Identifying the best predictive diagnostic criteria for psoriasis in children (< 18 years): a UK multicentre case-control diagnostic accuracy study (DIPSOC study).

BACKGROUND: In children, psoriasis can be challenging to diagnose. Difficulties arise from differences in the clinical presentation compared with adults. OBJECTIVES: To test the diagnostic accuracy of previously agreed consensus criteria and to develop a shortlist of the best predictive diagnostic criteria for childhood psoriasis. METHODS: A case-control diagnostic accuracy study in 12 UK dermatology departments (2017-2019) assessed 18 clinical criteria using blinded trained investigators. Children (< 18 years) with dermatologist-diagnosed psoriasis (cases, N = 170) or a different scaly inflammatory rash (controls, N = 160) were recruited. The best predictive criteria were identified using backward logistic regression, and internal validation was conducted using bootstrapping. RESULTS: The sensitivity of the consensus-agreed criteria and consensus scoring algorithm was 84·6%, the specificity was 65·1% and the area under the curve (AUC) was 0·75. The seven diagnostic criteria that performed best were: (i) scale and erythema in the scalp involving the hairline, (ii) scaly erythema inside the external auditory meatus, (iii) persistent well-demarcated erythematous rash anywhere on the body, (iv) persistent erythema in the umbilicus, (v) scaly erythematous plaques on the extensor surfaces of the elbows and/or knees, (vi) well-demarcated erythematous rash in the napkin area involving the crural fold and (vii) family history of psoriasis. The sensitivity of the best predictive model was 76·8%, with specificity 72·7% and AUC 0·84. The c-statistic optimism-adjusted shrinkage factor was 0·012. CONCLUSIONS: This study provides examination- and history-based data on the clinical features of psoriasis in children and proposes seven diagnostic criteria with good discriminatory ability in secondary-care patients. External validation is now needed.

Chronic urticaria in the real-life clinical practice setting in the UK: results from the noninterventional multicentre AWARE study.

BACKGROUND: Chronic urticaria (CU) is a skin condition characterized by repeated occurrence of itchy weals and/or angio-oedema for > 6 weeks. AIM: To provide data demonstrating the real-life burden of CU in the UK. METHODS: This UK subset of the worldwide, prospective, noninterventional AWARE study included patients aged 18-75 years diagnosed with H1-antihistamine (H1-AH)-refractory chronic spontaneous urticaria (CSU) for > 2 months. Baseline characteristics, disease activity, treatments, comorbidities and healthcare resource use were documented. Quality of life (QoL), work productivity and activity impairment were assessed. RESULTS: Baseline analysis included 252 UK patients. Mean age and body mass index were 45.0 years and 29.0 kg/m2 , respectively. Most patients were female (77.8%) and had moderate/severe disease activity (mean Urticaria Activity Score over 7 days was 18.4) and a 'spontaneous' component to their CU (73.4% CSU; 24.6% CSU and chronic inducible urticaria). Common comorbidities included depression/anxiety (24.6%), asthma (23.8%) and allergic rhinitis (12.7%). A previous treatment was recorded for 57.9% of patients. Mean Dermatology Life Quality Index score was 9.5, and patients reported impairments in work productivity and activity. Healthcare resource use was high. Severity of CSU was associated with female sex, obesity, anxiety and diagnosis. Only 28.5% of patients completed all nine study visits, limiting analysis of long-term treatment patterns and disease impact. CONCLUSIONS: Adult H1-AH-refractory patients with CU in the UK reported high rates of healthcare resource use and impairment in QoL, work productivity and activity at baseline. The differing structures of UK healthcare may explain the high study discontinuation rates versus other countries.

Oral propranolol in the treatment of proliferating infantile haemangiomas: British Society for Paediatric Dermatology consensus guidelines.

BACKGROUND: Infantile haemangiomas (IH) are the most common vascular tumours of infancy. Despite their frequency and potential complications, there are currently no unified U.K. guidelines for the treatment of IH with propranolol. There are still uncertainties and diverse opinions regarding indications, pretreatment investigations, its use in PHACES (posterior fossa malformations-haemangiomas-arterial anomalies-cardiac defects-eye abnormalities-sternal cleft and supraumbilical raphe) syndrome and cessation of treatment. OBJECTIVES: To provide unified guidelines for the treatment of IH with propranolol. METHODS: This study used a modified Delphi technique, which involved an international treatment survey, a systematic evidence review of the literature, a face-to-face multidisciplinary panel meeting and anonymous voting. RESULTS: The expert panel achieved consensus on 47 statements in eight categories, including indications and contraindications for starting propranolol, pretreatment investigations, starting and target dose, monitoring of adverse effects, the use of propranolol in PHACES syndrome and how to stop treatment. CONCLUSIONS: These consensus guidelines will help to standardize and simplify the treatment of IH with oral propranolol across the U.K. and assist in clinical decision-making.

Cartilage hair hypoplasia with cutaneous lymphomatoid granulomatosis.

Cartilage-hair hypoplasia (CHH) is an autosomal recessive chondrodysplasia characterized by short-stature, sparse hair and impaired cellular immunity. We describe a young girl who was diagnosed with CHH based on the findings of recurrent infections, short stature with metaphyseal chondrodysplasia, and a confirmed bi-allelic RMRP gene mutation. At 13 years, the patient developed an Epstein-Barr virus (EBV)-driven lymphoproliferative disorder involving the lung, which responded partially to chemotherapy. Simultaneously, she developed multiple indurated plaques involving her face, which had histological findings of granulomatous inflammation and EBV-associated low-grade lymphomatoid granulomatosis. The patient received a matched unrelated peripheral blood stem cell transplant at 15 years of age, and her immunological parameters and skin lesions improved. Lymphomatoid forms of granulomatosis and cutaneous EBV-associated malignancies have not been described previously in CHH. This case highlights the possibility of EBV-associated cutaneous malignancy in CHH.

