bluuebunny/crossref_metadata_2025_split huggingface dataset:
PA17 Multiple lesions in a child mimicking juvenile xanthogranulomas
Authors: Hiranput Siwaporn , Raweily Essam , Alsaadi Dalia Saidely 
Published: June 2024

Abstract
An 11-year-old White girl presented with a lesion on her left arm, present since she was a toddler. The lesion had increased in size over the past year with associated discomfort. Two further lesions appeared over a 12-month period. She was born at full term, and had no developmental delay and no past medical history. On examination, a 15 × 20-mm firm yellow–orange plaque was noted on her left upper arm without epidermal changes, and two smaller lesions with similar appearance were present on her right upper arm and right posterior thigh. There was no palpable lymphadenopathy or hepatosplenomegaly. She did not have dysmorphic facial features or short stature. The clinical impression was that of juvenile xanthogranulomas. As the arm lesion was symptomatic, it was excised with a narrow margin. Excisional biopsy showed cells with abundant eosinophilic granular cytoplasm, bland chromatin and nuclei. There was no lymphovascular or perineural invasion, mitotic figures, necrosis or apoptotic cells. Features were consistent with granular cell tumour (GCT). Subsequently, a biopsy of the thigh lesion confirmed GCT. GCT is a rare soft-tissue tumour, thought to originate from Schwann cells. The expression of S100 proteins in GCT supports its neural cell origin. There is a predilection for women in their fourth to sixth decades. Half of cases are found on the head, neck and oral cavity; although any body site can be affected, including internal organs. GCTs are usually solitary, but multiple lesions have been described in up to 25% of cases. Multiple GCTs in paediatric populations have been reported to be associated with genetic syndromes including Leopard syndrome, Noonan syndrome and neurofibromatosis type 1. Most cases of GCTs are benign; however, 1–2% can be malignant. The latter may be suspected when specific clinical and histological features are present. High-risk clinical features include lesions more than 4 cm in size, rapid growth, ulceration, necrosis and lymph node involvement. High-risk histological features are high mitotic activity, necrosis, high nuclear-to-cytoplasmic ratio, spindle cells, vesicular or large nuclei, and pleomorphism. The recommended treatment for benign and malignant GCTs is complete surgical excision to prevent recurrence. Our patient was reviewed by genetics, who felt that further genetic testing was not indicated given her normal physical examination. There was no sign of disease recurrence at 1 year. She remained under our regular follow-up. In conclusion, we report an unusual case of multiple cutaneous GCTs arising in the extremities in a paediatric patient. Although the presentation is rare, our case highlights that GCTs should be considered in the differential diagnosis of cutaneous tumours in the paediatric population.
PA26 Severe pyoderma gangrenosum in a child and the response to adalimumab
Authors: Zwain Ahmed , Crawford Alexander , Belgi Avinash , Morgan Caroline , August Suzannah 
Published: June 2024

Abstract
Pyoderma gangrenosum (PG) is an uncommon ulcerating skin condition that can be associated with underlying problems such as inflammatory bowel disease, rheumatic or haematological diseases and malignancy. The peak incidence is between age 20 and 50 years, and it is rare in children (Graham JA, Hansen KK, Rabinowitz LG, Esterly NB. Pyoderma gangrenosum in infants and children. Pediatr Dermatol 1994; 11: 10–17). A 7-year-old boy presented with multiple erythematous and ulcerated plaques affecting the lower back and abdomen. He had recovered from chickenpox 3 weeks previously. He had been admitted under the paediatric team but had not responded to intravenous antibiotics. A skin biopsy was carried out under general anaesthesia, which showed dense dermal neutrophilic inflammation on histology. Investigations showed no evidence of inflammatory bowel disease, atypical viral infection, granulomatous disease, connective tissue disease or lymphoma. The ulcers became increasingly extensive and painful. A diagnosis of PG was made, and intravenous methylprednisolone was given followed by oral and topical cortico­steroids. There was an initial improvement, but the ulcers flared on reduction of the corticosteroid dose. Ciclosporin was therefore added but there was no improvement after 2 months of therapy, with the ulceration progressing to involve a large proportion of the abdominal skin. Adalimumab was then commenced and there was a marked improvement in the ulceration within 3–4 weeks of starting treatment. It has been shown that adalimumab is effective in children with PG (Kechichian E, Haber R, Mourad N et al. Pediatric pyoderma gangrenosum: a systematic review and update. Int J Dermatol 2017; 56: 486–95). Our patient showed a dramatic response to adalimumab with complete healing of large areas of ulceration after 2 months of therapy, enabling complete cessation of ciclosporin and oral corticosteroids. PG is a rare condition in childhood but can lead to devastating cutaneous ulceration. Adalimumab is a well-tolerated and effective treatment in this patient group.
BI07 (P063) Understanding the landscape of Kaposi sarcoma hospitalizations in the USA: demographic associations stratified by AIDS/HIV status
Authors: Mendoza Roy , Espinoza Norma , Saoub Jessica , Kieron Leslie , Ge Shaokui 
Published: June 2024

