bluuebunny/crossref_metadata_2025_split huggingface dataset:
BH15 The burden of alopecia areata: examining baseline health-related quality of life and clinical assessment tools
Authors: Boyle Aoife , Wall Dmitri , McDonald Ian 
Published: June 2024

Abstract
Alopecia areata (AA) is an autoimmune-mediated, nonscarring hair loss that affects 2% of the population. Patients with AA experience significant impairment in health-related quality of life (HRQoL), particularly mental health (Senna M, Ko J, Glashofer M et al. Predictors of QOL in patients with alopecia areata. J Invest Dermatol 2022; 142: 2646–50). The Severity of Alopecia Tool (SALT) is a physician-derived, semiobjective measure, frequently used in clinical practice, that calculates the extent of scalp hair loss. Nonscalp hair or quality of life is not incorporated and may explain why SALT can underestimate the overall impact of AA (Senna et al.; Wall D, Rees H, Bokhari L et al. Signposts to the promised land in alopecia areata. J Invest Dermatol 2023; 143: 9–10). The Dermatology Life Quality Index (DLQI) and Hospital Anxiety and Depression Scale (HADS) are common HRQoL assessments. Emerging disease-specific tools, which examine both hair loss extent and quality-of-life impact, include the Alopecia Areata Patient Priority Outcomes (AAPPO) and the Alopecia Areata Symptom Impact Scale (AASIS). This study aimed to determine the baseline HRQoL burden among adult patients with AA in a newly developed hair research clinic. A secondary aim was to examine how HRQoL assessments compared with SALT vs. newer compound measures, AAPPO and AASIS. A retrospective chart review identified 20 patients with complete data. The mean SALT score was 61.2. The mean DLQI score was 11.05, indicating a very large impact on QoL as measured by DLQI bands. Overall, 15% of patients experienced no effect, 25% a small effect, 10% a moderate effect, 30% a very large effect and 20% an extremely large effect. The mean HADS-D (depression) score was 8, indicating a borderline score. In total, 47% had normal scores, 21% had borderline scores and 32% had abnormal scores. Similarly, the mean HADS-A (anxiety) score was borderline at 9.9; 26% had normal scores, 21% borderline scores and 53% abnormal scores. The mean total AAPPO score was 16.8 out of 44. The highest mean subscore was the emotional symptoms subscore of 9.9 out of 16, followed by a hair loss subscore of 9 out of 16 and an activity limitations subscore of 3.9 out of 12. The mean total AASIS score was 118 out of 170. The highest mean subscore was the symptoms subscore of 36.9 out of 70, followed by the feelings subscore of 17.5 out of 40 and daily functioning subscore of 24.1 out of 60. SALT had a nonsignificant positive association with HRQoL. AAPPO and AASIS had significant positive associations with HRQoL. In conclusion, our study has demonstrated a significant impact on HRQoL among patients with AA, irrespectively of the degree of hair loss. Our study has also shown that the emerging disease-specific tools, AAPPO and AASIS, are superior in determining the overall impact of AA compared with SALT.
British Photodermatology GroupOral presentationsPD01 Accelerated oral psoralen–ultraviolet A therapy in the management of polymorphic skin eruption: real-world data from a specialist centre
Authors: Campbell Fiona , Montgomery Rachel , Goulden Victoria 
Published: June 2024

Abstract
Polymorphic light eruption (PLE) is a common, debilitating disease. First-line management includes photoprotective measures, which are restrictive, and ineffective for severe cases. Many patients notice increased tolerance (hardening) to sunlight as summer progresses. This phenomenon can be induced by exposure to artificial sources of ultraviolet. Many studies have demonstrated the efficacy of both narrowband ultraviolet B radiation (NB-UVB) and psoralen–ultraviolet A therapy (PUVA) in reducing PLE flares (Ling TC, Clayton TH, Crawley J et al. British Association of Dermatologists and British Photodermatology Group guidelines for the safe and effective use of psoralen–ultraviolet A therapy 2015. Br J Dermatol 2016; 174: 24–55). NB-UVB is generally used first line due to simplicity of treatment and long-term safety. PUVA is second-line phototherapy and, as PUVA is associated with a dose-dependent risk of skin cancer, shorter treatment courses are desirable. A study by Palmer and Friedmann showed that six PUVA treatments provided as much protection as 12 in patients with PLE (Palmer RA, Friedmann