Propranolol in the treatment of infantile haemangiomas: lessons from the European Propranolol In the Treatment of Complicated Haemangiomas (PITCH) Taskforce survey.

BACKGROUND: Oral propranolol is widely prescribed as first-line treatment for infantile haemangiomas (IHs). Anecdotally, prescribing practice differs widely between centres. OBJECTIVES: The Propranolol In the Treatment of Complicated Haemangiomas (PITCH) Taskforce was founded to establish patterns of use of propranolol in IHs. METHODS: Participating centres entered data on all of their patients who had completed treatment with oral propranolol for IHs, using an online data capture tool. RESULTS: The study cohort comprised 1097 children from 39 centres in eight European countries. 76·1% were female and 92·8% had a focal IH, with the remainder showing a segmental, multifocal or indeterminate pattern. The main indications for treatment were periocular location (29·3%), risk of cosmetic disfigurement (21·1%) and ulceration and bleeding (20·6%). In total 69·2% of patients were titrated up to a maintenance regimen, which consisted of 2 mg kg(-1) per day (85·8%) in the majority of cases. 91·4% of patients had an excellent or good response to treatment. Rebound growth occurred in 14·1% upon stopping, of whom 53·9% were restarted and treatment response was recaptured in 91·6% of cases. While there was no significant difference in the treatment response, comparing a daily maintenance dose of < 2 mg kg(-1) vs. 2 mg kg(-1) vs. > 2 mg kg(-1) , the risk of adverse events was significantly higher: odds ratio (OR) 1 vs. adjusted OR 0·70, 95% confidence interval (CI) 0·33-1·50, P = 0·36 vs. OR 2·38, 95% CI 1·04-5·46, P = 0·04, Ptrend < 0·001. CONCLUSIONS: The PITCH survey summarizes the use of oral propranolol across 39 European centres, in a variety of IH phases, and could be used to inform treatment guidelines and the design of an interventional study.

Phototherapy in the elderly.

BACKGROUND: Elderly patients present with a unique spectrum of dermatoses that pose particular management opportunities and challenges, which will be increasingly encountered in dermatological practice. The skin of elderly patients differs from that of younger patients not only in appearance but also in structure, physiology and response to ultraviolet (UV) radiation. However, little is known about the safety and efficacy of phototherapy in elderly patients and how phototherapy is currently being utilized to treat them. AIM: To investigate the safety, efficacy and utilization of phototherapy in elderly patients. METHODS: In January 2014, we analysed all patients recently referred for, currently receiving or recently having completed a course of phototherapy at a university teaching hospital in England (UK). RESULTS: In total, 249 patients were identified; 37 (15%) were over the age of 65 years (the WHO definition of an elderly or older person). The dermatoses being treated were psoriasis (51%), eczema (11%), nodular prurigo (11%), pruritus (11%), Grover disease (5%) and others (11%). One patient with dementia was deemed not safe to embark on phototherapy, and five patients were yet to start. The remaining 31 elderly patients received 739 individual phototherapy treatments: 88% narrowband (NB)-UVB and 12% systemic, bath and hand/foot psoralen UVA (PUVA). The acute adverse event (AE) rate was 1.89%, all occurring in those receiving NB-UVB. No severe acute AEs occurred. Of those who completed their course of phototherapy, 80% achieved a clear/near clear or moderate response, while just two patients (8%) had minimal response and two (8%) had worsening of the disease during treatment. Of those receiving NB-UVB for psoriasis, 91% achieved a clear or near-clear response. CONCLUSIONS: In this small survey, the first of its kind to focus on elderly patients, phototherapy appears to be well-tolerated, safe and efficacious in the short term. Further thought and investigation should be given to delivering phototherapy to an ageing population.

Malnutrition and a rash: think zinc.

Endemic zinc deficiency is recognised to be a common and serious problem in developing countries. However, it may be seen in routine practice in the UK, and can be easily overlooked. Malnutrition from any cause in conjunction with an undiagnosed cutaneous problem should alert the clinician to the diagnosis. Investigations may be unreliable, and if in doubt, a therapeutic trial of zinc supplementation is indicated. We present three cases of malnourished patients, in whom zinc deficiency was diagnosed after the development of cutaneous features. The malnutrition resulted from alcoholism in two cases and anorexia nervosa in the third. The heterogeneity of underlying causes of zinc deficiency is discussed, along with its effects, treatment and zinc homeostasis.

A retrospective study of the referral patterns to a vulval clinic: highlighting educational needs in this subspecialty.

A retrospective study of the referral patterns to a specialist vulval clinic was performed. A total of 200 clinical records were reviewed of new patients seen between January 2004 and June 2005. A provisional diagnosis was stated in 45.5% of referrals, of which 27.5% had the diagnosis changed at the clinic. The majority of referrals were from general practitioners (GPs) (77%) and smaller proportions from gynaecologists (11%), dermatologists (9%) and others (3%). The most common conditions seen were lichen sclerosus (39%), eczema/lichen simplex (30.5%), lichen planus (11.5%), pain syndromes (10.5%) and others (8.5%). Lichen sclerosus was accurately diagnosed in 54.5% of referrals but for eczema/lichen simplex it was only recognised in 11.5% of referrals. Nine cases of lichen sclerosus had been referred by gynaecologists after histological confirmation, for advice on management. Education of vulval diseases to non-specialists should focus on conditions that they are most likely to encounter and find difficult to manage. This study illustrates an effective method to define what the local teaching requirements are.