bluuebunny/crossref_metadata_2025_split huggingface dataset:
PA01 Crusted nodules in returning travellers
Authors: Fox Natalya , White Jonathan , Jiyad Zainab , Ferguson Leila 
Published: June 2023

Abstract
We present the cases of two brothers, aged 10 and 13 years, who, on returning from a 6-week holiday in Sri Lanka, presented to the emergency department with painful, pruritic crusted nodules. Both children were usually fit and well, and up to date with their childhood immunizations; the older brother had a background of mild eczema. On examination, there were multiple widespread 1–2 cm well-defined, superficial excoriated ulcers with overhanding crusted edges. These were present over the arms, legs, back and feet of the younger brother, and the lower legs and feet of the older brother. There was no history of prodromal illness, and they remained afebrile throughout, with no evidence of lymphadenopathy or systemic illness. Bacterial wound swabs demonstrated Corynebacterium diphtheriae. Real-time polymerase chain reaction confirmed the presence of TOX, followed by the Elek test, which demonstrated the expression of the diphtheria toxin, confirming the diagnosis of cutaneous diphtheria due to a toxigenic strain. Both children were treated empirically with intravenous flucloxacillin and clindamycin as inpatients. Both were clinically well and discharged home on oral clindamycin and co-amoxiclav for a total of 10 further days. Public Health was informed of this notifiable disease, with contact tracing and appropriate family screening performed. Our patients isolated appropriately and made a full recovery, with negative swabs. Historically, most cases of diphtheria have been caused by C. diphtheriae (a Gram-positive nonencapsulated, nonsporulating, nonmotile bacillus) with an incubation period of 2–7 days, although other strains, notably Corynebacterium ulcerans, become increasingly prevalent. Despite diphtheria being classically described as a respiratory illness, Public Health England data found that nearly two-thirds of toxigenic C. diphtheriae infections were cutaneous in presentation. Concurrent respiratory infection and cutaneous infection are rare. Either form can be caused by toxigenic or nontoxigenic strains; the former may result in toxic complications (such as myocarditis, polyneuritis, septic arthritis, osteomyelitis and death); however, these are rare in cutaneous diphtheria, even if TOX-positive. Systems review of our patients did not highlight any of these features in our patients. Our patients exemplify classic features of cutaneous diphtheria and raise an important reminder to consider diphtheria in patients returning from endemic areas, despite vaccination schedules being in place.
PA17 Paediatric-onset mastocytosis: a retrospective review
Authors: Hiranput Siwaporn , Nallathamby Kasthuri , Smith Lucy , Yesudian Paul Devakar 
Published: June 2023

Abstract
Mastocytosis is characterized by an abnormal accumulation of mast cells in different organs. The skin is the most frequently affected site. Most cases of paediatric-onset mastocytosis are benign, localized to the skin and regress spontaneously. We retrospectively reviewed the case notes of paediatric patients with a diagnosis of mastocytosis in dermatology departments within our region between 2021 and 2022. Seventeen patients were identified. The disease was more prevalent in males (male-to-female ratio 1.8s : 1). The median age at onset was &lt; 1 year (range birth–4 years). The lesions were described as solitary (n = 9), macular (n = 6) and maculopapular (n = 2). The torso was involved in 16 patients. Other body sites affected were the face (n = 2), scalp (n = 1), limbs (n = 5) and buttock (n = 2). Most patients were asymptomatic (n = 12). Symptoms, including abdominal pain (n = 3), pruritus (n = 2), musculoskeletal pain (n = 1), diarrhoea (n = 1), flushing (n = 1), headache (n = 1) and dizziness (n = 1), were reported. Darier sign was positive in 11 patients and negative in three (not documented in three). None of the patients had bone marrow examination. One patient underwent a skin biopsy, which confirmed mastocytosis in the skin; the remaining 16 patients were diagnosed with cutaneous mastocytosis based on the characteristic clinical features. The disease was classified into mastocytoma (n = 8) and urticaria pigmentosa (n = 9). Five patients with urticaria pigmentosa were assessed for lymphadenopathy and organomegaly: four had blood investigations, including serum tryptase (n = 4), full blood count (n = 2) and liver function tests (n = 2); one had an abdominal ultrasound. No abnormality was found. Patients with mastocytoma were not examined for lymphadenopathy or organomegaly, or had further investigations for systemic involvement. Eight patients were managed by watchful monitoring. Nine patients received active treatments, including topical steroids (n = 6), antihistamines (n = 6), oral sodium cromoglicate (n = 1), phototherapy (n = 1), epinephrine autoinjector (n = 1) and cosmetic camouflage (n = 1). Partial regression was observed in seven cases at a median follow-up of 3 years (range 1–14), and seven had persistent disease. Six patients remain under long-term follow-up, and eight were discharged with a median follow-up period of 1.5 years (range 1–14). Five patients were followed to adolescence (disease was persistent in three cases and there was partial regression in two cases; none had complete resolution). While serum tryptase levels do not appear to have a prognostic value in the paediatric population, we would recommend checking these in patients whose disease persists beyond adolescence as this may indicate progression to systemic mastocytosis.
PA19 A rare case of Buschke–Ollendorff syndrome
Authors: Sathanantham Kugili , Javed Aysha 
Published: June 2023

