bluuebunny/crossref_metadata_2025 huggingface dataset:
CD13 Effect of ciclosporin on serum inflammatory cytokines in patients with allergic contact dermatitis due to the weed Parthenium: a preliminary study
Authors: Verma Kaushal , Mohan Braj , Sharma Alpana
Published: June 2024

Allergic contact dermatitis due to the weed Parthenium is common in India, resulting in high morbidity and significant impact on quality of life in these patients. The allergic response is primarily mediated by a T helper 1 response, and various inflammatory cytokines have been implicated in the pathogenesis of the disease, as well as disease activity. We evaluated the effect of ciclosporin on inflammatory cytokines and disease activity in patients with Parthenium dermatitis. Adult patients with a clinical diagnosis of Parthenium dermatitis, confirmed on patch test, were included in the study. The severity of the disease was determined in each patient using Clinical Severity Score (CSS) and serum proinflammatory [tumour necrosis factor (TNF)-α, interleukin (IL)-17 and IL-6] and anti-inflammatory cytokines [IL-10, transforming growth factor (TGF)-β]. The patients were treated with ciclosporin 2.5 mg kg−1 per day for 12 weeks or until complete remission of the disease. The cytokine levels and disease severity were reassessed following treatment. The study had 35 patients (28 male and 7 female; age range 32–80 years, mean 56.0, SD 11.1), with a disease duration of 1–30 years (mean 10.7, SD 7.8). The pretreatment CSS of these patients ranged from 12.8 to 78.3 (mean 41.7, SD 16.3). The proinflammatory cytokines (TNF-α, IL-6 and IL-17) showed a decreasing trend following ciclosporin treatment. The levels of TNF-α decreased from a mean (SD) 4.0 (9.4) pg mL−1 to 1.4 (1.8) pg mL−1 (P = 0.05); IL-6 from 47.2 (34.7) pg mL−1 to 26.1 (17.6) pg mL−1 (P = 0.005) and IL-17 from 154 (163) pg mL−1 to 75.6 (73.2) pg mL−1 (P = 0.10), with statistically significant changes for TNF-α and IL-6 but not for IL-17. Serum levels of the anti-inflammatory cytokine IL-10 increased from 4.20 (1.20) pg mL−1 to 3.40 (1.10) pg mL−1 (P = 0.05), which was also statistically significant, but serum TGF-β values remained largely unchanged, going from 0.40 (0.27) pg mL−1 to 0.30 (0.23) pg mL−1 (P = 0.08). The mean CSS decreased from 41.7 (16.3) to 1.5 (5.2) (P < 0.001) following treatment. The changes in inflammatory cytokine levels seem to correspond with change in CSS and clinical disease activity. Therefore, this study has shown that the proinflammatory cytokines TNF-α, IL-17 and IL-6 decreased significantly, while the anti-inflammatory cytokine IL-10 increased following ciclosporin therapy in patients with Parthenium dermatitis, with a significant decrease in CSS and excellent control in disease activity.
DS02 The first 3 years of national data from the British Society for Dermatological Surgery Mohs micrographic surgery logbook
Authors: Bray Adam
Published: June 2024

A secure online electronic logbook for UK Mohs surgeons was launched in February 2021. We present the first 3 years of data. The purpose is to facilitate all units to record the British Society for Dermatological Surgery (BSDS) Mohs surgery minimum dataset, to use for training, professional development, quality assurance, and improving outcomes for patients. Anonymized data were aggregated using the logbook reporting functions, and raw data in Excel. In total, 8693 cases were logged from 2021 to 2024, with a mean of 580 per active unit (range 1–1526); 23 units are registered, with 15 logging regularly. Seventy-six surgeons logged cases, with an average of 113 cases per surgeon (median 63; range 1–795). The mean patient age was 69 years (range 15–99; 2.3% ≥ 90 years old), and the male-to-female ratio was 0.99. The mean number of resection stages (Mohs layers) was 1.5 (range 1–8). The mean maximum tumour/defect diameters were 14 mm preoperatively and 20 mm postoperatively. Overall, 80% were reconstructed on the same day; 32% were by linear or partial closure, 30% local flap, 16% graft, and 9% secondary intention. In total, 90% were reconstructed by dermatology. Overall, 43% of cases had a preoperative histology diagnosis entered: 2734 basal cell carcinoma (93%), 133 squamous cell carcinoma (5%), 12 sebaceous carcinoma, 10 dermatofibrosarcoma protuberans, 9 microcystic adnexal carcinoma, 3 unspecified follicular tumours, 1 atypical fibroxanthoma, 1 Merkel cell carcinoma, and 36 others not specified. Most (99.6%) were on the head or neck, with 39% on the nose or paranasal. Each unit completed on average 81% of the mandatory logbook items (range 0–100%). The commonest missing data were related to postoperative outcomes collected after 3 months, for example the final histology diagnosis (28% completed), complications (6%), aesthetic outcomes reported by patient and doctor (5–6%), and functional outcome (5%). Of those with postoperative complications completed, 84% had no complications, while 8.1% reported dehiscence or necrosis, 5.0% wound infection, and 2.9% bleeding or haematoma. Therefore, approximately 1 in 6 patients with data experienced one of these complications. Functional deficit was captured separately, with 84% of those reported as having no functional deficit, 7.5% numbness, 3.8% ectropion or epiphora, 2.4% nasal breathing difficulty, 2.2% pain or discomfort, and 0.8% localized paresis. Overall, 95% of those with complications required no additional treatment, with 3.2% needing a scar revision, 0.64% an unplanned readmission to hospital, and 0.32% an unplanned return to theatre. Of those with aesthetic outcome data, 68% of patients answered ‘not at all’ to the question ‘How much does your scar appearance bother you?’, while 26% reported ‘a little’, 5.2% ‘a lot’ and 0.3% ‘worst possible’. Use of the logbook is now standard practice for many UK Mohs surgeons, and most major training centres. Challenges remain for engaging others, and in gathering postoperative outcomes.
DS09 Nasalis myocutaneous island pedicle flap for nasal defects after Mohs surgery: a case series and video demonstration
Authors: Aslam Arif
Published: June 2024