PS. A comparison of six and 12 PUVA treatments in the prophylaxis of polymorphic light eruption. Clin Exp Dermatol 2004; 29: 141–3). We have retrospectively reviewed our patients with PLE who did not gain benefit with NB-UVB hardening and were treated with ‘accelerated’ PUVA. In the early spring, patients were treated on exposed sites with four UVA exposures after taking oral psoralen. The initial irradiation dose was 70% of the minimal phototoxic dose. Doses were then escalated by 1 J cm−2 each visit for a total of four exposures only. To prevent a significant flare of PLE during treatment, patients were prescribed oral prednisolone 20 mg once daily, commencing on the day of the first exposure and continued for the 2-week course of treatment. Fourteen patients (12 female, two male) with severe PLE and poor response to narrowband NB-UVB were treated. The age range was 25–67 years. Thirteen patients experienced a significant reduction in severity of their PLE flares. Only one patient (male, 39 years) found the treatment unsuccessful. Half of the patients went on to have further courses of accelerated PUVA. No patient experienced significant adverse effects. Our experience has demonstrated that only four exposures of oral PUVA can induce significant skin hardening, preventing PLE flares. This has the significant advantage of a short course of treatment and reduction in cumulative exposure for patients who frequently require repeated courses of treatment. Oral steroid cover is necessary to suppress reactions during treatment but was well tolerated in all cases.
PD11 Simultaneous development of photosensitivity in a father and daughter: de novo or exposure induced?
Authors: Ross Hannah , Parkin Donna , Ayer Jean 
Published: June 2024

Abstract
Chronic actinic dermatitis and photoaggravated eczema are rare photosensitivity disorders that, while considered to be immunologically mediated, usually develop de novo. A 4-year-old White British girl and her 33-year-old White British father presented to a tertiary photobiology clinic with similar photodistributed eruptions that occurred acutely following exposure to an airborne glyphosate organophosphate-containing weedkiller. The eruptions persisted thereafter on exposure to sunlight. Approximately 15 min of sun exposure is sufficient to provoke a red, scaly and blistering eruption around 6–12 h later. The eruption affects only photoexposed sites, including the posterior neck, elbows, dorsal hands and cheeks, and episodes persist for several days. The father underwent monochromated light testing to narrowband widths of ultraviolet (UV)B, UVA and visible radiation and demonstrated exquisite photosensitivity. Provocation testing to broadband UVA and a solar simulator were abandoned after the first exposure due to a significant erythemal and oedematous response. The daughter underwent modified monochromator testing due to her age, but similarly demonstrated significant photosensitivity with reduced thresholds from 300 to 370 nm. Reduced-dose provocation with broadband UVA and a solar simulator resulted in a mild, blotchy, papular erythema. Several months later, both father and daughter reported worsening of their photosensitivity. Repeat photoinvestigation carried out 15 months following the initial exposure to the glyphosate, suspected to be the precipitant, showed an objective deterioration in photosensitivity in both patients, resulting in formal diagnoses of chronic actinic dermatitis. Reports of phototoxicity after contact with glyphosate formulations are exceedingly rare. However, there is a report of the preservative coformulant benzothiazolinone (BIT), found in glyphosate formulations, that was confirmed to be phototoxic through exposure of the skin for 24 h followed by UVA exposure (Hindson C, Diffey B. Phototoxicity of glyphosate in a weedkiller. Contact Dermatitis 1984; 10: 51–2.) The reported papulovesicular eruption that followed BIT and UVA exposure is in keeping with the eruption seen in our patients. However, photopatch testing to BIT was negative in both patients. There are no other reports in the literature of photosensitization following organophosphate exposure with prolonged and worsening effects 15 months after exposure. Therefore could it be possible that two de novo cases have occurred in one family at exactly the same time, or could the initial phototoxic reaction have triggered an immune response that has subsequently caused the development of chronic actinic dermatitis?
