bluuebunny/crossref_metadata_2025_split huggingface dataset:
BI21 Lues maligna mimicking pyoderma gangrenosum and cutaneous lymphoma
Authors: McKenzie Ning , Hickey Matthew , de la Sancha Carlo , Coates Sarah , Leslie Kieron 
Published: June 2024

Abstract
A 44-year-old man was referred for 2 months of erythematous ulcerative papules and plaques on the scalp, face and lower legs. The lesions started as painful ‘pimples’. He also reported fever and chills. He had a 5-year history of well-controlled HIV on antiretroviral therapy since diagnosis and a prior history of recurrent syphilis. Exam showed a large violaceous plaque on the right cheek with surrounding erythematous papules and an additional erythematous papule on the left ear. On the bilateral ankles and calves were four ulcers with necrotic centres and purple borders. The differential diagnoses included pyoderma gangrenosum and cutaneous lymphoma. A punch biopsy of the right zygomatic area showed dermatitis with a dense, diffuse plasma cell infiltrate suggestive of syphilis. The acid-fast bacteria (AFB), periodic acid–Schiff with diastase (PAS-D), and spirochete stains were all negative. Rapid plasma regain (RPR) was reactive at 1 : 4096. HIV was detected at &lt; 30 copies per mL. Absolute CD4 T-cell count was 529 cells per μL. AFB and fungal cultures were negative, and bacterial culture showed rare Staphylococcus aureus. Although the spirochete stain was negative, it is only 70% sensitive and the positive RPR with histopathology supportive of syphilis in a patient who was positive for HIV confirmed the diagnosis of lues maligna. The patient received one dose of 2.4 million units of intramuscular penicillin G benzathine. He continued empiric doxycycline 200 mg daily, which had previously been initiated, and continued doxycycline postexposure prophylaxis after diagnosis. The patient reported healing with no new lesions 3 weeks after treatment. A new question brought on by this case is whether prior history of recurrent syphilis infection has a role in developing lues maligna. This patient in particular required syphilis treatment four times in the 3 years leading up to his presentation. Current literature describes a patient who was treated for secondary syphilis, then reinfected with malignant syphilis. However, further research is needed to elicit whether recurrent syphilis infections increase the chances of severe infection or malignant syphilis.
BI30 An extensive follicular eruption in a transplant recipient
Authors: Orpin Sid , Patel Bhavnisha 
Published: June 2024

Abstract
A 46-year-old woman, who had been a patient with poorly controlled type 1 diabetes since age 18 years, received a combined pancreas and renal transplant in 2020. She was taking tacrolimus and mycophenolate as an immunosuppression regimen and presented with a 6-month history of an extensive rash. On examination, there was a widespread, symmetrical eruption affecting face, trunk and limbs consisting of folliculocentric pigmented keratinous papules. On the face and trunk in particular, these demonstrated a spiny appearance. Clinically, this was considered to be trichodysplasia spinulosa (TS), a rare dermatological condition first described in 1995 and established to be caused by TS-associated polyomavirus (TSPyV) in 2010. All cases are associated with either immunosuppressive medication or diseases that cause reduced immune function. Biopsies taken from the patient’s trunk and left thigh showed striking features of dilated follicles with prominent dyskeratosis and the follicular wall replaced by cells with grey cytoplasm and vesicular nuclei, alongside numerous homogeneous, eosinophilic intracytoplasmic inclusion bodies. These features have been described in only one case of a form of acquired epidermodysplasia verruciformis (EV) caused by type 49 human papillomavirus (HPV). More often, HPV-3, 4, 5, 8 and 51 are seen in EV. Subsequent use of SV40, an immunohistochemical stain that is a surrogate marker for polyomavirus infection, failed to demonstrate TSPyV, confirming the diagnosis as EV. There are currently no published case reports of acquired EV presenting in this way, simulating the follicle-centred spiculated rash seen exclusively in TS up to this point. EV, whether of inherited or acquired origin, presents either as a spreading eruption of pityriasis versicolor-like scaly macules, flat planar wart-like papules or verrucous seborrhoeic keratosis-like papules and plaques. Although the acquired form of EV does not appear to have the same risks of developing cutaneous squamous cell carcinomas as the inherited type, there is still an increased incidence of such tumours in transplant populations, so treating EV in our patient remains an important goal. Unfortunately, there are no established, effective treatment modalities. With less widespread lesions cryotherapy, topical imiquimod or topical 5-aminolaevulinic acid photodynamic therapy might be considered. In this case, with extensive disease, systemic treatment with oral retinoids, interferons or cimetidine could be considered but the results are unpredictable, and even when this treatment is effective at reducing the number of EV-related lesions, there is a high risk of recurrence upon cessation of therapy. In the first instance, following discussion with the renal team, it has been decided to cautiously reduce the doses of the patient’s immunosuppressive drugs as this is often effective in cases of TS.
BI32 A case of pembrolizumab-induced psoriasiform rash treated with risankizumab
Authors: Agarwal Vani , Chamberlain Georgie , Bala Laksha , Shimy Noha El 
Published: June 2024