The technically difficult superiorly based nasalis myocutaneous island pedicle flap has been described as a useful repair option for distal nose defects after Mohs surgery. Both the bipedicle and single pedicle modification have been described. With the use of a video demonstration the author demonstrates the method of preparing, raising and executing the single pedicle nasalis sling flap, with pre- and postoperative results of representative cases. The author also describes his 6-year experience with this flap with pearls on how to execute it for the best results.
DS10 Postoperative complications after two-stage interpolated flap repairs for nasal defects after Mohs surgery
Authors: Aslam Arif
Published: June 2024

Dermatologists perform more flaps after skin cancer surgery than all other specialties. This study evaluated complications after interpolated flap repairs (forehead and cheek–nose) of the nose after Mohs surgery. This was a single-surgeon prospective study of 64 patients undergoing two-stage interpolated flap repairs over a 6-year period. Complications such as bleeding, infection, flap necrosis and hospitalization were reported. Seventeen patients experienced complications after flap placement, and six after the flap division and inset. The most common complication after the flap was created was bleeding, with infection being the most common after takedown. This study shows that two-stage flap repairs for the nose after Mohs surgery can be safely performed in a hospital outpatient setting under local anaesthesia.
DS23 Evaluation of American Joint Committee on Cancer and alternative tumour classification systems for primary penile squamous cell carcinoma
Authors: O’Connell Katie , Thomas Jacob , Murad Fadi , Sonpavde Guru , Mossanen Matthew , Clinton Timothy , Xu Antonio Ji- , Spiess Phillippe , Rossi Anthony , Schmults Chrysalyne
Published: June 2024

Survivorship overlap exists among patients classified according to the American Joint Committee on Cancer (AJCC) 8th edition tumour (T) staging system for penile squamous cell carcinoma (PSCC). At present, the AJCC 8th edition guidelines for PSCC stratify T1 tumours by the presence or absence of perineural invasion (PNI) and lymphovascular invasion, and the degree of differentiation. T2 and T3 tumours are stratified based on the anatomical depth of invasion into the corporal structures, with T4 tumours invading into the scrotum, prostate or pubic bone. This study evaluates the current performance of PSCC AJCC T staging and compares it to an alternative. Different combinations of tumour risk factors were evaluated in terms of distinctiveness (prognostic groupings without overlapping confidence intervals) to determine a new system with the best risk stratification. These characteristics have been set forth by the AJCC to describe the ideal staging system. An alternative staging system was developed from a previously published cohort of 265 patients with PSCC. Tumours were staged via the alternative and AJCC T staging systems. Predictions of metastasis and disease-specific death (DSD) were compared. The alternative and AJCC systems were compared regarding distinctiveness, homogeneity and monotonicity. Three independent predictors of poor outcomes were included in the alternative PSCC T staging system: PNI, poor differentiation, and invasion into corporal structures. In the alternative system, T1 represents 0 risk factors (RF), T2 is 1 RF, T3 is 2 RF, and T4 is 3 RF. The alternative system was superior to AJCC staging in detecting tumours at risk for metastasis (C-statistic: alternative 67% vs. AJCC 63%) and DSD (C-statistic: alternative 74% vs. AJCC 72%). Homogeneity and monotonicity were similar. For alternative T1 disease, the 5-year cumulative incidence of metastasis was 9% and risk of death 5%. The risk of metastasis climbed to 24%, 42% and 74% for alternative T2, T3 and T4 cases, respectively, and 21%, 33% and 60% for DSD. The alternative PSCC T staging system appears to have superior distinctiveness. External validation of the alternative staging system against the AJCC stage should be undertaken. Alternative T1 N0 disease may benefit from nodal staging given the 9% risk of metastasis. Alternative T2/T3/T4 N0 PSCC carries a significant risk of metastasis and death and merits further study for optimal management.
DS24 Is surgery the best management for keratoacanthoma centrifugum marginatum?
Authors: Surgenor Laura , Austin Sinead , Dolan Olivia , Lannon Declan , Ashraf Sana , Goh Alicia
Published: June 2024

