bluuebunny/crossref_metadata_2025_split huggingface dataset:
DS20 Challenges and considerations of Mohs surgery in the ear
Authors: Kloczko Ewa , Wilsher Mark , Naparus Andreas , Kulendra Kevin , Anjum Navara 
Published: June 2024

Abstract
Malignancy of the external ear represents 6% of all skin cancers, and basal cell carcinomas (BCCs) account for 40% of those. When infiltrative, these BCCs may pose unique treatment challenges given the complexity of the local anatomy and the proximity to other local structures, such as the parotid gland, the external auditory canal, the tympanic membrane, the middle ear and the facial nerve. We describe a case of a 69-year-old man who presented with a 5-year history of a crusted lesion in the left year. Inability to wear a hearing aid due to pain and discharge from the left ear prompted the patient to attend the emergency department. Following the ear–nose–throat assessment, he was referred to skin cancer clinic. Initial biopsy confirmed a clinical suspicion of an infiltrative BCC affecting the left antihelix and conchal bowl extending to the entrance of the external auditory meatus (EAM). Following a multidisciplinary approach between plastic surgery, dermatology and the head and neck team, three treatment options were discussed with the patient: Mohs micrographic surgery, standard excision, and radiotherapy. Preoperative imaging revealed the tumour abutting the parotid gland, but there was no involvement of the bony or cartilaginous aspects of the EAM. The patient pursued the Mohs procedure to preserve function and cosmesis of the ear. After two stages margins remained positive. The decision was made to stop the procedure, given the evidence of extension to the EAM. Two days later the patient underwent partial pinnectomy, which had evidence of a BCC extending to all margins with infiltration into the parotid gland and cartilaginous ear canal. Focal perineural invasion was present. Subsequently, this case was discussed with the head and neck team, with a multidisciplinary decision recommending a definitive resection. The patient underwent superficial left parotidectomy and complete pinnectomy including the cartilaginous external ear canal and tympanic membrane, followed by reconstruction with an anterolateral-thigh free flap. The patient received a prosthetic left ear and is currently awaiting an assessment for a bone-anchored hearing aid. Histologically, aggressive BCC was detected in clinically unremarkable areas of the pinna. It is likely that the multiple embryonic fusion planes within the EAM account for less predictable tumour spread. This case highlights the complexity of Mohs surgery on the ear and the need for a collaborative approach in management of those areas to provide the best quality of care for our patients.
DP14 ALK-positive histiocytosis with involvement confined to the skin: report of two cases in adults
Authors: Lim Su Xin , Pugalenthy Sarajeni , Wilson Niall , Vasile Cristina , Bakshi Arti , Cohen Stuart 
Published: June 2024

Abstract
We present two interesting cases of anaplastic lymphoma kinase (ALK)-positive histiocytosis in adults. The first was a 79-year-old woman with a 10-year history of a firm, violaceous, exophytic nodule on her right upper arm. It was asymptomatic, and stable in size and colour. It measured 11 mm in diameter and had a scaly surface. Dermoscopy was nondiagnostic, showing a featureless, violaceous appearance. Histology revealed a dermal histiocytic infiltrate with epithelioid and foamy histiocytes and occasional Touton-type giant cells. Immunohistochemistry showed the histiocytes to be CD68 and ALK positive (clone AlkD5F3). S100 and CD1a were negative. The second case was a 42-year-old man with an enlarging, latterly tender lesion on the chest for many years. Examination showed a nonspecific 5 × 4-mm shiny pink papule. Excision histology revealed a dermal histiocytic infiltrate, with numerous lymphocytes and focal emperipolesis. Immunohistochemistry showed the histiocytes to be CD68 and S100 positive with patchy ALK positivity (clone AlkD5F3). CD1a was negative. A diagnosis of ALK-positive histiocytosis was concluded in both cases. This rare subtype involves a genetic alteration in genes encoding receptor tyrosine kinases. Its full clinicopathological features have not been fully elucidated. It most commonly presents in young infants as a multisystem disease with liver and haematological disturbances. Localized disease is more common in adults, for which the prognosis is good. Clinicopathological correlation is important as the clinical features are usually nonspecific. Previous reports involving cutaneous disease have tended to focus on extracutaneous manifestations, although papules, which may be yellow or purple, at various sites have been described. Symptoms from the skin lesions have not been clearly reported. Histopathologists need to be aware of this new entity and include ALK immunostain in all dermal histiocytic lesions. The morphology is variable, consisting of an admixture of epithelioid and foamy histiocytes, sometimes with emperipolesis and thus mimicking juvenile xanthogranuloma and Rosai–Dorfman disease morphologically. Previous cases have found gene fusions of KIF5B–ALK, TPM3–ALK, CLTC–ALK, TFG–ALK, EML4–ALK and DCTN1–ALK, and ALK-fluorescence in situ hybridization positivity. The clinical relevance of this gene fusion is not known. Blood tests and imaging to assess for other organ involvement should be considered. Retrospectively our cases showed no signs or symptoms of other organ involvement, and investigations were clear. Targeted ALK inhibitor therapy has shown responsive results in treating ALK-positive histiocytosis with multisystem involvement. Surgical resection has been successful in treating cutaneous disease, with no reported recurrence. As further cases of this rare condition come to light, its full clinicopathological and molecular features may become more apparent.
PA27 Expanding the phenotype of congenital erosive and vesicular dermatosis
Authors: Moxham Lindy , Felton Sarah , Lam Joseph 
Published: June 2024