Abstract
Kaposi sarcoma (KS), a low-grade vascular tumour associated with human herpesvirus 8 (KSHV/HHV-8), has demonstrated a notable increase among Black men despite an overall decreasing trend in the USA. Given the lack of information regarding the national rates of KS in the USA, this study aims to characterize the national inpatient burden of individuals affected by KS, exploring demographic disparities on a broader scale. We queried the National Inpatient Sample (NIS), a subset of the Healthcare Cost and Utilization Project database containing US hospital discharge data, from 2016 to 2019, to identify all patients aged ≥ 18 years with a diagnosis of KS. We used the data to estimate the national representation and KS-relevant hospital admissions compared with the national civilian population. Stratifying by HIV/AIDS status (AIDS-related KS vs. non-AIDS-related KS), we examined the demographic associations of inpatients with KS using a Wald χ2-test. We determined the incidence rate ratios of admission for KS-related concerns using a Poisson regression model. All statistical analyses were performed with SAS 9.4 (SAS Institute Inc., Cary, NC, USA). In total, 12 246 patients with KS were estimated from the NIS. Overall, the inpatients with KS were predominantly male (91.1%), Black (41.4%) and in the 18–44-year age group (47.7%). AIDS-related KS constituted the majority of patients with KS (10 065, 82.2%), predominantly affecting Black individuals (45.7%) compared with patients classified as White, Hispanic and other (30.2%, 17.6% and 6.5%, respectively). Male patients were more often affected, at a 16 : 1 male-to-female ratio, the largest recorded thus far. Black patients with AIDS had a significantly higher risk of hospitalization. Non-AIDS-related KS comprised 17.8% of cases, primarily among White (51.4%) and male (77.5%) patients, and those aged 65–84 years (49.3%). Despite the differences by KS type, Black patients and those aged ≥ 85 years with non-AIDS-related KS still faced a heightened risk of hospitalization. This study underscores the distinct demographic patterns of inpatients with KS by HIV/AIDS status. For AIDS-related KS, focused efforts are required for Black individuals – addressing their elevated risk, initiating early therapeutic intervention, and promoting adherence to antiretroviral therapy. In cases of non-AIDS-related KS, attention must be given to the older age groups, especially those with a history of immunosuppression. These findings advocate for specialized dermatological strategies considering demographic nuances, contributing to more effective and targeted care for patients with KS.
BI15 (P048) Immunosuppression and vaccination: a cross-specialty survey on current practices
Authors: Lim Chris Puo Nen , Maliampurakal Akash , Saunders Jayne , Paton Elaine , Drummond Angela 
Published: June 2024