Keratoacanthoma centrifugum marginatum (KCM) is a rare variant of keratoacanthoma (KA). KCM typically presents as large solitary tumours with raised, rolled borders with distinctive patterns of continual peripheral tumour growth and central atrophic scarring. Although the clinical features are distinguishable from typical KA, KCM poses a diagnostic challenge due to its rarity, and lack of characteristic histopathological features. Classically, surgical intervention is the preferred treatment option; however, when tumours involve important anatomical structures we pose the question: Is surgery the best management approach? A 28-year-old woman presented with an 8-week history of a rapidly evolving tumour involving the right nasal sidewall. Initially measuring 2.6 × 2.5 cm, it progressively increased in size, extending intranasally with fullness of the right nasal septum. As the lesion was too large to entirely excise, it was debulked by shave excision. The clinical and pathological findings were compatible with KCM. Further surgical intervention to clear the deep margin was not feasible due to risk of significant functional impairment and disfigurement. Numerous other treatment options for KCM have been reported, including oral retinoids. Following review of the literature it was our intention to commence low-dose isotretinoin, but the patient declined as she had plans to conceive. However, reassuringly, with close follow-up and serial photographs there has been evidence of further involution. KCM was first described as a unique subtype of KA by Miedzinski and Kozakiewicz in 1962 {Miedzinski F, Kozakiewicz J. [Keratoacanthoma centrifugum – a special variety of keratoacanthoma]. Hautarzt 1962; 13: 348–52; in German}. While tumour involution is a typical feature of KA, in contrast, KCM rarely involutes and can display a protracted long-term growth pattern, leading to significant tissue destruction and disfigurement. Therefore, active intervention is preferred. KCM is best regarded as a benign entity. It has been associated with local recurrence; however, no reported cases of metastatic KCM exist to date. Despite this, KCM is still regarded by some within the spectrum of well-differentiated squamous cell carcinoma. The management dilemma triggered by this case poses an opportunity to consider the importance that retinoids play in the context of keratinocyte-based tumours. Retinoids are known to inhibit early stages of carcinogenesis via DNA damage. Retinoic acid is a potent antioxidant against tumour development, and can induce apoptosis via creation of reactive oxygen species in cancerous cells. Oral retinoids are well recognized as effective chemoprophylaxis against the formation of keratinocyte tumours in organ transplant patients. We present this case to highlight a rare entity in a young person. There is a lack of consensus regarding the use of retinoids in KCM due to the paucity of cases; however, they do merit consideration in selected cases.
DP07 Diagnostic implication of skin biopsy for a rare neurodegenerative disorder
Authors: FitzGerald Holly , Ardakani Nima Mesbah
Published: June 2024

Neuronal intranuclear inclusion disease (NIID) is a rare neurodegenerative disorder characterized by eosinophilic hyaline intranuclear inclusions in various cells of the central nervous system and extranervous system, including the skin and subcutis. This case report details a 67-year-old man presenting with progressive short-term memory impairment, paranoia, anxiety and bladder dysfunction, leading to a diagnosis of NIID. It emphasizes the utilization of skin biopsy as a diagnostic tool beyond dermatological conditions. NIID is a slowly progressive neurodegenerative disorder associated with diverse clinical manifestations. Despite its heterogeneity, characteristic eosinophilic hyaline intranuclear inclusions in nervous system cells and other organs define the disease. Clinical presentations include pyramidal and extrapyramidal symptoms, dementia, ataxia, autonomic dysfunction, convulsions and neuropathy. A 67-year-old man presented with progressive short-term memory loss, daytime somnolence, paranoia, nocturia and incontinence. Clinical examination revealed a Montreal Cognitive Assessment score of 21/30, with significant points lost in delayed recall. Magnetic resonance imaging indicated increased diffusion-weighted imaging signal at the corticomedullary junction and cerebral hemisphere, suggestive of NIID. Two axillary skin punch biopsies were performed, showing occasional eosinophilic intranuclear inclusions in the nuclei of the epithelial cells lining the sweat glands. These intranuclear inclusions were strongly positive for p62 (ubiquitin) immunostain. In addition, p62-positive intranuclear inclusions were identified in the nuclei of adipocytes, endothelial cells lining dermal and subcutaneous blood vessels, and stromal fibroblasts. NIID is an unusual disease entity with varied clinical phenotypes, including infantile, juvenile and adult-onset forms. The age of onset and dominant symptoms further classify patients. Challenges in antemortem diagnosis historically limited understanding, with postmortem dissection being the primary diagnostic method until 2011. The discovery of intranuclear inclusions in skin biopsies, specifically ubiquitin-positive inclusions in sweat gland cells, adipocytes and fibroblasts, has revolutionized NIID diagnostics. Skin biopsy, although invasive, is preferable to other methods in terms of accessibility, acceptability, complication risk and sensitivity. This case report underscores the significance of skin biopsy in diagnosis beyond dermatological conditions. Further research is needed to enhance understanding, elucidate the pathogenesis, and develop targeted treatments for this complex neurological disorder. Ongoing efforts in research will contribute to a clearer clinical picture and improved management strategies for individuals affected by NIID (Pang J, Yang J, Yuan Y et al. The value of NOTCH2NLC gene detection and skin biopsy in the diagnosis of neuronal intranuclear inclusion disease. Front Neurol 2021; 12: 624321).
DP09 (P002) Inflammatory vitiligo with raised borders: a rare case of pseudolymphoma mimicking hypopigmented mycosis fungoides
Authors: Daruish Maged , Grossmith Cecilia , Taibjee Saleem
Published: June 2024

