British Journal of Dermatology

doi: 10.1093/bjd/ljad260pmid: 37620020

Shareef, Narin; Price, Josephina; Ferguson, Leila; Abdul-Wahab, Alya

doi: 10.1093/bjd/ljad259.010pmid: N/A

Paradoxical psoriasis is well recognized in adults receiving anti-tumour necrosis factor-(TNF)-α therapy for inflammatory bowel disease (IBD) [Bucalo A, Rega F, Zangrilli A et al. Paradoxical psoriasis induced by anti-TNFα treatment: evaluation of disease-specific clinical genetic markers. Int J Mol Sci 2020; 21: 7873. https://doi.org/10.3390/ijms21217873]. Biologics are increasingly being initiated earlier in the treatment ladder for IBD in the paediatric population, who are also vulnerable to paradoxical psoriasis. This observational study aimed to describe the clinical features and management in this group, as there are limited data on how paradoxical psoriasis differs in children. Eight patients with paradoxical psoriasis were referred between 2019 and 2022: five females and three males; age range 12–19 years (mean 15.4 years). They all had Crohn disease and were receiving infliximab (7/8 patients) or adalimumab (1/8). The onset of symptoms following anti-TNF-α initiation was 3 months to 3 years (mean 12.75 months). Involvement included scalp (7), flexural areas (3), trunk (4) limb (3) and face (1). In adults, palmoplantar and pustular forms were commonly reported whereas our cohort demonstrated eczematous forms requiring antibacterial agents, rarely seen in adults. Management included switching from infliximab to ustekinumab in three patients; and the addition of methotrexate (two). Topical treatments alone were sufficient in three patients. There were florid presentations in our cohort and the scalp was predominantly affected (7/8). Five of eight patients required a switch of their anti-TNF-α therapy or an additional systemic treatment. It is crucial for physicians to be aware of this phenomenon and work collaboratively to recognize this early to optimize patients’ symptoms and quality of life [Bucalo et al.].

Balboa, Pablo Lopez; Bartram, Jack; Martinez, Anna; Petrof, Gabriela

doi: 10.1093/bjd/ljad259.034pmid: N/A

Leukaemia is the most frequent malignancy of childhood, accounting for approximately 30% of all malignancies. Acute leukaemia may present in a variety of extramedullary manifestations, the commonest being leukaemia cutis (LC). LC is the infiltration of the epidermis, dermis or subcutis by neoplastic leukocytes with skin lesions preceding the development of leukaemia in the peripheral blood or bone marrow in just 2–3% of cases. A 6-month-old baby girl was reviewed due to multiple skin lesions which appeared on her left forearm 2 months previously, and spread widely, showing over 50 in the following weeks. They were well-circumscribed, nontender, violaceous nodules. She was clinically well and thriving with no lymphadenopathy or organomegaly. Blood tests and abdominal ultrasound were normal. A skin biopsy of a nodule demonstrated an infiltrate of medium-sized atypical cells involving the dermis and subcutis. Immunostaining showed CD45 expression and the Ki67 proliferation index was high, in keeping with a leukaemic infiltration. Cytogenetics performed on the skin biopsy showed KMT2A rearrangement by FISH. Bone marrow aspirate confirmed the diagnosis of acute myeloid leukaemia with monoblastic morphology, and cytogenetics confirmed the rearrangement as a KMT2A–MLLT10 fusion, which is classified as poor cytogenetic risk. Cerebrospinal fluid analysis was negative. It is vital to get a histological diagnosis (with cytogenetics) in a child presenting with violaceus nodular lesions even if the child is well. Importantly, LC can present with a normal full blood count. A bone marrow aspiration is paramount to confirm the diagnosis. Once leukaemia is diagnosed, cytogenetics plays a key role in stratifying treatment intensity.

Oyedepo, Jadesola; Glover, Mary; O’Neill, Claire; Polubothu, Satyamaanasa; Solman, Lea

doi: 10.1093/bjd/ljad259.004pmid: N/A

Congenital haemangiomas (CH) are rare vascular tumours present at birth. These high-flow vascular lesions can lead to significant morbidity secondary to high-output cardiac failure (HOCF) and Kasabach–Merritt phenomenon (KMP). They are classically round/oval lesions and present anywhere on the body. The majority involute spontaneously in the first 2 years of life. We describe three patients who presented with an unusual segmental pattern CH of the head/scalp: the first with a segmental CH of the left forehead, extending over the left eye with associated ipsilateral heterochromia and anisocoria; the second with a segmental ulcerated CH of the right scalp – catastrophic haemorrhage from the CH sadly resulted in death at age 6 weeks; the third with a left forehead segmental CH. None of the CH in this series developed HOCF or KMP. All lesions were consistent with CH on sonography and histology where available. Patients 1 and 3 exhibited spontaneous involution in the first 2 years of life. Segmental CH have been reported once before in a series of three patients, although involving the arm/trunk rather than the head. Two of the three patients developed KMP/HOCF. The genetic basis in this cohort was identical to that of classic nonsegmental CH, activating somatic mutations in GNAQ/11. The striking segmental pattern in our cohort is likely attributable to the timing of the somatic hit during development. Identification of this pattern in CH gives important insights into vascular tumorigenesis. Further cohort studies are needed to determine if this group has differing clinical outcomes from classical CH.