Abstract
We present the case of a 77-year-old female patient with a pT3a clear cell renal cell carcinoma who underwent a nephrectomy and adjuvant pembrolizumab therapy. She had a past medical history of hypercholesterolaemia, for which she took atorvastatin. After 11 cycles of pembrolizumab she developed widespread erythematous plaques with overlying scale. The initial clinical diagnosis was a lichenoid drug eruption secondary to immunotherapy. Pembrolizumab was paused for 4 weeks and the rash significantly improved with daily application of 0.1% mometasone ointment. After two further cycles of pembrolizumab, the rash reoccurred with worsening severity. A 4-mm punch biopsy was taken from the right thigh. This showed parakeratotic scales, dense neutrophilic inflammation, hypogranulosis, psoriasiform hyperplasia and a dense dermoepidermal mixed acute and chronic inflammatory infiltrate. This was consistent with a psoriasiform pattern of inflammation. Of note, she did not have a personal or family history of psoriasis. Despite maximum topical therapy and stopping immunotherapy, her Psoriasis Area and Severity Index (PASI) score increased from 10 to 33 over a few weeks. She had a body surface area coverage of 70%. Her Dermatology Life Quality Index (DLQI) score was 24. Owing to the severity of her skin and relative contraindications (history of malignancy) to conventional systemic therapies, the decision was made to commence risankizumab, an interleukin-23 inhibitor. She had an excellent response with an improvement of PASI score to 8.4 and DLQI score to 4, 4 weeks after initial induction. Pembrolizumab is an immune checkpoint inhibitor that inhibits programmed cell death 1. It has been associated with a wide variety of cutaneous adverse events including eczematous, morbilliform and lichenoid dermatoses, as well as vitiligo and pruritus. It has less commonly been associated with psoriasiform dermatoses, bullous disorders and severe cutaneous adverse reactions. In the literature, there have been reported cases of immunotherapy-induced psoriasis that have been successfully treated with apremilast and secukinumab. To our knowledge, this is the second reported such case of successful treatment with risankizumab. As immunotherapy continues to become more widely used for the treatment of malignancies, reporting cases of safe, rapidly responsive and efficacious treatments is of great importance.
BI34 Lung toxicity with methotrexate: fact or fiction?
Authors: Gold Adam , Yesudian Paul D 
Published: June 2024

Abstract
Methotrexate (MTX) is widely used as a first-line immunomodulator for inflammatory skin diseases. MTX-induced lung toxicity is a rarely reported complication, and its prevalence is unclear. We conducted a survey of UK clinicians on their experiences with MTX lung toxicity. In total, 109 clinicians, including 54 dermatologists (49.5%), 19 radiologists (17.4%), 26 respiratory physicians (23.9%) and 10 respondents not stating their specialty (9.2%), completed an online questionnaire that assessed their experiences with MTX-induced lung toxicity. A total of 12 questions were sent to clinicians across the UK from October 2023 to December 2023. Overall, 93 clinicians (85.3%) reported seeing either no cases (n = 58, 53.2%) or between 1 and 5 cases (n = 35, 32.1%) of MTX-induced lung toxicity. Of these, only four (3.7%) respondents identified MTX-induced lung changes in patients with dermatological indications, in the absence of other causes. One respondent did not answer the question of how many cases they had seen. In the subgroup of clinicians who observed more than 5 cases (n = 15, 13.8%), there were no dermatologists, and none reported MTX use for dermatological indications. All patients had rheumatological/connective tissue diseases (CTDs). Of those who reported seeing more than 5 cases, 10 (67%) were respiratory physicians and 5 (33%) were radiologists. Of those who reported seeing no cases of MTX-related lung changes, 41 (71%) were dermatologists. The main lung complications reported were pulmonary fibrosis and pneumonitis, with variable outcomes regarding improvement. In a 2009 literature review of 3463 patients with rheumatoid arthritis treated with MTX, 84 patients (2%) experienced some form of lung toxicity, with only 15 cases (0.4%) being attributed to MTX. In contrast, in 2015 a review of clinical trials involving patients with psoriasis, psoriatic arthritis or inflammatory bowel disease found no significant increase in respiratory adverse events for MTX users over treatment periods of 16–52 weeks. Of 1630 participants in 7 trials, only 1 MTX-treated patient developed pneumonitis (0.06%). These findings are reassuring and seem to suggest that lung toxicity is more commonly linked to CTD or other conditions rather than to MTX treatment for skin disease.
Poster presentationsPS09 User experiences of Melanotan II injection for tanning
Authors: McKenzie Ning , Gonzalez Nathaly , Huang Cathleen , Barzallo Devin , Ware Olivia , Leslie Kieron 
Published: June 2024