Poster presentationsDS12 A rare ophthalmological consequence of cutaneous malignant melanoma
Authors: Mahendiran Thushika , Orpin Stephen , Patil Ajay 
Published: June 2024

Abstract
A 66-year-old man was referred urgently to dermatology from ophthalmology with a short history of developing multiple pigmented lesions on the trunk and in the axillae, the concern being that he had developed a malignant melanoma. He had been under their care for almost a year with a history of a subacute decline in vision in both eyes leading to ‘tunnel vision’. His medical history was complex and included acute myeloid leukaemia secondary to myelodysplastic syndrome treated with fludarabine, melphalan and alemtuzumab (FMA) reduced-intensity sibling allograft. Following bone marrow transplant and chemotherapy, he had post-transplant complications including cytomegalovirus reactivation, cytopenia secondary to splenomegaly, and possible graft-versus-host disease (bronchiolitis obliterans and brachial plexitis). He had presented to ophthalmology with his vision at 6/12 and 6/9 in the right and left eye, respectively. On examination, there were poorly reactive pupils with normal pupil reflexes and normal colour vision in both eyes. The visual field to confrontation was significantly constricted. Optical coherence tomography scans demonstrated outer retinal loss in the parafoveal region with macular oedema in both eyes. An autoimmune retinopathy was suspected due to these findings. Electrodiagnostic tests were consistent with this. A secondary survey was undertaken, and a positron emission tomography scan showed no evidence of active acute myeloid leukaemia or other malignancy. Neuroimaging demonstrated generalized cortical atrophy with no acute intracranial abnormalities. He was treated for a presumed nonparaneoplastic autoimmune retinopathy. He was commenced on dexamethasone intravitreal implants and mycophenolate mofetil 1 g twice a day. At review, as well as him having multiple seborrhoeic keratoses, there was an atypical melanocytic lesion on the back, which was excised urgently. This proved to be a malignant melanoma in situ with extensive regression; subsequent re-excision revealed no residual tumour. Therefore, he was diagnosed with presumed melanoma-associated retinopathy (MAR) and commenced on monthly intravenous immunoglobulin 1.5g kg−1. On subsequent visits he subjectively felt his vision had remained stable with no further deterioration. MAR is a rare paraneoplastic ocular condition. This is an autoimmune retinopathy, where a molecular mimicry occurs between the neuroectodermally derived melanocytes and the retinal proteins. MAR can precede the diagnosis of malignant melanoma, with other initial investigations being negative. This case highlights the importance of referring patients for a thorough dermatology examination for an individual presenting with an autoimmune retinopathy. With early detection and treatment, irreversible immune-related retinal damage and associated visual loss can be mitigated.
DS17 Two cases of traumatic pseudoaneurysm of the superficial temporal artery
Authors: Phillips Chris , Smith Kirsty , Potter Matthew , Turner Richard 
Published: June 2024

Abstract
We present two cases of traumatic pseudoaneurysm of the superficial temporal artery (STA). An 87-year-old woman was referred to the dermatology 2-week-wait clinic with a 4-cm painless nodule on the left forehead. Three weeks prior she sustained a fall at home, and computed tomography revealed a subgaleal haematoma over the left forehead. She was on warfarin for deep vein thromboses (international normalized ratio 4.2). The nodule was compressible and had a purple hue. A diagnosis of haematoma was made and incision and drainage was booked. On the day of surgery her procedure was cancelled as clinical re-examination revealed the mass was pulsatile and overlying the STA. The diagnosis was revised to STA pseudoaneurysm. She was referred to plastic surgery and underwent ligation of the artery and excision of the pseudoaneurysm. A 51-year-old man presented to dermatology with a 1-month history of a 1-cm painless, pulsatile subcutaneous mass on the right forehead continuous with the STA. Further questioning revealed a cycling accident 3 months prior with polytrauma. STA pseudoaneurysm was diagnosed clinically. He underwent ligation of the artery and resection of the pseudoaneurysm under local anaesthetic in dermatology theatres successfully, with no complications or recurrence. STA pseudoaneurysm is a rare presentation of an enlarging nodule on the forehead or temple, which typically occurs following blunt head trauma. However, cases have been reported following Botox injections, hair transplants and other procedures. The interval between the causative trauma and the onset of the lump can vary (reported range 2–6 weeks to 10 years), and therefore it may not be obvious or volunteered by the patient. Although STA pseudoaneurysm is usually asymptomatic, ear pain, headache and facial nerve palsies have been described. Examination reveals a typically spherical subcutaneous lesion, continuous with the course of the STA. Unless thrombosed, palpation should reveal a characteristic pulse or thrill and a bruit on auscultation. Diagnosis is usually clinical. Doppler ultrasound (demonstrating vessel dilatation and turbulent flow), magnetic resonance imaging, computed tomography (CT), and CT angiography can confirm the diagnosis and are useful to investigate other post-traumatic injuries. Treatment is usually surgical ligation of the artery and resection of the pseudoaneurysm. Embolization is an alternative if surgery cannot easily be performed. In summary, STA pseudoaneurysm is a rare but important clinical diagnosis to consider in a subcutaneous mass on the forehead, before biopsy or aspiration is performed. A history of trauma does not always directly precede its onset despite it being the likely causative factor. Although the clinical history can be suggestive, examination to identify the pulsatile nature is important, so the diagnosis may be missed on reviewing teledermatology images only.