Abstract
According to the 2018 General Medical Services contract in Scotland, the responsibility for vaccination delivery has shifted from general practitioners to secondary care. Dermatologists working in NHS Scotland now have an active role in recommending vaccinations for their patients who are on immunosuppressants. This recommendation is outlined in the Green Book published by the UK Health Security Agency. Currently, dermatology patients on immunosuppressants are required to receive the pneumococcal vaccine before starting treatment and every 5 years thereafter. However, this requirement is not included in the patient group direction produced by Public Health Scotland. As a result, individual prescriptions for the vaccination are needed, which adds to the workload on the services. Our objective was to assess current vaccination practices for patients on systemic medications across multiple specialties (dermatology, rheumatology, gastroenterology) in our local health board. We developed an online questionnaire, which was iteratively refined and distributed between June and August 2023 to clinicians working in dermatology, rheumatology and gastroenterology locally, as well as other dermatology units in Scotland through the Scottish Dermatological Society. The questionnaire aimed to gather information on clinicians’ recommendations regarding influenza, pneumococcal and COVID-19 vaccines for patients taking various immunosuppressants, including methotrexate, ciclosporin, azathioprine, mycophenolate mofetil, fumaric acid esters, apremilast, biologics, and Janus kinase inhibitors. In total, 64 responses were obtained from dermatology (44 of 64, 69%), rheumatology (8 of 64, 13%) and gastroenterology (12 of 64, 19%). Most respondents in dermatology (68%) and rheumatology (88%) were consultants or associate specialists, while 50% of the respondents in gastroenterology were specialist trainees. Almost all respondents across the three specialties recommended vaccinations routinely in their clinical practice. The majority of respondents recommended yearly vaccinations against influenza. There were mixed responses on recommendations regarding pneumococcal vaccinations, with varying frequencies between yearly and 5-year intervals. A significant proportion of respondents were unsure of the latest COVID-19 vaccine recommendations. Free-text comments revealed an interest in the project and the need for standardization of vaccination recommendations. A review of the Green Book highlighted the following recommendations for patients on immunosuppressants: annual influenza vaccine, one-off pneumococcal vaccine unless in high-risk groups (asplenia, splenic dysfunction, and chronic kidney disease) and booster COVID-19 vaccines during seasonal campaigns. In conclusion, we highlight the variations in vaccination practices among these three specialties, and the importance of standardization in vaccination recommendations.
BI25 Crusted scabies in a patient with Down syndrome on risankizumab: a complex interaction of biologics, neurological background and immunodeficiency in psoriasis management
Authors: Bajwa Dalvir Singh , Jassal-Prior Pooja , Reynolds Nick J 
Published: June 2024

Abstract
Scabies, caused by Sarcoptes scabiei, presents a significant global health challenge and is primarily transmitted through skin-to-skin contact. Despite the remarkable advances in the use of biologics to treat psoriasis, a concerning trend of increased susceptibility to infections and infestations has emerged. In this comprehensive case study, we report the complex clinical scenario of a patient with a neurological disorder and psoriasis undergoing biologic treatment who was subsequently diagnosed with crusted scabies predominantly localized to her psoriatic plaques. A 37-year-old female patient with Down syndrome undergoing risankizumab biologic therapy for psoriasis was reviewed in the biologics clinic on a routine appointment. The patient exhibited a notable escalation in her Psoriasis Area and Severity Index (PASI) score, increasing from 11.4 to 30.6, accompanied by crusted plaques resembling the distribution of her psoriasis. Dermoscopic examination revealed distinctive burrows and mites, with microscopic confirmation of three scabies mites. A scabies outbreak in the patient’s care facility prompted a comprehensive treatment regimen involving topical permethrin and oral ivermectin, resulting in nearly complete clearance. However, residual crust necessitated additional topical treatments for resolution. Crusted scabies, prevalent in individuals with immunodeficiencies and neurological disorders such as Down syndrome, is intricately linked to cognitive impairment, altering itch perception and fostering parasite proliferation despite elevated IgE and eosinophils. Notably, crusted scabies demonstrates elevated interleukin (IL)-17 levels, potentially indicating T helper (Th)17 dysregulation. Risankizumab, an IL-23 inhibitor, influences the inflammatory function of CD4+ helper T cells, particularly Th17 cells, which produce cytokines such as IL-17A and IL-17F. The pathogenesis of psoriasis involves a positive feedback loop wherein IL-17 stimulates keratinocytes to produce inflammatory mediators, exacerbating inflammation and keratinocyte proliferation. Our hypothesis suggests that risankizumab-induced downregulation of Th17, combined with the patient’s Down syndrome background, heightened the risk of scabies infestation, leading to crusted scabies development in specific areas. This comprehensive case study highlights the importance of considering crusted scabies in immunosuppressed or neurologically affected patients with refractory psoriasis, providing valuable insights into the intricate interplay between biologics, patient characteristics and cutaneous manifestations.
BI26 HIV-associated sarcoidosis as a manifestation of immune reconstitution inflammatory syndrome
Authors: Diong Sophie , Gnanamoorthy Pranav , O’Rourke Michéal , Byrne Joanne , Bergin Colm , McMenamin Mairin , Wynne Bairbre 
Published: June 2024