Sprenger, Cathryn; Fleming, Andrew; Ferguson, Leila; Abdul-Wahab, Alya

doi: 10.1093/bjd/ljad259.037pmid: N/A

A 12-month-old Asian female underwent syndactyly release surgery with advancement flap closure of the first web space of the right hand. The procedure was complicated by enlarging keloids over the surgical site and dactylitis, a rare complication with only a few previously reported cases [Muzaffar AR, Rafols F, Masson J et al. Keloid formation after syndactyly reconstruction: associated conditions, prevalence, and preliminary report of a treatment method. J Hand Surg Am 2004; 29: 201–8. https://doi.org/10.1016/j.jhsa.2003.10.017]. Initial treatment of the keloid scarring with intralesional triamcinolone under general anaesthetic was unsuccessful. Subsequently, we elected to surgically excise the keloids closing with a full-thickness skin graft from the groin, with adjunct oral methotrexate (0.4 mg kg–1 weekly) for 9 months post-surgery. The use of oral methotrexate for this indication is rarely reported: one series included two cases of successful keloid suppression with 4 years of follow-up [Muzaffar et al.] and another series of four patients with successful keloid suppression after surgery for syndactyly with dactylitis [Tolerton SK, Tonkin MA. Keloid formation after syndactyly release in patients with associated macrodactyly: management with methotrexate therapy. J Hand Surg Eur 2011; 36: 490–7]. Low-dose methotrexate exerts anti-inflammatory effects by stimulating adenosine A2 receptors and increasing adenosine release at sites of inflammation, such as surgical sites [Muzaffar et al.]. In our patient, methotrexate was well-tolerated, keloid recurrence and dactylitis were avoided, and range of movement and functioning of the digits was maintained. Keloid formation after syndactyly release poses a complex management challenge and we propose methotrexate should be considered as an adjunct to reduce recurrence risk.

Ananthan, Lokapriya; Hamid, Iman; Goodwin, Richard

doi: 10.1093/bjd/ljad259.040pmid: N/A

Tinea capitis is a highly contagious infection, with peak incidence at 3–9 years; transmission occurs between infected persons or via contaminated surfaces. We examine three different cases, all presented to our department this summer. Each gave a synchronous history of a friend or family member that contracted tinea capitis from the same ‘Turkish barbershop’ visit. We compare similar clinical features and timeframes. Two brothers aged 12 and 9 years, presented with multiple inflamed scaly plaques of alopecia on the occipital scalp. An 8-year-old boy presented with a 3-month history of tinea capitis infection affecting his occipital and temporal scalp. A 9-year-old boy presented with a 2-month history of inflammatory scaling scalp alopecia, spreading to involve his face and neck. All three groups attended different schools, different barbershops and had received a ‘skin fade’ haircut a few weeks prior to developing symptoms. Mycology confirmed Trichophyton tonsurans. ‘Skin fade’ is a type of haircut that has a graded hair length, starting at top of the head and gradually getting shorter towards the neckline. This is produced by using clippers and often cut-throat razors. The technique was introduced in 1930s. Its popularity has varied since and has enjoyed another resurgence recently in South Wales. T. tonsurans is well recognized as an important cause of inflammatory scarring alopecia in South Wales and barbers represent an important source of cross-contamination if hygiene measures are not followed carefully. These consecutive cases suggest that ‘skin fade’ haircuts represent a risk factor for the spread of tinea capitis.

O’Connor, Cathal; McCarthy, Siobhan; McCauliffe, Michael; Bennett, Mary

doi: 10.1093/bjd/ljad259.006pmid: N/A

The assessment of disease activity in paediatric morphoea is challenging. Clinical examination and the Localized Scleroderma Cutaneous Assessment Tool (LoSCAT) are subjective. Thermography is the monitoring tool of choice but has a high rate of false-positive results, with low specificity. Multispectral imaging (measuring depth, volume, haemoglobin and melanin) may provide a noninvasive objective assessment of disease activity. The aim of this study was to compare activity assessment with multispectral imaging vs. clinical assessment in children with morphoea. 3D images of site-matched affected and unaffected skin were obtained using a handheld portable Antera 3D camera (Miravex Ltd). Analysis of depth, volume, haemoglobin and melanin was performed using the Antera Clinical Studies software. Objective and subjective clinical assessment was performed using LoSCAT and Children's Dermatology Life Quality Index (CDLQI). To date, 17 children (age range 7–18 years) have been recruited. Two-thirds (65%) had linear morphoea and 58% were treated with systemic agents (pulsed methylprednisolone, methotrexate, mycophenolate mofetil, abatacept). Average LoSCAT scores were 20.6 (range 5–73), and average CDLQI scores were 3.7 (range 0–14). The average haemoglobin gradient between affected and unaffected skin was 0.3 in children with active disease and 0.08 in those who had persistent disease remission (P < 0.0001). The sensitivity of multispectral imaging in paediatric morphoea is 90% with specificity of 100%, giving a positive predictive value of 100%, and a negative predictive value of 95%. Multispectral imaging of affected skin may aid activity detection in paediatric morphoea and is particularly useful in darkly pigmented skin.