Abstract
Melanotan II (MT2) is an injectable melanocortin agonist advertised as a tanning agent, despite not being approved by the Food and Drug Administration. Sold online as a ‘research peptide’, individuals can purchase and reconstitute the lyophilized (freeze-dried) form with bacteriostatic water to inject with insulin needles. While MT2 has been previously studied for erectile dysfunction treatment, its potential adverse effects (dysplastic naevi, renal infarction) and lack of ongoing clinical trials limit scientific research on its effects (Habbema L, Halk AB, Neumann M, Bergman W. Risks of unregulated use of alpha-melanocyte-stimulating hormone analogues: a review. Int J Dermatol 2017; 56: 975–80; Peters B, Hadimeri H, Wahlberg R, Afghahi H. Melanotan II: a possible cause of renal infarction: review of the literature and case report. CEN Case Rep 2020; 9: 159–61). This study aims to understand MT2 users’ motivations, hesitations, side-­effects and tanning effects, and their relationship with healthcare providers. Participants were recruited for this qualitative study through online forums such as Reddit.com, as well as snowballing via personal contacts. Virtual interview audio was recorded, transcribed into text, and coded using thematic analysis with two coders. Twenty-eight participants have been interviewed, aged 18–64 years (mean 38.4, SD 10.2); gender breakdown is 21 cisgender men, 6 cisgender women, and 1 transgender man. Three participants identify as LGBTQ+. Two of the 28 participants identify as Hispanic; all identify as White, with one who also identifies as Native American. All participants started MT2 for tanning, but some emphasized burn prevention as a motivator. Commonly reported side-effects include nausea, reversible darkening of moles, and an increase in libido or erections. All participants had darkening of skin tone, but their sun-exposure habits varied from none to dedicated, timed sun tanning. Dosage and dosage frequency also vary widely. Common topics that emerged include participants being introduced to MT2 through the bodybuilding community and frustration with the lack of available scientific research or medical knowledge regarding MT2. While physicians have reported dysplastic naevi in people using MT2, some participants perceive that MT2 is protective against skin cancer through preventing sunburns. Many patients regularly visit doctors or even dermatologists, but do not openly discuss their MT2 usage due to general distrust or doubt that their providers are knowledgeable enough to help. This provides an opportunity for dermatologists to build trust with patients and encourage harm reduction.
SG07 Evaluation of the Foot Function Index in pachyonychia congenita
Authors: Oldham Jaimie , Stuart Beth , McCarthy Rebecca , Schwartz Janice , Evans Holly , Hansen David , O’Toole Edel 
Published: June 2024

