology (2024)

Abstract Learning in an outpatient environment is a key part of any medical trainee’s curriculum but is particularly pertinent for internal medical trainees, who need to demonstrate competence and evidence attendance at approximately 80 clinics on their portfolio to progress through training. With increasing demands on outpatient services and a lack of clinic space, it can be challenging to obtain this experience, and the logistics of doing so can significantly detract from the educational opportunities available. A review of outpatient care by the Royal College of Physicians in 2018 highlighted how we need to adapt our services not just to ensure we meet the increasing needs of patients in this clinical setting, but to make sure we are providing a high level of quality training for clinicians. With dermatology being a predominantly outpatient specialty, it is even more important to optimize learning experiences for students, general practitioners, internal medicine trainees and other senior trainees coming through the department, moving through the steps of Miller’s pyramid from being an observer to being able to practise independently. It can be hard to successfully bridge this gap in a busy, time-restricted outpatient setting. There is no perfect outpatient training model because, understandably, every department has different demands and resources. We would like to discuss our department’s model, an ‘umbrella clinic’ style, from the feedback of both trainees and consultant supervisors. The umbrella clinic is a model where a supervising consultant oversees multiple trainees in clinic at the same time. Clinic rooms are allocated for trainees to carry out the consultation, followed by a brief presentation to the consultant and a second senior patient review with the trainee and consultant. The trainees work from a single list of patients and review patients with the consultant in turn. We currently utilize this method for our general dermatology, paediatric and skin cancer clinics. This method allows for trainees of different levels of experience to naturally progress through Miller’s pyramid with immediate senior support available, becoming more confident and efficient over time. It also allows for supervising clinicians to have a better understanding of an individual trainee’s needs and supervision requirements. From an administrative perspective, this system allows flexibility by adapting the number of patients booked in each clinic depending on how many trainees and consultants are working together. Our experience demonstrates that this model of outpatient practice provides a higher level of satisfaction for the educational needs of trainees while maintaining an effective level of service provision for patients.
PA31 Traction alopecia in the paediatric Sikh population
Authors: Akash Sharma, James Halpern, Alexis Cuell, Raakhee Ramesh, Manrup Hunjan
Journal: British Journal of Dermatology (2024)

Abstract Sikh boys and girls, traditionally, keep their hair long and tend to tie it up in a knot over the vertex of their scalp; this is colloquially referred to as a ‘jura’. The repeated traction on the hair coupled with the weight of hair often leads to a distinct hair loss pattern that can be concerning and distressing for patients and parents alike. We describe two cases of traction alopecia in young Sikh boys, who presented to our tertiary paediatric service. The first case is 14-year-old boy who presented with reduced hair density of hair follicles and recession of the frontotemporal hairline. Similarly, the second case was a 14-year-boy, who presented with increasing hair loss over the frontal scalp. He had been tying his hair in a jura since the age of 4 years. He was also vitamin B12 deficient and on replacement treatment. With both cases the hair loss was particularly prominent over the frontotemporal scalp and was distributed in a bandlike pattern. There was sparing of the eyebrows, eyelashes and body hair. Trichoscopy findings included mild perifollicular scale without erythema, single follicular hair units and perifollicular inflammation. In both patients the initial diagnosis was traction alopecia with coexisting seborrhoeic dermatitis. Histology from scalp biopsies demonstrated intrafollicular coverage and follicular distortion with absence of inflammatory infiltrate. This was in keeping with the clinical impression of traction alopecia. Hair loss can have a profound impact on quality of life in both the paediatric and adult populations. In those with traction alopecia, early intervention is paramount in order to prevent progression and aid recovery of hair. Thus, we advised our patients to avoid putting their hair up in a tight jura for long periods of time, whenever feasible, as well as advising that hair be left open or loosely tied in a bun or ponytail. For topical treatment, minoxidil 2% or 5% foam can be tried to see whether it will help regrowth; however, this is within the remits of tolerability. Any coexisting diagnoses, such as seborrhoeic dermatitis, should be treated, and if there are doubts with regards to diagnosis, or features of a scarring alopecia, then it would be prudent to do a biopsy. These cases highlight a pattern of reversible hair loss seen in the Sikh paediatric population. It is important for dermatologists to recognize this, in order for early intervention and education on a potentially reversible condition.
H02 Superficial X-rays and grenz rays for benign dermatoses: from saviour to pariah over the course of the 20th century – the story of the remarkable rise and slow decline of an entire therapeutic modality and its potential for revival
Authors: Zinah Sharara, David J Gawkrodger
Journal: British Journal of Dermatology (2024)