A 51-year-old woman was referred to our department with a mildly itchy persistent rash that had started 3 years earlier on the back of her thighs, leaving pale skin as it progressed. She noted worsening of the rash during winter months. There was a history of psychological stress preceding the onset of the rash. Clinical examination revealed bilateral symmetrical annular hypopigmented patches with erythematous and slightly scaly raised edges. Symmetrical patches of depigmentation were also seen on her forearms. An incisional biopsy from the left posterior thigh, including the hypopigmented centre and adjacent erythematous rim, revealed unremarkable epidermis corresponding to the hypopigmentation, while the advancing edge showed interface changes with a bandlike lymphohistiocytic infiltrate and prominent lymphocytic exocytosis. The lymphocytes were positive for pan T-cell markers (CD2, CD3, CD5, CD7), with relatively equal CD4 and CD8 populations. melan-A indicated reduced density of melanocytes. There was no evidence of clonality on T-cell receptor (TCR) gene rearrangement studies. Punch biopsy from a depigmented patch on the right forearm was unremarkable on haematoxylin and eosin sections. However, melan-A indicated that the epidermis was completely devoid of melanocytes. The diagnosis of inflammatory vitiligo with raised borders (IVRB) was made. IVRB is a rare subtype of vitiligo that typically presents with a raised erythematous, scaly and pruritic edge surrounding depigmented patches. Differentiating IVRB from hypopigmented mycosis fungoides can be challenging, both clinically and histologically, due to the prominent lichenoid infiltrate with epidermotropism and the predominance of CD8+ lymphocytes that can be found in both. Helpful clues include the characteristic erythematous border, as well as lack of clonality on TCR gene rearrangement studies in IVRB (Gomez-Moyano E, Vera-Casaño A, Gamero AH et al. Inflammatory vitiligo or hypopigmented mycosis fungoides? J Pediatr Hematol Oncol 2010; 32: 338–9). Melanocytes and epidermal melanin are more likely to be preserved in hypopigmented mycosis fungoides [El-Darouti MA, Marzouk S, Azzam O et al. Vitiligo vs. hypopigmented mycosis fungoides (histopathological and immunohistochemical study, univariate analysis). Eur J Dermatol 2006; 16: 17–22]. Clinicopathological correlation is crucial to make the distinction.
PA13 A hair’s breadth from misdiagnosis
Authors: Kearney Nicola , Stefanovic Nicholas , Murray Gregg , Molloy Kevin , Watchorn Richard
Published: June 2024

A 15 year-old boy presented with a 2-year history of patches of hair loss on his body, and skin changes. Initial examination revealed scattered erythematous patches with associated follicular accentuation and concurrent nonscarring alopecia, predominantly affecting his limbs. His background included acne, gynaecomastia and exercise-induced asthma. His only regular medication was a salbutamol inhaler. Punch biopsies from the left shin and right arm demonstrated mild acanthosis of the epidermis with slight hyperkeratosis, focal parakeratosis and minimal spongiosis with occasional lymphocyte exocytosis. The initial biopsies were reported as nonspecific changes of lymphocytic folliculitis with spongiosis. Following clinicopathological review a diagnosis of alopecia mucinosa was considered. Further histopathological review confirmed spongiosis in the superficial to midpart of the hair follicle. Additionally noted was exocytosis of lymphocytes, which displayed subtle cytological atypia marked by nuclear irregularity and slight enlargement, and immunohistochemistry indicated a predominance of CD4+ T cells, raising the possibility of mycosis fungoides. Subsequent incisional biopsies were performed for T-cell clonality studies using the BIOMED-2 protocol and were polyclonal. Expert external histopathologist opinion was sought and this supported a diagnosis of folliculotropic mycosis fungoides (FMF), a condition rarely described in paediatric patients. The patient had patch-stage disease of his arms and legs with follicular accentuation and his modified Severity-Weighted Assessment Tool (mSWAT) score was 3.9. He had no lymphadenopathy or B symptoms. He was initiated on mometasone furoate 0.1% and his current management plan is for observation for clinical response and for further investigations, including lymphocyte subsets and high-throughput sequencing of the T-cell receptor, which are awaited. Although T-cell gene receptor rearrangement studies indicated polyclonality, their sensitivity is estimated at 50–70%. Based on the clinical and histological features the patient was diagnosed with stage IA FMF. FMF is characterized histologically by atypical CD4+ T lymphocytes that surround and infiltrate the hair follicles (folliculotropism) and should be considered in lesions resembling keratosis pilaris but in an unusual distribution and/or in clusters. Epidermotropism is typically absent in FMF, and concomitant follicular mucinosis is frequently observed. Paediatric follicular MF is rare, but this case highlights that in cases of suspected alopecia mucinosa, even in paediatric patients, a diagnosis of FMF merits careful consideration (Reiter O, Amitay-Laish I, Oren-Shabtai M et al. Paediatric mycosis fungoides – characteristics, management and outcomes with particular focus on the folliculotropic variant. J Eur Acad Dermatol Venereol 2022; 36: 671–9).
PA23 Glomuvenous malformation or blue rubber bleb naevus syndrome: a case highlighting how a genetic diagnosis can avoid invasive testing and the need for long-term follow-up
Authors: Lapsley Rebecca , Darné Sharmela , Brennan Paul , Carmichael Andrew
Published: June 2024