Abstract
In up to 30% of patients with HIV treated with highly active antiretroviral therapy (HAART), paradoxical reactions occur following immune system recovery, with improvements in CD4 counts and resultant T-helper responses to inflammation – a process known as immune reconstitution inflammatory syndrome (IRIS). Sarcoidosis, a T-helper-cell-mediated disease, has been associated with this state. We present a case of HIV-associated sarcoidosis as a manifestation of IRIS. A 47-year-old South African female patient with HIV and pulmonary sarcoidosis reported a 3-month history of a new rash. She acknowledged intermittent compliance with HAART 8 months prior. Examination revealed an erythematous papular eruption affecting her face and upper extremities. Interferon-γ release assay was negative. Serum angiotensin-converting enzyme was elevated. Serum soluble interleukin-2 receptor was normal, perhaps due to underlying HIV diagnosis. Initial biopsy highlighted non-necrotizing granulomas in keeping with sarcoidosis. Five months later, following a further period of poor HAART compliance, the eruption was more florid, associated with nasal congestion and xerophthalmia. Examination revealed multiple erythematous indurated nodulopapular lesions affecting her nasal bridge, bilateral cheeks and glabella, with scattered erythematous indurated papules affecting the patient’s trunk, neck and limbs. Similar lesions in the scalp were observed, with associated alopecia. Mucous membrane involvement was present with nasal asymmetry, hard palate plaques and florid erythematous conjunctival nodules. No further multisystem involvement was noted on cardiovascular and endocrinology investigations. Treatment has been challenging due to underlying comorbidities and aggressive mucocutaneous disease. Cutaneous features responded to a combination of weaning oral prednisolone, hydroxychloroquine and methotrexate, healing with scarring. Flares have been exacerbated by cutaneous bacterial superinfection with Streptococcus pyogenes. Ocular disease remains recalcitrant, requiring lesional steroids. It has been four decades since the onset of the HIV/AIDS epidemic. Due to advances in pharmacotherapeutics, HIV has transitioned from a life-threatening infection to a chronic condition, bringing comorbidities into focus. Dermatological conditions are extremely common in HIV infection, and cutaneous IRIS manifestations account for up to 75% of all IRIS events. This IRIS manifestation is unique in its latent presentation, occurring months to years after HAART introduction – hypothesized to correlate with recovery of naive T cells. Clinically and histologically, presentations are similar to those of sarcoidosis occurring in patients with no HIV infection. However, comorbidities compounded with side-effects from immunosuppressants pose significant therapeutic obstacles in managing this challenging condition.
BI33 A case of disseminated herpes zoster in a patient on abrocitinib
Authors: Doolan Brent , Drummond Sarah , Pink Andrew , Smith Catherine , Agius Elaine 
Published: June 2024

Abstract
Janus kinase (JAK) inhibitors are disease-modifying agents with efficacy in treating a spectrum of burdensome dermatological conditions. Abrocitinib, which is a JAK1 selective inhibitor, has recently been approved for the treatment of moderate-to-severe atopic dermatitis in adults and young people. Data from randomized controlled trials and real-world analyses have shown herpes zoster to be one of the most frequent serious infections. We present a rare case of disseminated herpes zoster infection with lumbar plexopathy. A 62-year-old White woman was commenced on 200 mg of daily abrocitinib after failure of multiple systemic agents for treatment of her severe atopic dermatitis. After 12 months of treatment, she awoke with spontaneous left-sided ­lower-limb pain that was worse on ambulation. She was given a presumed diagnosis of sciatica and provided with analgesia. The following day the pain remained and she developed pruritic, ulcerated lesions on her face and chest and within her mouth, with periorbital oedema. She attended her local district hospital and was considered to have had a reaction to the analgesia and given oral corticosteroids. She was then reviewed 1 week later and was confined to a wheelchair as she was unable to bear weight on her left leg secondary to hip pain. Neurological examination revealed marked weakness to ankle plantar and dorsiflexion, and loss of sensation. Skin examination showed haemorrhagic crusted papules on the face, abdomen, back and lower limbs, with some intact vesicles on her thigh. Skin swabs confirmed varicella zoster virus infection and she was diagnosed with disseminated herpes zoster. In light of the neurological deficits, she was referred to the neurological team. A lumbosacral magnetic resonance imaging scan showed oedema, thickening and contrast enhancement of the left L4, L5 and S1 nerve roots, in keeping with lumbar plexopathy. She was commenced on high-dose intravenous antiviral therapy, with ongoing, significant neurological impairment to her left leg. Potential risk factors for treatment-emergent herpes zoster have been noted to include abrocitinib dose, baseline age, past history of herpes zoster and absolute lymphocyte count prior to infection. For patients with atopic dermatitis who have achieved acceptable control of their disease, abrocitinib dose reduction should be considered. Patients should be counselled on these serious infections, have their risk factors assessed and have the shingles vaccination recommended for those at risk, prior to commencing treatment.
BI41 Azacitidine-induced neutrophilic dermatosis
Authors: Oldham Jaimie , Goldsmith Portia 
Published: June 2024