Abstract
We present a case of Buschke–Ollendorff syndrome (BOS) in a 6-year-old girl. The BOS is rare genodermatosis distinguished by the presence of short stature and skeletal and connective tissue defects. This patient first attended the dermatology department with a history of small asymptomatic dermal papules and nodules noted overlying the skin on her abdomen since the age of 2 years. They became more numerous and evolved over her right buttock and left posterior calf. Subsequent investigation with ultrasound imaging illustrated that these lesions were elasotomas and, on a separate occasions, also suggested the possibility of a lipoma or collagenoma, and ruled out a sarcoma or malignancy. Skeletal survey of the upper limbs, hands, pelvis, lower limb and both feet highlighted the presence of well-defined sclerotic lesions involving the periarticular regions. This condition was again confirmed by clinical geneticists who advised that future family planning and confirmatory testing would identify the presence of LEMD3, responsible for this autosomal dominant condition. There was no history of genetic disorder within the family, but it was noted that the patients and both parents had a short stature, which is another hallmark feature of this condition. On successive reviews, the patient continued to display gradual enlargement of her elastomas and developed other cutaneous manifestations, including congenital naevi. This case report contributes to the appreciation of the clinical manifestation of this uncommon condition.
PA23 Primary cutaneous Ewing sarcoma in a 14-year-old boy
Authors: O’Sullivan Gavin , Salih Hamisha , Davidson Anne , Wynne Catherine , Taylor Mark , George Susannah 
Published: June 2023

Abstract
A 14-year-old boy presented with a 7-month history of a nodule on the right shoulder. It was initially pink and pea-sized but developed black areas as it grew. It was nontender but bled occasionally. He was otherwise well and took no regular medications. He was originally from the Philippines, having moved to the UK 2 years previously. His mother had a history of breast cancer. This was suspected to be a pyogenic granuloma and treated with a shave biopsy. Histology showed largely patternless sheets of small, blue, round cells, with diffuse and strong membranous reactivity for CD99 on immunohistochemistry. Further molecular analysis revealed EWSR1–FLI1 fusion, confirming Ewing sarcoma. Staging imaging showed no metastatic disease. Following further surgery, he was treated as per the Euro-Ewing protocol with vincristine (V), doxorubicin (D), cyclophosphamide (C), ifosfamide (I), etoposide (E; nine induction cycles: alternate VDC/IE followed by five consolidation cycles IE/VC). Ewing sarcoma most commonly arises from bone and is the second most common bone malignancy in children with an incidence of 2.93 per million. Primary cutaneous Ewing sarcoma is extremely rare, with most of the literature consisting of case reports. A 2015 retrospective review of 56 cases of cutaneous/subcutaneous Ewing sarcoma from the UK and France found that most cases were on the extremities (48%) or trunk (39%). Primary cutaneous Ewing sarcoma tends to occur in late-adolescent or young-adult females, whereas bone disease affects younger patients with a male predominance. Current treatment is similar to treatment for Ewing arising from bone. However, primary cutaneous Ewing sarcoma has a more favourable prognosis, with a 5-year overall survival rate of 93.8%. In summary, cutaneous Ewing sarcoma is a very rare malignancy, particularly in the paediatric population. It is important to consider this in the differential diagnosis of erythematous nodular lesions in children, in order to minimize delays in treatment.
PA25 Persistent and widespread infantile transient smooth muscle contraction of the skin: an atypical presentation of a rare condition.
Authors: Keeling Elizabeth , Devereux Clare , Browne Fiona 
Published: June 2023