Glomuvenous malformation is an inherited autosomal dominant venous malformation that presents with multifocal cutaneous blue purple vascular lesions. An important differential that should be excluded is blue rubber bleb naevus (BRBN) syndrome, which presents with similar cutaneous lesions. However, the critical difference is that the latter is associated with visceral lesions including in the gastrointestinal tract. This can cause intussusception or haemorrhage with significant sequelae including severe anaemia, consumptive coagulopathy and sometimes death. These patients require gastrointestinal investigations and close long-term follow-up. We describe the case of a 16-year-old girl who presented to clinic with multiple small venous malformations affecting her eyelid, arms, knee and back. Most lesions were present from birth; however, some were acquired in childhood and had increased in size. Some were tender but there was no reported bleeding. She had no significant past medical history. Her mother and maternal grandmother had similar lesions. Full blood count and coagulation studies were normal. Genomic DNA from blood samples was sent for a venous malformation panel, which demonstrated a glomulin (GLMN) c.157_161del mutation. This confirmed the diagnosis of familial glomuvenous malformation rather than BRBN syndrome. BRBN syndrome and glomuvenous malformation present as clinically similar entities with primary purple–blue papules, which are often multifocal and widespread. Lesions are often congenital but can increase in number and size particularly during puberty. Both conditions are inherited in an autosomal dominant manner (Lu R, Krathen R, Sanchez R et al. Multiple glomangiomas: potential for confusion with blue rubber bleb nevus syndrome. J Am Acad Dermatol 2005; 52: 731–2). Histopathology can be useful to distinguish between the two conditions; however, this requires a biopsy. Loss-of-function mutations in the GLMN gene cause glomuvenous malformations. These were first reported by Brouillard et al. in 2002, and c.157­_161del is the most frequent mutation (Brouillard P, Boon LM, Revencu N et al. Genotypes and phenotypes of 162 families with a glomulin mutation. Mol Syndromol 2013; 4: 157–64). With this case we wish to highlight how useful genetic testing is diagnostically for patients presenting with these cutaneous lesions. The discovery of the GLMN gene has allowed clinicians to distinguish glomuvenous malformations from other more concerning venous anomalies such as BRBN syndrome and thus avoid an invasive biopsy. Patients can be reassured and avoid the need for long-term follow-up, regular blood tests or gastrointestinal investigations.
British Society for Skin Care in Immunosuppressed IndividualsOral presentationsBI01 Intralesional triamcinolone acetonide and antiretroviral therapies: a lesser considered drug interaction
Authors: Fagan Nicole , Ryan Aisling
Published: June 2024

Antiretroviral therapies (ART) have transformed the management of HIV, leading to increased life expectancy. Dermatologists now encounter a substantial number of patients on ART, necessitating awareness of potential drug interactions. While interactions with oral and intravenous medications are well documented, the impact of ART on intralesional triamcinolone acetonide (TA) is less well recognized. A 43-year-old female patient presented with a 10-year history of keloid scarring secondary to a previous minor trauma. She had a history of HIV and was managed on a regimen of elvitegravir, cobicistat, emtricitabine and tenofovir alafenamide. Her most recent viral load was undetectable. On examination she had Fitzpatrick skin type VI. There was a 2 × 1-cm firm, thick, nontender, dumbbell-shaped nodule consistent with a keloid scar on her central chest. Her quality of life was significantly affected due to the cosmetic appearance, itch and pain. She was treated with intralesional TA 40 mg mL−1 at a dose of 0.1 mL per cm2. Shortly after treatment, correspondence was received from her treating infectious diseases team identifying a contraindication between cobicistat and TA due to the risk of iatrogenic Cushing syndrome (ICS) and secondary adrenal insufficiency (SAI). On review, 33 cases of ICS and SAI in patients concurrently treated with TA and ritonavir have been described in the literature. Three cases have been reported in patients on concurrent cobicistat. The method of administration of TA in these reported cases included intra-articular, epidural, intramuscular and intrabursal routes. Only one case was documented in a dermatology journal, emphasizing the need for increased awareness among dermatologists. Importantly, in some of these cases ICS and SAI developed after a single injection. Ritonavir and cobicistat are potent pharmacokinetic enhancers in HIV treatment, inhibiting cytochrome P450 3A4 (CYP3A4) activity. TA is also metabolized through CYP3A4 leading to increased serum cortisol with concomitant administration. A search using the University of Liverpool’s ‘HIV Drug Interactions Checker’ identified 10 regimens in which coadministration of TA injections are contraindicated and 1 potentially significant interaction. While ART and associated medications are typically managed by HIV specialists, prospective identification and avoidance of harmful drug interactions require communication and heightened awareness among clinicians and patients. The risk of ICS and SAI, even with a single injection, underscores the importance of thorough medication documentation and consideration for drug–drug interactions in patients with HIV, even in topical or intralesional routes.
BI03 The presence of VEXAS-related UBA1 mutation in a patient with myelodysplastic syndrome
Authors: Vetsiou Evangelia , Sekaran Arvindh , McDonald Sarah , Crawley Charles , Khoo Andre
Published: June 2024