Abstract Dermatologists in the decade spanning the turn of the 19th century thought they were on to something with the discovery of X-rays by Wilhelm Röntgen at Würzburg in 1895. Effects on the skin were soon apparent from the earliest experiments, and dermatologists, keenly aware of Niels Finsen’s success using ultraviolet radiation for lupus vulgaris, quickly transmuted their interest to the new type of radiation. They were impressed with its benefits not only in lupus vulgaris but also in a wide range of disorders including important conditions such as acne, eczema, psoriasis, fungal infections and skin cancers. Major hospitals set up ‘electrical’ departments – the Middlesex Hospital had one from 1896 – and textbooks heavily featured the new modality. Malcolm Morris’s 1911 Diseases of the Skin lists X-ray treatment for no fewer than 38 conditions. Grenz rays, with wavelengths nearer the ultraviolet and not so penetrative as X-rays, found similar applications. In the absence of an overabundance of effective topical therapies, and despite the growing realization that overdosage was a potential hazard, superficial X-rays represented a major limb of the dermatologist’s armamentarium through the interwar years and beyond. Fifty-five per cent of office dermatologists in the USA of 1975 had their own X-ray machine. Most British skin clinics had machines and dedicated radiographers to boot. The decline started with the topical corticosteroid revolution of the 1960s and accelerated after that, such that by the 1980s, with the removal of X-ray machines from dermatology departments, radiologists were disinclined to permit the use of superficial X-rays for benign dermatoses. Some centres pressed on with their use for a while in hard-to-treat eczema or psoriasis of the hands or feet, but the current guidelines no longer support this, describing the treatment as ‘obsolete’. The use of more penetrating radiation has been maintained for certain skin cancer indications. Considering that many biologics with serious and not uncommon side-effects are now prescribed at quite a low severity bar, it is tempting to think that the toxicity of judiciously applied superficial X-rays might be of a similar or even lesser order. Perhaps this previously indispensable modality is due a revival?
P004 Morphoea in a patient with clear cell renal cancer and lung metastases
Authors: George Palaiologos, Anastasios Mavrogiorgos
Journal: British Journal of Dermatology (2024)

Abstract Morphoea (localized scleroderma) is an autoimmune fibrotic skin disease with an estimated prevalence of 200 per 100 000. Although the pathogenesis remains unknown, the disease is characterized by an initial T-cell-mediated inflammatory phase followed by localized fibrosis of the skin. Most cases of morphoea in patients with cancer occur after radiation therapy (65%), chemotherapy (8.5%) or immunotherapy–immune checkpoint inhibitor (ICI) therapy (5.7%). In two-thirds of cases of morphoea in patients with cancer, the mean time to onset of morphoea is 3.2 years after radiation, 1.7 years after chemotherapy, and 8 months (rapid manifestation of morphoea) after ICI immunotherapy. We present the case of a 65-year-old male patient with a history of clear cell renal carcinoma, who underwent surgical removal of his right kidney 5 years previously. Two years postoperatively, he developed a metastatic nodule in the right upper lobule of his lung. The patient was under treatment with pazopanib 800 mg per day for 15 months. Pazopanib is a tyrosine kinase inhibitor that blocks tumour growth and inhibits angiogenesis. The metastatic nodule disappeared but a new one appeared (computed tomography and magnetic resonance imaging). While the second metastasis remained stable and the patient was not undergoing any treatment, he developed the typical skin lesions of morphoea, with numerous sclerotic, or atrophic lesions with lilac ring, 2.5 years after the cessation of pazopanib. The diagnosis was confirmed by biopsy and he is currently under topical treatment with cortisone and calcineurin inhibitors. To date, several studies have reported sudden development of morphoea either before or after a brief interval following the diagnosis of malignancy. Scleroderma is characterized by immunological dysregulation, vasculopathy and hyperproduction of the extracellular matrix by activated fibroblasts. Endothelial inflammatory and mesenchymal cells produce cytokines, growth factors (interleukins 1 and 6 and tumour necrosis factor-α), connective tissue growth factor, and basic fibroblast growth factor. In our case study, we examined a patient with a localized type of scleroderma (morphoea) without confirming the markers for systemic involvement. Morphoea developed 5 years after the diagnosis of clear renal cell carcinoma and 2.5 years after the cessation of pazopanib – a tyrosine kinase inhibitor – so it should be considered a paraneoplastic phenomenon rather than a secondary effect of pazopanib cancer therapy.