Abstract
Congenital erosive and vesicular dermatosis (CEVD) is a rare condition that typically presents at birth with erosions and vesicles (Cohen B, Esterly N, Nelson P. Congenital erosive and vesicular dermatosis healing with reticulated and supple scarring. Arch Dermatol 1985; 121: 361–7). Lesions heal with distinctive reticulate and supple scarring and can involve &gt; 75% of the skin surface (Tlougan BE, Paller AM, Schaffer JV et al. Congenital erosive and vesicular dermatosis with reticulated supple scarring: unifying clinical features. J Am Acad Dermatol 2013; 69: 909–15). The pathogenesis of CEVD is unknown, although hypotheses include intrauterine infection, amniotic adhesions, or a developmental defect in skin healing. Histopathological findings early in the disease process can include epidermal necrosis, subepidermal vesiculation and an eroded epidermis. Older lesions demonstrate scar formation, decreased hair follicles, and absent eccrine glands. Historically, CEVD has been described as preferentially affecting the trunk and limbs, with sparing of the face and volar surfaces. Patients with CEVD have been reported to have higher prevalence of premature birth, neurodevelopmental abnormalities, nail abnormalities, tongue atrophy or sores, alopecia, and cardiac defects. Here, we highlight two patients with presumed CEVD with atypical features. Patient A presented at 13 months with a history of linear erosions and vesiculation over their scalp, forehead and posterior left flank. The lesions were present at birth and healed with reticulate scarring with no further flares. Patient B presented at 11 months with a history of stellate scarring over the lower abdomen, with noncontiguous involvement of the right and left axillae, scalp, and right forehead. The lesions were also present at birth and were initially more erythematous and developed milia-like cysts. The cysts resolved, leaving reticulate and supple scarring. Of note, patient B had no erosions or vesicles. At the time of assessment, both children were otherwise healthy and developing normally. These cases add to evidence that CEVD is neither facial nor scalp sparing, as commonly described, and can be more localized than previously thought, but affect noncontiguous sites. Case B also demonstrates that, despite the name, CEVD can present without erosions, and instead with milia-like cysts. The cases also demonstrate that CEVD can occur in the absence of other congenital abnormalities; outcomes for children with the condition are then more favourable. Both families were provided with reassurance that although the scarring would not disappear completely, and can be of cosmetic concern, CEVD is a nonprogressive condition and thus no specific treatment is indicated.
PA28 Reactive infectious mucocutaneous eruption: a case series of four children
Authors: Muttoni Elisabetta , Hale Gordon , Jury Catherine , Beattie Paula 
Published: June 2024

Abstract
Reactive infectious mucocutaneous eruption (RIME) is a reactive inflammatory mucositis most commonly but not exclusively associated with Mycoplasma pneumoniae. It presents with a mucosal- predominant eruption with sparse to no skin involvement. We describe four recent cases in children between 6 and 13 years of age. All four children presented with increasing lip discomfort with subsequent lip swelling, sloughing and crusting. The intraoral mucosa was affected to varying degrees. All described a prodromal illness with cough and general malaise, and three patients also reported fevers. Skin manifestation varied, with two patients having no cutaneous involvement while two patients had sparse pink macules and negative Nikolsky sign. Investigations included an extended respiratory screen and chest X-ray to check for underlying infection, and M. pneumoniae was detected in two patients. Skin swabs for virology and bacteriology were also taken. Management focused on pain control, nutritional support and good mouth care. The patients required admission to hospital for fluid resuscitation and analgesia. Mouth care was implemented with regular benzydamine hydrochloride spray and Gelclair gel, and white soft paraffin alongside Trimovate cream (oxytetracycline and nystatin) for the lips. Due to ongoing diffuse erosions of the buccal mucosae with haemorrhagic crusting of the lips, one patient required a short course of oral steroids. Reactive infectious mucocutaneous eruption was previously termed Mycoplasma pneumoniae-induced rash and mucositis; however, reports of this entity being caused by other infections including enterovirus, influenza B virus and SARS-Cov-2 has led to a change in terminology (Mahama A, Kojder P, Thibodeaux Q, Ruth J. Reactive infectious mucocutaneous eruption following COVID-19 in an adolescent boy: case report and review of the literature. Pediatr Dermatol 2023; 40: 162–5). Mucosal membranes are significantly more affected than skin in RIME. Skin involvement can be sparse or absent, differentiating it from Stevens–Johnson syndrome/toxic epidermal necrolysis, which presents with rapidly progressive skin necrosis and is often drug induced, and from erythema multiforme, which is characterized by typical target lesions (Tonkin R, Ladha M, Johnson N et al. Reactive infectious mucocutaneous eruption – repeat etanercept after intravenous immunoglobulin: a case report. SAGE Open Med Case Rep 2022; doi: 10.1177/2050313X221117887). Investigations for underlying respiratory infection and treatment with appropriate antibiotic therapy should be implemented. Supportive care in the form of pain relief, and nutritional and fluid support is important. Systemic steroids may be required to truncate the inflammatory response, although there is a lack of prospective data. In conclusion, our aim is to bring awareness to this condition to facilitate its recognition, diagnosis and treatment.
PA32 A rare case of symmetrical giant facial plaque-type juvenile xanthogranuloma
Authors: Darne Sharmela , Makanjuola Abiola 
Published: June 2024