Abstract
We present the case of a 66-year-old lady with a background of myelofibrosis that had progressed from Janus kinase 2-positive essential thrombocythaemia. She had recently been started on azacitidine and ruxolitinib and had received two cycles of this medication. She was admitted under the care of haematology with progressive pus-filled blisters and had been treated with broad-spectrum intravenous antibiotics for presumed cellulitis with significant deterioration. She was delirious and significantly unwell with a raised respiratory rate, reduced oxygen saturations, hypotension, tachycardia and a temperature. On examination she had tender erythematous, necrotic and exudative areas over both lateral upper arms and her abdomen (which was an injection site for treatment with azacitidine). A skin biopsy was performed and advice was given to start 60 mg of prednisolone (a dose of just under 1 mg per kg) as well as adding in cover for fungal infection. Almost immediately she reported feeling better and by the next day her observations had normalized and she was afebrile so the antifungals and antibiotics were stopped. She was discharged on a weaning course of prednisolone (40 mg for 3 days, 30 mg for 3 days and then weaning by 5 mg every 5 days) and was advised to stop azacitidine. Her skin improved and healed quickly and her symptoms did not return on cessation of steroids. Her biopsy showed a pandermal neutrophilic infiltrate and was negative for all bacterial and fungal stains. Neutrophilic dermatoses have been reported both in patients with underlying myelodysplastic syndrome and with use of azacitidine. The correlation with the drug timeline and the fact that the skin has not worsened since, despite the underlying myelodysplastic syndrome worsening, would lend support to this being a reaction to the medication.
BI43 Sounds fishy: a case of Mycobacterium marinum infection secondary to infliximab infusion
Authors: Khan Attam , Jain Dimple 
Published: June 2024

Abstract
Mycobacterium marinum is a waterborne, atypical mycobacterium that is known to cause infection in fish, mice and bats. M. marinum in humans is rare and can manifest as a skin/soft tissue infection following exposure of damaged skin to polluted water from aqueous environments such as swimming pools, wells, rivers and fish tanks. We report the case of a 73-year-old White man with a history of collagenous colitis on treatment with infliximab. He presented with pustules, ulcerated plaques and nodules on his left wrist and left deltoid as well as some nodules on the right forearm in a sporotrichoid distribution following a second infliximab infusion. A skin biopsy revealed prominent dermal abscess formation with extension of acute inflammation into the subcutis with fat necrosis. Small granulomas were also seen at the edges without caseating necrosis. Numerous acid-fast bacilli were present on a Ziehl–Neelsen stain in keeping with mycobacterial infection. He was started empirically on rifampicin, ethambutol and clarithromycin; however, minimal improvement was noted. A subsequent mycobacteria microscopy, culture and sensitivity revealed sensitivity to ciprofloxacin, and fortunately his skin responded well to this. Tumour necrosis factor (TNF)-α inhibitors such as infliximab are widely used treatments in managing chronic inflammatory dermatoses as well as inflammatory bowel disease. These agents increase the risk of granulomatous disease. A diagnosis of M. marinum is challenging and multiple biopsies and tissues cultures may be necessary for identifying the correct diagnosis. Although this case did not have any typical exposures to M. marinum, it highlights the importance of screening and educating patients regarding the risks of certain environmental exposures prior to commencing anti-TNF treatments.