Abstract
Pachyonychia congenita (PC) is a rare autosomal dominant disorder caused by variants in the keratin genes KRT6A, KRT6B, KRT6C, KRT16 or KRT17 (PC-K6a, PC-K6b, PC-K6c, PC-K16, PC-K17). All subtypes are associated with plantar calluses, and 98% with plantar pain. Our aim was to assess the utility of the Foot Function Index (FFI) in quantifying the impact of painful calluses on function in PC. The FFI is a self-administered questionnaire composed of 23 questions. It produces a total score and three subscale scores (activity limitation, disability and pain), each of which range from 0% (no effect) to 100% (maximum effect). Patients from the International Pachyonychia Congenita Research Registry (IPCRR) with confirmed PC were asked to complete the FFI three times over 3 months. As well as the FFI, respondents were asked to document the peak temperature that day and their sex. Data were collected from the IPCRR for age and gene variants. In total, 389 responses were received from 211 patients with PC (125 female, 69 male, 17 question not answered). Their age ranged from 6 to 85 years. Genotypes included PC-K6a (62), PC-K6b (29), PC-K6C (11), PC-K16 (95) and PC-K17 (14). The mean score across different timepoint observations was consistent, with overall mean scores of 40.2% (SD 19.8), 40.6% (SD 19.0) and 38.1% (SD 17.0) across the first, second and third measurements, respectively. All groups have lower overall scores than the KRT16 group (44.4%, SD 17.9), but that difference is only statistically significant for the KRT6B group [−9.93%, 95% confidence interval (CI) −17.9 to −1.96]. The disability score and the activity limitation score also showed lower scores in the other gene groups compared with KRT16 (disability 45.3%, SD 20.8; activity limitation 25.2%, SD 17.7), but only the KRT6B group showed statistically significantly lower scores: −13.0%, 95% CI −21.7 to −2.22 for disability, and −8.25%, 95% CI −15.7 to −0.82 for activity limitation. For the pain score, all gene groups had a significantly lower score than KRT16 (54.1%, SD 20.3), apart from KRT6C. The values were KRT6A −8.99% (95% CI −15.7 to −2.3), KRT6B −12.5% (95% CI −21.3 to −3.8) and KRT17 −14.1% (95% CI −25.8 to −2.4). There was no clear relationship between age, temperature or sex and any FFI score. Overall, the results show a significant impact of PC on foot function. The results suggest that some genotypes have a bigger impact on foot function than others, particularly KRT16 variants. The FFI may be a suitable patient-reported outcome measure in PC clinical trials.
SG15 A topical matter: iatrogenic adrenal crisis following excessive prescription corticosteroid use
Authors: Khanna Divya , Bardhan Ajoy , Heagerty Adrian , Harper Natasha 
Published: June 2024

Abstract
A 39-year-old man with intermediate junctional epidermolysis bullosa (EB) was admitted with a 2-week history of nausea, vomiting, diarrhoea, weakness, loss of appetite and abdominal pain. He described worsening pain within his chronic wounds, which were widespread, crusted and producing extensive green exudate. On admission, he was hypotensive, tachycardic and pyrexial. Investigations showed anaemia (haemoglobin 86 g L−1), hyponatraemia (132 mmol L−1), hypokalaemia (3.3 mmol L−1), raised lactate (3.29 mmol L−1), raised C-reactive protein (263 mg L−1), and leucocytosis (white cell count 22.2 × 109 L−1, neutrophils 10.5 × 109 L−1, and eosinophils 1.88 × 109 L−1). Despite treatment for sepsis secondary to cutaneous infection, inflammatory markers remained high, and the wounds deteriorated, with continuous production of yellow exudate, increasing pain and new blistering. Additionally, medical interventions failed to resolve persistent abdominal symptoms, haemodynamic compromise and pyrexia. On further review, the dermatology team noted that, unbeknownst to them, the patient had been prescribed 100 g of clobetasol propionate monthly by his general practitioner for at least the last year. Adrenal insufficiency was suspected and subsequently confirmed by a 09.00 h cortisol test (79 nmol L−1). The dermatology team initiated a 100-mg intravenous hydrocortisone bolus, followed by a 6-hourly 50-mg regimen, with consequent resolution of hypotension, tachycardia, pyrexia, hyponatraemia and eosinophilia. Targeted antibiotic therapy (tazobactam and vancomycin), 50 : 50 ointment and wound management improved inflammatory signs. The endocrinology team confirmed secondary adrenal insufficiency and provided a steroid emergency kit and advice on sick day rules at point of discharge. Adrenal crisis in secondary adrenal insufficiency is a life-threatening medical emergency requiring immediate intervention. In this case, adrenal insufficiency was caused by repeated exposure to excessive exogenous topical corticosteroids, and acute wound infection precipitated adrenal crisis. Given the nonspecific presentation, adrenal crisis is frequently overlooked in favour of other more common differential diagnoses. However, eosinophilia, hypotension, unresolving pyrexia and ongoing acute systemic inflammatory response symptoms aid accurate diagnosis. This patient’s hypokalaemia was likely secondary to vomiting, highlighting the importance of clinical context, as hyperkalaemia is more commonly noted during adrenal crisis. Most importantly, this case highlights the importance of clinician-led corticosteroid stewardship and the need for a higher index of suspicion for adrenal insufficiency in patients with a history of significant topical corticosteroid use.
SG16 CBM-opathies for the dermatologist: a rapidly expanding delineated spectrum of heterogeneous phenotypes
Authors: Dubash Faisal , Henderson Louise 
Published: June 2024