DP12 Comparison of clinical, dermoscopic and histopathological characteristics of salt and pepper pigmentation in systemic sclerosis and vitiligo: a prospective controlled study
Authors: Viswan Parvathy , Behera Biswanath 
Published: June 2024

Abstract
Systemic sclerosis (SS)-associated salt and pepper pigmentation (SAPP) and vitiligo are rather indistinguishable. This study aimed to compare the clinical, dermoscopic and histopathological characteristics of SAPP in SS with vitiligo. Twenty-one patients with SS-associated SAPP, and 43 patients with vitiligo attending the outpatient department were recruited. The clinical, dermoscopic and histopathological characteristics of the depigmented lesions were analysed for a predefined set of parameters by two dermatologists. Sites prone to friction, like the extensor aspect of the arms (P &lt; 0.05), the flexor aspect of the thigh (P &lt; 0.05) and the inguinal folds (P &lt; 0.05), were more commonly involved in SAPP. Depigmentation or hypopigmentation of the palms and soles (P &lt; 0.05) were invariably absent in SS. Clinically, perifollicular depigmentation (P = 0.02), confetti lesions (P ≤ 0.01), trichrome sign (P ≤ 0.01) and leucotrichia (P = 0.02) were seen in vitiligo, while perifollicular hyperpigmentation (P &lt; 0.01) was seen in SAPP. Among dermoscopic features, trichrome sign (P &lt; 0.01), dermoscopic leucotrichia (P = 0.02), perifollicular depigmentation (P = 0.02), lesional and perilesional pigment network (P &lt; 0.01) and satellite white clod (P &lt; 0.01) were characteristically present in vitiligo, whereas perifollicular hyperpigmentation over a background of depigmentation (P &lt; 0.01), white network (P &lt; 0.01), hypo- or depigmented structureless area with angulated border (P &lt; 0.01), alternating hyper- and depigmentation longitudinally (P &lt; 0.01), blue–grey dots (P &lt; 0.01) and telangiectasia (P &lt; 0.01) were more common in SAPP. The histopathological features of depigmented areas of SAPP and vitiligo showed the following significant differences: more epidermal lymphocytes (P &lt; 0.05) and more dermal fibroblasts were seen in vitiligo (P &lt; 0.01), while dermal sclerosis (P &lt; 0.01) and periadnexal infiltrate (P &lt; 0.01) were more common in SS. Histopathological features suggestive of dermal sclerosis such as pandermal sclerosis (P &lt; 0.01), line sign (P &lt; 0.01), square biopsy sign (P &lt; 0.01), cookie cutter sign (P &lt; 0.01), lone arrector pili sign (P &lt; 0.01) and high eccrine gland sign (P &lt; 0.01) were seen exclusively in SS. When in doubt, careful clinical, dermoscopic and histopathological examination of the depigmented lesions in SS and vitiligo will help in diagnosis. This study is limited by the small sample size, and only patients with Fitzpatrick skin types IV and V were studied.