Abstract
Juvenile xanthogranuloma (JXG), a form of non-Langerhans cell histiocytosis, is a rare benign condition estimated to affect 1 per million children. In 80% it presents as a solitary papule or nodule. There are several described subtypes of JXG including mixed, giant, subcutaneous, eruptive, clustered and plaque-like. Symmetrical giant facial plaque-type JXG is a very rare subtype, first described by Gunson and Birchall in 2008 [Gunson TH, Birchall NM. Symmetrical giant facial plaque-type juvenile xanthogranuloma. J Am Acad Dermatol 2008; 59 (2 Suppl. 1): S56–7]. We present a 2-year-old girl with a slowly progressive, asymptomatic, symmetrical orange-coloured rash on both cheeks that started around age 1 year. She was otherwise well. Examination showed well demarcated, orange-coloured indurated plaques covering both cheeks in a mask-like distribution. There was no lymphadenopathy or hepatosplenomegaly. There were no signs of neurofibromatosis. Full blood count, renal and liver function tests and lipids were normal. Biopsy showed subepidermal sheets of histiocytic proliferation. Lesional cells were positive for CD68 and negative for SOX10, compatible with JXG. She is currently awaiting ophthalmology assessment and an ultrasound scan of her abdomen. Over the course of her follow-up, the plaques have slowly extended, spreading onto the temples and chin, the axillae and both upper arms. JXG belongs to the proliferative dendritic-cell-related disorders and is the commonest form of non-Langerhans histiocytosis (Itin K, Häusermann P, Itin P, Fosse N. Symmetrical facial giant plaque-type juvenile xanthogranuloma: case report and review of the literature. Case Rep Dermatol 2021; 13: 399–406). It is most often characterized by a solitary red–yellowish benign papule (&lt; 0.5 cm) or nodule (&lt; 2 cm) that usually involves the head and neck (42%) or trunk (20–40%), although involvement at any body site can occur, including extracutaneous sites (the eye being the most common). It may be congenital, but most cases develop in the first year of life. It more commonly affects male patients, in a ratio of up to 7 : 1. There is usually spontaneous resolution, which takes months to years, leaving an atrophic scar. The giant symmetric facial subtype of JXG is exceptionally rare, with only five reported cases worldwide. In these cases, there was no spontaneous resolution during a follow-up period of between 2 and 6 years. None of the reported cases had systemic or extracutaneous involvement. Treatment options are undefined – one child had a partial response to a single fractionated CO2 laser treatment. We present this case to highlight awareness of this unusual presentation.
BI13 (P065) Attitudes and practices of patients with inflammatory bowel disease towards skin cancer risk and sun protection
Authors: Herlihy Maeve , Blake Eve , Courtney Garry 
Published: June 2024