Abstract
CBM [Caspase recruitment domain (CARD) protein–B cell lymphoma/leukaemia 10 (BCL10)–MALT1 paracaspase (MALT1)] complex proteins regulate critical signalling of innate and adaptive immunity. They cause a spectrum of primary immunodeficiency disorders, primary atopic disorders and immunodysregulatory syndromes. Germline mutations associated with CBM-opathies can cause antifungal immunodeficiency, CADINS, BENTA, atopic dermatitis, psoriasis, pityriasis rubra pilaris, and CARD14-associated papulosquamous eruption. Genes related to human germline CBM-opathies include CARD9, CARD11, CARD14, BCL10 and MALT1. We have reviewed key dermatological phenotypes related to human germline CBM-opathies. A notable example includes autosomal dominant hypomorphic CARD14 mutations associated with severe atopic dermatitis. Patients tend to be in their first year of life (between 3 and 12 months) with severe atopy, recurrent pyogenic and viral skin infections, and chest infections, as a consequence. CARD14 mutations may impair nuclear factor-κB activation, decreasing inflammatory gene expression, and impact epidermal secretion of antimicrobial peptides. Although hypomorphic mutations in CARD11 and CARD14 cause atopic dermatitis, gain of function mutations are associated with BENTA and inflammatory psoriatic skin disease. Psoriasis or pityriasis rubra pilaris diagnosed in individuals with earlier onset may warrant a high suspicion for sequencing of CARD14 in patients and families to aid therapeutic decisions. Genetic diagnoses can be made using next-generation sequencing techniques, such as whole-exome sequencing, whole-genome sequencing, or an immunodeficiency gene panel. For diagnostic confirmation, novel variants for CBM-opathies may be identified using methods to assess expression of proteins such as CARD11, phosphorylation of nuclear factor-κB, and degradation of IkBa.
BT21 Teledermatology pathway performance for detection of all skin cancer categories
Authors: Walls Joseph , Hall Per , Mills Louise , Kilford Claire , Wishart Gordon 
Published: June 2024

Abstract
The aim of this study was to audit the performance of a UK nurse-led teledermatology pathway for private patients with skin lesions suspicious of melanoma, basal cell carcinoma (BCC) or squamous cell carcinoma (SCC). Patients with suspicious skin lesion symptoms (change in colour, shape or size; &gt; 7 mm; inflamed or oozing) access the teledermatology pathway without general practitioner referral and are initially seen in private, community clinics by specialist skin cancer nurses trained in lesion detection and dermoscopy. Following a detailed lesion history, digital and dermoscopic images are taken then uploaded to the electronic patient management system for telemedicine reporting by experienced teledermatology specialists. Following telemedicine review, patients are either reassured or advised to attend a consultation with a skin cancer specialist for face-to-face examination with or without biopsy on the same day. Between 1 January 2018 and 31 December 2022, a total of 14 790 patients (6089 male, 8701 female; mean age 42 years, range 18–92) entered the diagnostic pathway. In total, 83% were offered a nurse appointment within 5 working days and 94% within 10 working days. Following telemedicine triage, 12 070 (81.6%) were reassured, and the remaining 2720 (18.4%) were advised to attend a consultant appointment. Of these 2720 patients, 2525 accepted this appointment, with the remaining 195 (0.01% of the total) attending the National Health Service (NHS) or alternative private provider. Of the 2525 who attended for further examination, 1634 underwent biopsy (11.0%) and 550 were diagnosed with skin cancer (cancer detection rate of 3.7%). Of the 550 skin cancers detected, there were 88 melanomas, 439 BCCs, 22 SCCs and one cutaneous B-cell lymphoma. Stage at diagnosis was available for 81 melanomas, with 78 (96%) of these patients diagnosed at American Joint Committee on Cancer (version 8) stage 1 or 2. There were no missed cancers documented during (or since) the audit period, giving a sensitivity of 100%, a specificity of 87.7% and an overall accuracy of 86.5% for the teledermatology cancer pathway. This audit confirms that a community-based and nurse-led teledermatology service for patients with one or more suspicious skin lesions can provide short waiting times to lesion assessment and diagnosis, with 81.6% reassured following telemedicine triage and only 18.4% attending a hospital appointment for further assessment and possible biopsy. The cancer detection rate is lower than in the NHS, but commensurate with the younger demographic of private patients. The early-stage melanoma diagnosis rate of 96% is well above the equivalent rates reported by NHS Digital for men (85%) and women (89%) in 2020. All authors are employed by the pathway provider.