A 56-year-old female patient with a background of myelo­dysplastic syndrome (MDS) was admitted with a febrile illness and a solitary, bullous skin lesion on her right inner thigh. This lesion started as a scaly patch 1 week prior to admission before developing into a large tense bulla with associated lymphadenopathy in the groin. The patient had a background of MDS, which was being treated with donor lymphocyte infusions, having relapsed from a previous allogeneic bone marrow transplant. Initial treatment was with intravenous antibiotics. While ultrasound did not reveal any clear underlying abscess, surgical debridement was performed due to the patient’s deteriorating clinical state and concerns about an infective focus within the wound. Histological analysis showed a neutrophilic infiltrate, raising suspicion of pyoderma gangrenosum or other neutrophilic dermatoses. A UBA1 mutation of uncertain significance was detected in the perivascular histiocytes, raising the possibility of VEXAS (vacuoles, E1 enzyme, X-linked, autoinflammatory, somatic) syndrome. Following debridement and exclusion of an infective aetiology, the wound was managed with vacuum-assisted wound closure and a slowly weaning course of 40 mg oral prednisolone over 4 months. The patient subsequently underwent a second allogeneic bone marrow transplant, complicated by sepsis and requiring admission to critical care. During this hospitalization, she developed haemorrhagic lesions that were also considered to be due to an inflammatory process driven by her MDS. These were resolved following initiation of cyclophosphamide immunosuppression after her transplant. The cutaneous manifestations of MDS encompass neutrophilic dermatoses, cutaneous vasculitis and granulomatous skin lesions. Recognition of these manifestations has therapeutic and prognostic importance, as they can precede a diagnosis of MDS and be an indicator of poor prognosis. Differentiating classic MDS from VEXAS syndrome is critical, as the latter lacks additional molecular abnormalities and exhibits less cytological heterogeneity. However, a complex interplay exists between MDS and systemic autoimmune/autoinflammatory diseases (AID), with an undefined pathophysiological mechanism. In this case, we describe the development of known AID associated with underlying MDS in the presence of a VEXAS-related UBA1 mutation, which is responding favourably to stem cell transplant and immunosuppression. The patient is currently in recovery without further skin manifestations.
BI09 Skin cancer burden and dermatological follow-up in solid-organ transplant recipients: a regional study
Authors: Gilbert Georgia , Gupta Girish , Jones Ruth
Published: June 2024

Skin cancer is the most common malignancy affecting solid-organ transplant recipients (SOTRs). This places a significant burden on dermatology and transplant services. The National Institute for Health and Care Excellence recommends that SOTRs with high-risk skin lesions should be reviewed in a dedicated skin surveillance clinic [National Institute for Health and Care Excellence. Improving outcomes for people with skin tumours including melanoma. Available at: https://www.nice.org.uk/guidance/csg8 (last accessed 24 March 2024)], although service provision varies throughout the UK. There is currently no routine skin surveillance service in the region assessed in this study. In anticipation of the British Association of Dermatologists’ expert consensus guidelines for skin cancer surveillance in SOTRs, we aimed to analyse the skin cancer burden and skin surveillance practice within our region. As of November 2023, there were 749 living SOTRs in the region. Patient demographics, transplant type(s), immunosuppression regimens, skin cancer burden, dermatological follow-up and provision of skin cancer prevention advice were retrospectively collected from medical records. After exclusions were made, 648 patients were included for analysis. Pearson’s correlation, χ²-tests and t-tests were used to assess between-group differences. Overall, 15.4% of SOTRs had a skin cancer diagnosis [n = 100; squamous cell carcinoma (SCC) n = 54, basal cell carcinoma n = 59, melanoma n = 6] since transplantation. We found that 17.2% of SOTRs were under regular dermatological follow-up for skin surveillance (n = 112). In terms of SCC frequency, 26 patients had 1 lesion and 27 patients had 2 or more lesions. A total of 68 patients (10.5%) were noted to have extensive actinic keratoses. Older age (P = 0.01), male sex (P = 0.03) and increased time on immunosuppression (P = 0.03) were correlated with presence of skin cancer. The most common immunosuppressant regimen was mycophenolate mofetil (MMF), tacrolimus and prednisolone. The use of MMF seemed to be correlated to SCC burden (P = 0.009), but this is difficult to interpret in the context of multiple immunosuppressant agent use. Only 7.3% (n = 47) of SOTRs were prescribed suncream and 3.7% (n = 24) were offered acitretin. Our study highlights that there is a lack of consistency with regard to optimal skin cancer prevention follow-up and advice, in a region that does not have a routine dermatological skin surveillance service for SOTRs. Limited service resources highlight the importance of collaborative consensus guidelines on best practice for the prevention of skin cancer in SOTRs. Therefore, we have instituted a set of recommendations within our region including guidance regarding prescribed sunscreen use, pathways on how best to access dermatology for SOTRs presenting with skin lesions, and follow-up based on stratification of risk factors.
BI16 Real-life experience using topical nitrogen mustard gel in the treatment of stage IA–IVA2 mycosis fungoides: a single-centre experience of 66 patients
Authors: Bakr Farrah , Mensah Rita Entsua , Lusted Claire , Child Fiona , Thomas Bjorn , Whittaker Sean , Morris Stephen , Sim Van , Wain Mary
Published: June 2024