Abstract
Skin cancer is the most common form of cancer in Ireland, with over 13 000 new cases diagnosed annually. People with inflammatory bowel disease (IBD) may be at higher risk of skin cancer. A meta-analysis performed on more than 170 000 patients diagnosed with IBD found that patients with IBD had a 37% higher risk of developing melanoma than expected in the general population (relative risk 1.37, 95% confidence interval 1.10–1.70). This increased risk was independent of IBD treatment. Additionally, many of the treatments used for IBD involve suppressing or altering the immune system. Immunosuppression is a risk factor for all types of skin cancer, and cutaneous malignancies in immunocompromised patients tend to behave more aggressively compared with those in the general population. The British Association of Dermatologists has published preventive guidelines for patients on immunosuppression, but specific gastroenterology recommendations are lacking. Prevention and early detection are therefore of paramount importance. Our aim was to assess knowledge of skin cancer risk factors and photoprotective behaviours in an Irish cohort of at-risk patients with IBD. A questionnaire was given to patients attending our IBD clinic. Clinical data were recorded including diagnosis, immunosuppressants, skin cancer risk factors and photoprotective behaviours. In total, 87 patients (52% women) with IBD (63% Crohn disease, 35% ulcerative colitis) were included. All participants were White, with 77% recorded as having ‘fair’ or ‘very fair’ skin. Most patients (97%) were on treatment for their IBD (66% on biologics, 25% on immunomodulators). Over one-third of participants (35%) were unaware of an increased skin cancer risk associated with IBD. Four patients had a history of skin cancer, two of which were melanoma. Half of patients (48%) had been sunburnt in the last year, and 36% had more than 10 burns in their lifetime. In total, 57% of patients reported a history of blistering sunburn and 22% of patients had used sunbeds. Knowledge of skin cancer risk factors and worrisome features was generally good, and the majority of our cohort (74%) always wore suncream abroad. However, less than half wore suncream frequently in Ireland (37%) or when it is cloudy (22%), and also did not take other important measures, such as reapplying suncream every 2 h (43%), or wearing a hat (26%) or protective clothing (32%). Our study highlights gaps in the education of our IBD cohort regarding skin cancer risk and prevention, and suggests that primary prevention of skin cancer in this IBD cohort is suboptimal. Ideally, educational interventions to enhance patient awareness and encourage adequate photoprotection and skin self-surveillance should be undertaken and assessed.
Poster presentationsBI18 The management of vulval intraepithelial neoplasia in immunosuppressed women: outcome and recommendations from a multidisciplinary vulval clinic
Authors: Bali Radhika , Chung Jenny , English Caroline , McDermott Jacqueline , Staley Helen , Harish Sapna , Cohen Charlotte , Palmer Elaine , Fuller Claire , Butler John , Akel Randa 
Published: June 2024

Abstract
Vulval intraepithelial neoplasia (VIN) is a premalignant change of the vulval epithelium that can progress to squamous cell carcinoma (SCC). Immunosuppressed patients are a high-risk subset, with a greater risk of recurrence with standard treatment. To date, there is limited research focusing on this vulnerable population. A retrospective study was designed to review the treatment outcomes of immunosuppressed patients with VIN in a tertiary, multidisciplinary vulval service. In total, 104 biopsy-proven adults with VIN were identified from a histological database from 1 April 2013 to 31 March 2023. Patient demographic data, disease type and treatment outcome were collected. Electronic records and medical photography, where available, were used to retrospectively map each vulval lesion in quadrants. Complete response (CR) was defined as being clinically and/or pathologically clear of disease at 3 months’ follow-up. Recurrence was defined as lesions reappearing in the same quadrant after achieving CR. Data analyses were performed using IBM SPSS Statistics for univariate analyses. Overall, 31 of 104 patients (30%) were immunosuppressed, developing 47 lesions in the study period. This was due to HIV (n = 9), diabetes (n = 5), active malignancy (n = 10) and other causes (n = 7). The mean age was 54 years (range 20–94) with a mean follow-up duration of 70 months (range 11–132). The histological subtypes of VIN included low-grade squamous intraepithelial lesion (11%), high-grade squamous intraepithelial lesion (72.3%), differentiated VIN (4.3%) and microinvasive SCC (14.9%). Immunosuppressed patients were more likely to have multifocal disease (57.4% vs. 29.8%, P = 0.003) with previous human papillomavirus (HPV) burden (66.7% vs. 36.6%, P = 0.008). For those screened during the study period, immunosuppressed patients were more likely to have associated anal intraepithelial neoplasia (25.8% vs. 6.9%, P = 0.02) and cervical intraepithelial neoplasia (29.0% vs. 15.2%, P = 0.031). The CR rate from first-line treatment (imiquimod, surgery, laser or PlasmaJet®) was 54.8% in the immunosuppressed cohort compared with 75.6% in the control group (P = 0.073). There was no difference in the rate of recurrence or progression to SCC. There was no death due to disease observed in the immunosuppressed group, compared with three patients in the control group. In summary, our high-risk population with VIN was found to have a higher burden of HPV and was more likely to have multicentric disease. The treatment outcome was noninferior to the control group’s, unlike similar previous case series. This highlights the need for multidisciplinary clinics offering long-term close monitoring, with early treatment and HPV surveillance.
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: Altayeb Alla , O’Kelly Sara Lupita , Howard Lucy , Deane Eleanor M , Walker-Smith Philippa , Birnie Andrew 
Published: June 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 &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.