Abstract
Infantile transient smooth muscle contraction of the skin is a rare entity. Eleven cases have been described in the literature to date. Herein, we describe an unusual presentation, persistent and widespread in nature. A 2-year-old girl presented with asymptomatic bilateral transient textural change of the skin overlying the thighs. Textural changes were noted from birth, occurring a number of times each day. Episodes resolved spontaneously, sometimes within minutes, on occasion lasting up to 1 h. The child remained systemically well during episodes. Textural change progressed to involve the skin overlying the abdomen. Friction and temperature change did not provoke episodes. No other triggers were elicited. Background history included metatarsus adductus, which was under routine follow-up with orthopaedics. Full skin examination between episodes revealed no cutaneous abnormality. The areas affected were normal to palpate. Photographs and videos of episodes revealed marked textural change of the skin overlying both thighs and abdomen, with a striking stippled appearance. Laboratory investigations, including tryptase, lactate dehydrogenase, immunoglobulins and complement, were unremarkable. A case series first described nine cases of infantile transient smooth muscle contraction in 2013 (Torrelo A, Moreno S, Castro C et al. Infantile transient smooth muscle contraction of the skin. J Am Acad Dermatol 2013; 69:498–500). Textural skin changes occurred most frequently on the lower limbs. In eight of these cases, episodes resolved within 1 min. Episodes occurred both spontaneously and secondary to light friction from clothing, cool air and bathing. The majority of cases were noted from birth or in the first days of life. Skin biopsies taken from three of these patients revealed no abnormality. Episodes resolved by 2 years of age in all cases. It has been suggested that this transient phenomenon may be secondary to autonomic nervous system immaturity, primitive reflex phenomena or transient hypersensitivity of the smooth muscle. A more recent case has since been described in the literature (Theodosiou G, Belfrage E, Berggård K, Cristiansen J. Infantile transient smooth muscle contraction of the skin: a case report and literature review. Eur J Dermatol 2021; 31:260–1). We describe a persistent case of infantile transient smooth muscle contraction of the skin, in a child aged &gt; 2 years, with prolonged episodes, progressing from lower limbs to truncal involvement. Recognition of this rare condition is important, to allow appropriate reassurance regarding its benign and self-limiting nature.
PA28 Case series of concomitant use of propranolol and prednisolone in the treatment of ulcerated infantile haemangiomas and an updated literature review
Authors: Kuan Amanda , Koh Mark 
Published: June 2023

Abstract
Infantile haemangiomas (IHs) are the most common benign tumours of infancy. Ten per cent of patients suffer complications, including ulceration, bleeding and functional impairment, and systemic treatment is required to reduce permanent disfigurement. Prednisolone was once the most commonly used and most efficacious treatment modality for complicated IHs, but propranolol has since overtaken its role as the first-line therapy in the last decade. In our practice, we have seen that propranolol monotherapy may not be sufficient in cases of IH with complex ulceration, and oral corticosteroids may serve as an effective adjunct in managing this group of patients. We present a case series of all patients diagnosed with ulcerated IH at KK Women’s and Children’s Hospital over the last 10 years. Fifteen patients were found to have ulcerated IHs. Eight patients were treated with both oral propranolol and oral prednisolone, and four patients were treated with oral propranolol alone. Patients were initiated on propranolol 1–2 mg kg−1 daily, and this dosage was titrated over an average of 9.6 months. Patients prescribed prednisolone had a tapering dosage starting from 0.5–2 mg kg−1 daily for 8–36 days. Average time to complete healing of ulcerated IHs in patients treated with both prednisolone and propranolol was 27.1 days, while patients treated with propranolol alone took 34 days. One patient had recurrence of ulceration after 2 months and had a second, 9-day course of prednisolone. None of the patients had complications from propranolol or prednisolone. Ulceration—the most common complication of IH—tends to heal poorly and is associated with pain, bleeding, infection and scarring. Early and efficacious treatment is needed to prevent functional impairment and permanent disfigurement. Several cases of ulcerated IH have been observed to be recalcitrant to propranolol monotherapy. In these patients, prednisolone used as an adjunct to propranolol has resulted in accelerated healing of these ulcerations. There are no guidelines on the dose and duration of prednisolone use, although a tapering dose starting at 1 mg kg−1 daily is usually given until complete healing of ulceration.
PA34 A case of disseminated herpes zoster in an immunocompetent child
Authors: Jacob Mareen Ann , Kelly Susan 
Published: June 2023