Mycosis fungoides (MF), the most common subtype of primary cutaneous T-cell lymphoma, is characterized by the proliferation of skin-homing, malignant CD4+ T cells. In early-stage disease (IA–IIA) patients develop cutaneous patches and plaques; late-stage disease (IIB–IVB) is characterized by tumours, erythroderma, and blood and/or lymph-node involvement. Effective skin-directed treatment options for all stages of disease are limited. Topical chlormethine gel (Ledaga), known as nitrogen mustard, is an antineoplastic alkylating agent, licensed in England in August 2021 for the treatment of MF in patients who have received prior skin-directed therapy (Lessin SR, Duvic M, Guitart J et al. Topical chemotherapy in cutaneous T-cell lymphoma: positive results of a randomized, controlled, multicentre trial testing the efficacy and safety of a novel mechlorethamine, 0.02%, gel in mycosis fungoides. JAMA Dermatol 2013; 149: 25–32). The aim of this study was to evaluate our experience of using chlormethine gel in a single UK centre and to offer practical guidance in its use. All patients who commenced topical chlormethine gel in our unit between January 2022 (when chlormethine gel was first available to us) and December 2023 were identified. An intention-to-treat, retrospective analysis of medical records was performed. In total, 66 patients (58% male sex) were identified with a mean age at the start of treatment of 60.3 years (SD 14.6). Multiple stages of MF (IA 35%, IB 47%, IIB 9%, III 2%, IVA1 2%, IVA2 6%) were represented. Prior to commencing chlormethine gel, all patients had used topical steroids; 17% had received no other prior treatment. Overall, 18% were receiving concomitant systemic therapy, phototherapy or radiotherapy for their MF during chlormethine treatment. The mean duration of treatment with chlormethine gel was 264 days; 64% of patients consider the treatment a success and continue ongoing episodic treatment or have stopped due to a complete or partial response. A total of 8% of patients stopped due to disease progression, 5% due to lack of response and 21% due to unacceptable side-effects. Overall, 82% of all patients experienced toxicities; these were exclusively cutaneous, most commonly erythema (32%) and pruritus (32%). Toxicities were largely managed with brief treatment breaks, reducing the frequency of chlormethine application, emollients and topical steroids, enabling patients to continue treatment. We present real-life data on the use of topical chlormethine gel in our cohort of patients with MF and demonstrate that it is an effective, well-tolerated skin-directed therapy. Skin toxicities can generally be managed conservatively.
BI29 Mycosis fungoides presenting in a patient with NFKB1 haploinsufficiency-associated common variable immunodeficiency
Authors: Carty Kieran , Crotty Gerard , Molloy Kevin
Published: June 2024

A 52-year-old man with a history of common variable immunodeficiency (CVID) secondary to NFKB1 haploinsufficiency was referred to the dermatology clinic. He had a worsening rash on his arms and legs with a background of a longstanding rash on his trunk for the last 2 years and his groin area for the preceding 12 years. He was diagnosed with CVID in 2009 and was receiving three-weekly intravenous immunoglobulin infusions. He had a history of childhood eczema and his son also had a history of eczema. On examination, the patient had erythematous infiltrated impetiginized plaques on his upper abdomen, buttocks and thighs and more subtle patches on his right chest consistent with a diagnosis of mycosis fungoides. His modified Severity-Weighted Assessment Score was 27.6 (patch 3.4%, plaque 12.1%). His staging investigations identified a weak clonal T-cell population in his bone marrow biopsy [B0b (L) lymphopenia]; a fludeoxyglucose F18 (FDG) avid lymph node in the region of his rash (likely dermatographic) and a discordant B-cell lymphoproliferation in biopsies of his FDG avid lung lesions. Lymphoma occurs in an estimated 2–8% of patients with CVID, the majority of which are non-Hodgkin lymphoma. To our knowledge, this is the first reported case of mycosis fungoides, the most common form of cutaneous T-cell lymphoma (CTCL) arising in a patient with NFKB1 haploinsufficiency. A recent report by Spangenberg et al. (Spangenberg MN, Grille S, Simoes C et al. Case report: mycosis fungoides as an exclusive manifestation of common variable immunodeficiency in a family with a NFKB2 gene mutation. Front Oncol 2023; 13: 1248964) described a family with two brothers who presented with mycosis fungoides as an exclusive symptom of a CVID, where whole-exome sequencing of the index patient revealed a pathogenic variant of the NFKB2 gene. High constitutive nuclear factor (NF)-κB activity in CTCL is responsible for increased malignant cell survival and proliferation and induction of NF-κB-dependent proinflammatory and anti-inflammatory cytokines. The role of NF-κB in CTCL lymphomagenesis warrants further investigation. Our case highlights that not only does a gain in NF-κB activity appear to confer an increased risk of developing mycosis fungoides, but a loss of such activity can also appear to confer an increased risk.
DE04 Using an ‘umbrella clinic’ format to optimize educational experiences for trainees and clinical supervisors in dermatology
Authors: Holloran Sophie , Myo Su Le , Whitley Helen
Published: June 2024