Parmar, Shivani; Gass, Julia; Trayers, Claire; Stefanos, Niki; Durack, Alana

doi: 10.1093/bjd/ljad259.036pmid: N/A

Traumatic anserine folliculosis (TAF) is an infrequently reported condition, presenting with grouped follicular papules, usually on the face of young people, induced by repeated frictional trauma or pressure. A suggestive history may allow clinical differentiation of TAF from other folliculocentric keratotic disorders. We present three children diagnosed with TAF in our department: (i) A 12-year-old boy with asymmetry of the mental protuberance, presented with a 1-year history of scaly, lichenified erythema with monomorphic nonpigmented papules over the more prominent side; histology showed mild hyperkeratosis and some rudimentary hair follicles with keratin-filled follicular openings. (ii) A 7-year-old boy with a 1-year history of a hyperpigmented patch on the chin with multiple skin-coloured papules and a sandpaper feel; dermoscopy showed follicular papules with keratin plug; he had a habit of reading with his chin resting on his knee; the skin changes improved with topical retinoid and postural changes. (iii) A boy with follicular papules on one cheek since the age of 8 years, with history of persistent leaning his hand on his cheek while reading; the papules resolved with a change in posture, but similar changes arose at a new pressure area on his chin. The clinical features of grouped monomorphic skin-coloured follicular papules on an erythematous or hyperpigmented background, and a suggestive history, may allow the diagnosis of TAF. Described histological findings are hyperkeratosis, rudimentary follicles and dilated follicular openings filled with keratotic material. Management includes identifying and correcting postural habits; topical keratolytic agents and retinoids may also be beneficial.

Constantinou, Sophie; Angus, Janet

doi: 10.1093/bjd/ljad259.035pmid: N/A

When I started working in the BRI Dermatology department, I noticed that team morale appeared to have been impacted by the COVID-19 pandemic, and also that the department was not running as efficiently as it could be. To improve departmental efficiency, team-working and staff wellbeing following the COVID-19 pandemic, I surveyed staff to assess their thoughts on departmental efficiency, team-working and a daily team ‘Huddle’. I presented my findings in a departmental meeting, following which a daily team Huddle was initiated. All team members were invited. I created a Huddle proforma which included a checklist troubleshooting issues likely to arise in the day, a moment for a wellbeing ‘check-in’ and a celebration of staff achievement. The Huddle team leader rotated daily. After 6 months, I re-surveyed staff: 92% strongly agreed/agreed that patient care was delivered efficiently (60% pre-Huddle, P = 0.01); 96% of staff strongly agreed/agreed that the team worked well together (71% pre-Huddle, P = 0.03); 62% strongly agreed/agreed that the department was running smoothly/efficiently (33% pre-Huddle, P = 0.08). Staff strongly agreed that the Huddle had improved their wellbeing. Nonclinicians felt more confident to approach the on-call Registrar and Consultant. Respondents observed nonclinical staff were less likely to attend. The Huddle allowed staff to share information/concerns. Rotating the Huddle lead helped break down hierarchical barriers. Celebrating achievements boosted team morale and helped members feel valued. Recommendations include encouraging administrative/reception staff to attend and to set up virtual access for staff who work from home.

Bageta, Maria L; Mee, John; Levine, Samantha; Petrof, Gabriela; Martinez, Anna E

doi: 10.1093/bjd/ljad259.028pmid: N/A

Pemphigus foliaceus (PF) is an autoimmune blistering disease caused by autoantibodies against desmoglein 1, which cause acantholysis and intraepithelial blisters. PF classically presents in adults and, excluding the endemic form of PF (fogo salvagem) found in North Africa, Turkey and South America, it rarely occurs in children. We present a previously fit and well 13-year-old girl who was referred with a 2-year history of multiple small blisters on the legs which gradually spread to the trunk and arms. Concomitantly she had severe abdominal pain and alternated constipation with diarrhoea. A skin biopsy suggested a diagnosis of PF. She was commenced on azathioprine which had to be subsequently discontinued due to severe muscle pain and fatigue. She has had a 15-month-course of oral prednisolone which was difficult to wean due to acute flares. A repeat biopsy showed features of pemphigus, direct immunofluorescence showed intercellular deposition of IgG and C3 in the epidermis and ELISA revealed positive autoantibodies against desmoglein 1 (110 U mL–1), in keeping with PF. She was commenced on erythromycin with small improvement and was therefore switched to azithromycin which was discontinued after 5 days due to severe abdominal pain. No abnormalities were seen on a colonoscopy and upper gastrointestinal endoscopy. Despite treatment with erythromycin and topical tacrolimus she continued experiencing acute flares, particularly over her chest and back. Dapsone 50 mg once daily was prescribed and was increased to 75 mg to fully resolve the skin lesions. She is under follow-up and her disease has now remained quiescent for a year.