Abstract
Herpes zoster (HZ) caused by the reactivation of varicella-zoster virus (VZV) affects mainly the adult population. The incidence is low in children (0.45 cases per 1000 individuals annually), especially in the immunocompetent. It is said to be disseminated when there are &gt; 20 lesions outside the primary and adjacent affected dermatome. We present this case to highlight a rare complication of cutaneous dissemination in a previously healthy child with HZ, following in utero exposure to varicella at 28 weeks’ gestation. A 15-year-old boy presented with painful blisters in the T2 dermatome of 1 week’s duration, which later spread to trunk, extremities and face. This was associated with fever and neuralgia. There was no previous history of varicella and no recent contact history. He had no significant past medical history and was not on any medications. Antenatal history revealed that his mother had varicella at 28 weeks’ gestation and was treated with aciclovir. He was febrile on admission (39°C). There were coalesced vesiculopustules and crusted lesions with marked perilesional erythema in the T2 dermatome. In addition, disseminated vesicular lesions were seen on the trunk, extremities and face. Remaining systemic examination was unremarkable. C-reactive protein was elevated. Complete blood count and renal function tests were normal. Liver function tests, namely alanine transaminase and γ-­glutamyl transferase were elevated. Blood culture showed no growth. Varicella-zoster IgG was positive. Viral swab (polymerase chain reaction) from the skin lesions was positive for varicella-zoster DNA. Infectious serology for HIV, hepatitis B and hepatitis C was negative. Antinuclear antibody and antineutrophil cytoplasmic antibodies were negative. A clinical diagnosis of HZ with cutaneous dissemination was made, and he was treated with intravenous aciclovir. Intravenous antibiotics were added for suspected secondary bacterial infection. Disseminated zoster is less common in children and mainly occurs in patients with underlying immunodeficiency like HIV, immunosuppressive drug use or malignancy. In immunocompetent children, this can happen when primary infection (varicella) has occurred in utero or the first year of life due to the low response in specific varicella-zoster virus immunity. Complications like cutaneous dissemination due to viraemia are rare in healthy children, but can affect 2–10% of immunocompromised patients. It occurs 3–4 days after the onset of dermatomal lesions. It is important to look for other complications, especially in the immunocompromised, as viraemia can affect the brain, kidneys, lung and liver. Sequelae like postherpetic neuralgia is rare, but it is important to follow-up these children to look for its development.
PS02 Illicit drug use in psoriasis and psoriatic arthritis and its relationship with suicidality in a tertiary population
Authors: Lada Georgia , Chinoy Hector , Talbot Peter S , Warren Richard B , Kleyn C Elise 
Published: June 2023