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.
Poster presentationsDE06 Incorporating the ‘clinical teacher’ role in job plans: a way to increase medical students’ hands-on experience?
Authors: Piranie Humayra , Helbling Ingrid
Published: June 2024

Around 60% of dermatological conditions are managed by nondermatology specialist doctors (Lowell BA, Froelich CW, Federman DG, Kirsner RS. Dermatology in primary care: prevalence and patient disposition. J Am Acad Dermatol 2001; 45: 250–5). Despite this, a survey of junior doctors found that only 8% of doctors felt confident in initiating basic therapy for a skin complaint (Hussain W, Hafiji J, Stanley AG, Khan KM. Dermatology and junior doctors: an evaluation of education, perceptions and self-assessed competencies. Br J Dermatol 2008; 159: 505–6). This highlights that there is a need to evaluate and improve current ‘hands-on’ dermatology undergraduate training. A quality-improvement project was done looking at fourth-year medical students on clinical placement in dermatology at a UK university hospital. Previous feedback showed that while attending dermatology clinics, 50% of students reported they did not have the opportunity to take any histories, and only 41% received feedback from clinicians. Students were asked to rate their satisfaction of their placement on a scale of 1 to 5; the average student score was 2.6. Based on this feedback, we decided to commit more clinicians to have a specific educational role as part of their job plan. Only one out of the existing nine consultants expressed an interest. This led to us organizing for one existing and four new senior dermatology colleagues, who had recently joined the department, to be assigned as ‘clinical teachers’ in their job plans. They were allocated 1–2 h per week dedicated to education (0.25–0.5 SPA – supporting programmed activity). Funding was from the existing dermatology undergraduate teaching budget. The clinical teachers were assigned to tutor a couple of students a week and to give them personalized feedback. Students were asked to assess the quality of this feedback through their online platform ‘My Knowledge Map’. The students also logged the number of histories they were able to take in clinic and with which clinician; this allowed us to organize a prize for the most accommodating clinician per block. The clinical teachers were also expected to deliver dermatology teaching sessions in a 1/7 rota, and to be involved in assessments. Through these changes, the percentage of students who were able to take a history during their dermatology week increased to 77% and 91% of these students received feedback on their skills. Overall student satisfaction in clinics increased from 2.3 to 3.4 out of 5. Our findings show that assigning a specific ‘clinical teacher’ role in the job plans of dermatology clinicians increased students’ hands-on experience, the amount of personalized feedback they received and overall student satisfaction.
GD02 An Indian study of various unusual presentations of cutaneous tuberculosis: challenges in its diagnosis and management
Authors: Bhargava Shashank , Kumar Ujjwal
Published: June 2024

Extrapulmonary tuberculosis (EPTB) is a serious health problem in both developing and developed countries. In India, cutaneous tuberculosis (TB) constitutes about 0.1–2% of all outpatients with skin diseases. We were surprised to see many cases of TB with unusual presentations, which led to a diagnostic dilemma. All suspected cases of cutaneous TB were studied over a period of 5 years (2017–2021). Written consent was obtained from all patients. Thorough dermatological examinations and later skin biopsy, Mantoux test, chest X-ray and other supporting tests were performed to confirm the diagnosis of TB. The treatment outcomes were also analysed. In total 88 patients were confirmed to have cutaneous TB. There were more female patients (58 of 88) than male. Lupus vulgaris (46 of 88) was the most common presentation, followed by TB verrucosa cutis (TBVC). Lupus vulgaris had head and neck involvement in the majority of the cases. Mantoux positivity was observed in 80% of patients (70 of 88), while raised erythrocyte sedimentation rate (ESR) was seen in 89% (78 of 88). A few cases of lupus vulgaris were very confusing and presented similarly to deep fungal infections, hypertrophic lichen planus, mycetoma, subacute lupus and cutaneous lupus erythematosus, to name a few. Other variants of EPTB also had very close differentials and were managed with antitubercular therapy (ATT). Seven patients had serology positive for HIV. Histopathology was conclusive in 93% of cases (82 of 88). All of the variants of cutaneous TB such as lupus vulgaris, scrofuloderma and TBVC responded excellently to ATT. In conclusion, the present study emphasizes the importance of clinicopathological correlation and therapeutic response in cutaneous TB. Although positive results of ESR, Mantoux reactivity and TB cultures facilitate the clinical diagnosis, negative results should not exclude the diagnosis of cutaneous TB. Also, cutaneous TB is a great mimicker in dermatology, especially in a tropical countries like India.