DP20 A case series identifying the clinical and histopathological characteristics of amelanotic melanoma
Authors: Sohail Aroon , Kavaklieva Svetlana 
Published: June 2024

Abstract
Amelanotic melanoma (AM) is a subtype of melanoma where the lesion demonstrates little to no pigmentation. This can lead to delays in referral due to misdiagnosis, with studies showing a higher mortality rate compared with pigmented melanoma. However, there are few reports in the current literature regarding the characteristics associated with AM. In a 10-year period at a single centre in the north-west of England, patients with a histological diagnosis of AM were enrolled and data were collated retrospectively to determine the characteristics of AM lesions. Of the 16 patients identified, 69% were male, and the mean age at diagnosis was 78 years. The most common location for AM was the head (38%), which also demonstrated a higher mitotic rate (10.7 mm2) compared with the average (7.3 mm2). More than half of the lesions (56%) had been present for &gt; 1 year. With a misdiagnosis rate of 88%, the likelihood of delays was evident. Dermoscopic findings were variable, but conspicuous vessels were noted in 63% of lesions. The most common vessel patterns were linear and irregular vessels, each identified in 19% of cases, with 13% of cases showing arborizing vessels. Our case series has demonstrated that AM is typically diagnosed in the older population, with a greater incidence rate noted in men. The age at diagnosis could be secondary to a delay in referral as AM is commonly not identified, with a misdiagnosis rate of 88% in our series. Lesions were most commonly located on the head region, whereas the back and legs were less commonly affected. This is contrary to melanoma, where the legs are most common for women and the back the most common for men. We suspect that a prolonged exposure of the head region to ultraviolet light is the reason for the highest distribution rate noted in the head. Furthermore, AM of the head was noted to have a greater mitotic rate than the average, potentially indicating more aggressive and lethal disease. The gold standard in clinical assessment is dermoscopic examination, with particular emphasis on identifying abnormal vessel features, a finding amplified due to a lack of pigmentation. We have demonstrated that AM can have a significant misdiagnosis rate with the possibility for a more aggressive cancer to form, potentially leading to unfavourable patient outcomes. We have identified key features of suspicious lesions that require assessment, notably abnormal vessels. Practitioners in primary and secondary care should be aware of and vigilant in identifying AM lesions. All suspicious lesions should undergo histopathological analysis to determine their exact nature.
British Society for Paediatric and Adolescent DermatologyOral presentationsPA01 (P094) Oral tofacitinib monotherapy for the management of moderate-to-severe alopecia areata in children and adolescents: a retrospective analysis of 36 cases
Authors: Mehta Hitaishi , Mahajan Rahul , De Dipankar , Handa Sanjeev 
Published: June 2024

Abstract
Alopecia areata (AA) poses a significant challenge in the paediatric population, with limited treatment options. Oral tofacitinib has emerged as a promising modality, with case reports and small series suggesting safe and effective use in children with AA. This retrospective analysis sought to expand the knowledge base around tofacitinib’s role in managing AA in the younger population and to provide valuable insights into challenges encountered when faced with no response to treatment. This retrospective chart review spanned from January 2021 to December 2023 and included 36 patients aged ≤ 16 years with moderate-to-severe AA [Severity of Alopecia Tool (SALT) score &gt; 25], who received ≥ 6 months of oral tofacitinib therapy. The treatment response was defined as ≥ 75% reduction in SALT score (SALT 75) at 6 months. Other outcome measures of interest included times to attain SALT 50, SALT 75, SALT 90 and SALT100, and incidence of adverse events. In total 36 patients were included, with a mean age of 10.1 years (SD 3.8, range 3–16), with 17 male and 19 female patients. AA subtypes included patchy (19%), ophiasis (17%), subtotalis (28%), totalis (8%) and universalis (28%). The mean SALT score at baseline was 73.1 (SD 26.7). The mean duration of oral tofacitinib treatment was 8.69 months (SD 3.17, range 6–16). SALT 50 was achieved by 26 patients, with an equivalent number attaining SALT 75. Additionally, 24 patients reached SALT 90, while 19 patients reached SALT 100. The mean (SD, range) durations in months to achieve SALT 50, SALT 75, SALT 90 and SALT 100 were 2.81 (1.16, 1–6), 4.08 (1.69, 1–8), 5.71 (2.14, 3–10) and 6.74 (2.35, 3–12), respectively. Ten of the 36 patients (28%) were classified as nonresponders. Nonresponders exhibited a higher median total duration of illness, higher baseline SALT score, and higher incidence of atopy and nail involvement compared with responders. Adverse events were noted in nine patients, with two cases necessitating treatment discontinuation due to adverse events. Among those not showing a response, three patients each underwent a transition to combination therapy of oral steroids with methotrexate and topical immunotherapy with diphenylcyclopropenone. Notably, regrowth was observed in two patients from the former group, while none from the latter group experienced similar positive outcomes after the therapeutic strategy shift. Limitations of the study include the retrospective nature and small sample size. This comprehensive analysis enriches the existing literature on the role of tofacitinib in paediatric AA, addressing gaps in approved treatments and providing valuable guidance for clinicians navigating the challenges of nonresponsiveness in this vulnerable population.