Abstract
Limited evidence suggests an increased lifetime prevalence of illicit drug use in psoriasis. To our knowledge, there are no data for psoriatic arthritis (PsA) in this regard. Given the pronounced psychiatric morbidity in psoriasis and PsA, including evidence of increased suicidality outcomes, we aimed to investigate illicit drug use prevalence and its relationship with disease characteristics and psychopathology in psoriasis. We surveyed 216 adults attending a specialist psoriasis service, 83 of whom had comorbid PsA. Participants completed paper questionnaires about the type, amount and frequency of lifetime and current illicit drug use (including cannabis, opioids, cocaine/stimulants, hallucinogens and volatile solvents). Participants also completed validated psychometric tools; suicidality was measured with the Sheehan Suicidality Tracking Scale. The prevalence of lifetime (21.7%) and current (6.0%) illicit drug use in PsA did not differ from the respective rates in psoriasis only (27.1% and 8.3%; P &gt; 0.1), but patients with PsA showed lower regular use rates (2.7% in PsA vs. 12.9% in psoriasis only; P = 0.03). Dermatology Life Quality Index and Psoriasis Area and Severity Index scores were numerically higher among illicit drug users than nonusers, but the differences were not statistically significant. In our sample, lifetime illicit drug use was associated with increased lifetime suicidal planning and behaviour but not suicidal ideation. The association of drug use with suicidal planning remained after adjusting for age, sex, psychiatric comorbidity and alcohol consumption scores (odds ratio 2.44, 95% confidence interval 1.04–5.76). Patients reporting current drug use were more likely to have been previously diagnosed with anxiety but were not disproportionately affected by depression and alexithymia. The prevalence of lifetime illicit drug use in our study is consistent with findings from a recent North American psoriasis cohort. Although it is not clear why regular drug use is less prevalent in patients with PsA, increased medical attention may play a role. The higher prevalence of suicidal planning/behaviours, but not ideation, among patients with psoriasis with illicit drug use is an important and concerning finding. This aligns with some previous research suggesting that illicit drug use is associated with suicidality progression toward action, possibly due to impulse-control deficits. Early detection and management of drug use may help prevent suicidal behaviours in psoriasis. Study limitations include the cross-sectional design and social desirability bias-related under-reporting. Prospective cohorts are needed to further investigate the role of illicit drug use in patients’ psychiatric morbidity and quality of life, and address its sequelae within dermatology settings.
PS05 A qualitative assessment of the impact of hidradenitis suppurativa on sexual function
Authors: Pearce Julian , Ferguson Leila , Maybury Catriona , Loibner Natalie , Lamb Ruth 
Published: June 2023

Abstract
Hidradenitis suppurativa (HS) is associated with significant physical and psychosocial morbidity. There are limited data in the literature on the impact of the condition on patients’ psychological, physical and emotional sexual functioning and wellbeing. A patient survey was collaboratively designed by dermatologists and a clinical psychologist to assess sexual function in HS. This was adapted from existing published scales assessing components of sexual functioning, as there is no validated tool for use in HS. Nineteen patients (10 men and nine women) with HS were surveyed. They had a mean age of 41 years and a mean Hurley stage of 2.2. Twenty-six per cent (n = 5) were current smokers. The mean body mass index of participants was 28 kg m−2. Seventy-nine per cent (n = 15) were in a current sexual relationship. Seventy-four per cent (n = 14) felt that HS affected their sexual relationships negatively and 50% (n = 8/16) documented that HS had prevented them starting a new sexual relationship. Eighty-four per cent (n = 16) reported that their HS caused them to avoid sexual intimacy. Sixty-three per cent (n = 12) suffered a degree of dyspareunia and 63% (n = 12) described that odour from HS lesions impacted negatively on sexual activity. Confidence with regard to emotional closeness with partners during sex was variable, with 31% (n = 5) reporting low confidence; 58% (n = 11) had low confidence when engaging in sexual activity. Fifty per cent (n = 9/18) felt sad when considering the impact of HS on their sexual life and 88% (n = 14/16) felt ashamed and stressed when considering being naked or intimate. Ninety-four per cent (n = 17/18) felt a degree of sexual unattractiveness due to HS, with 44% (n = 8/18) rating it as ‘extremely’ or ‘very much’. Eighty-nine per cent (n = 16/18) reported that HS had negatively impacted on the sexual quality of their lives, with 33% (n = 6) rating it as ‘extremely’ or ‘very much’. Sexual function in HS is an important, often overlooked component of quality of life. Sexual dysfunction is of particular concern in HS due to the location and nature of lesions, which are often close to or affect erogenous zones (Varney P, Guo W, Brown M et al. A systematic review and meta-analysis of sexual dysfunction in patients with hidradenitis suppurativa. Int J Dermatol 2022; 85:AB23). This study demonstrates that HS has a significant impact on patients’ psychosexual, physical and emotional sexual functioning and wellbeing. The Permission, Limited Information, Specific Suggestions, Intensive Therapy model is a useful, structured framework for clinicians to incorporate the discussion and management of sexual dysfunction into consultations, and clinicians should work with local psychological, sexual and biomedical services to address any identified morbidity.