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Competing Interests: Conflicts of interest P.S.H. has received speaker fees from Novartis and Group for Research and Assessment of Psoriasis and Psoriatic Arthritis and acted in an advisory capacity for Amgen. The remaining authors declare no conflicts of interest.

The objective of this guideline is to provide up-to-date, evidence-based recommendations for the management of SLE that builds upon the existing treatment guideline for adults living with SLE published in 2017. This will incorporate advances in the assessment, diagnosis, monitoring, non-pharmacological and pharmacological management of SLE. General approaches to management as well as organ-specific treatment, including lupus nephritis and cutaneous lupus, will be covered. This will be the first guideline in SLE using a whole life course approach from childhood through adolescence and adulthood. The guideline will be developed with people with SLE as an important target audience in addition to healthcare professionals. It will include guidance related to emerging approved therapies and account for National Institute for Health and Care Excellence Technology Appraisals, National Health Service England clinical commissioning policies and national guidance relevant to SLE. The guideline will be developed using the methods and rigorous processes outlined in 'Creating Clinical Guidelines: Our Protocol' by the British Society for Rheumatology., (© The Author(s) 2023. Published by Oxford University Press on behalf of the British Society for Rheumatology.)

Background: Real-world data evaluating effectiveness and persistence of systemic therapies for patients with psoriasis are limited. Objectives To determine the effectiveness and persistence of acitretin, ciclosporin, fumaric acid esters (FAEs) and methotrexate in patients with moderate-to-severe psoriasis., Methods: Data from the British Association of Dermatologists Biologics and Immunomodulators Register (BADBIR), a prospective, multicentre pharmacovigilance register of patients with moderate-to-severe psoriasis receiving biologic and/or conventional systemic therapies, were analysed. Eligible patients were ≥ 16 years of age receiving a first course of acitretin, ciclosporin, FAEs or methotrexate between 2007 and 2021 with ≥ 6 months' follow-up. Effectiveness was defined as achieving absolute Psoriasis Area and Severity Index (aPASI) ≤ 2 reported ≥ 4 weeks after treatment start date until date of cessation. To identify baseline clinical variables associated with treatment effectiveness, we used multivariable logistic regression models estimating the adjusted odds ratio (aOR) of achieving aPASI ≤ 2. To describe drug persistence associated with ineffectiveness, occurrence of adverse events or other reasons for discontinuation, survival estimates with 95% confidence intervals (CIs) were obtained using a flexible parametric model. Results were obtained using multiple imputed data., Results: In total, 5430 patients were included in the analysis. Overall, 1023 (19%) patients were receiving acitretin, 1401 (26%) patients were on ciclosporin, 347 (6%) patients were on FAEs, and 2659 (49%) patients were receiving methotrexate at registration. The proportion of patients who achieved aPASI ≤ 2 was lower for those treated with acitretin [n = 118 (21%)] compared with those receiving ciclosporin [n = 233 (34%)], FAEs [n = 43 (29%)] and methotrexate [n = 372 (32%)]. Factors associated with ineffectiveness included prior experience to previous nonbiologic systemic therapies (acitretin) (aOR 0.64, 95% CI 0.42-0.96), male sex (methotrexate) (aOR 0.58, 95% CI 0.46-0.74), comorbidities (aOR 0.70, 95% CI 0.51-0.97) and alcohol consumption (≤ 14 units per week) (ciclosporin) (aOR 0.70, 95% CI 0.50-0.98). Persistence associated with all reasons for discontinuation showed better survival for methotrexate compared with acitretin, ciclosporin and FAEs cohorts at 12 months [survival estimate 46.1 (95% CI 44.0-48.3), 31.9 (95% CI 29.4-34.7), 30.0 (95% CI 27.5-32.4) and 35.0 (95% CI 29.9-40.9), respectively]., Conclusions: The real-world effectiveness and persistence of acitretin, ciclosporin, FAEs and methotrexate were generally low. Previous nonbiologic systemic therapies, male sex, comorbidities and alcohol consumption were risk factors associated with treatment ineffectiveness., Competing Interests: Conflicts of interest P.J.H. has received educational grants, consultancy fees and research funding from Janssen, AbbVie, Eli Lilly and LEO Pharma. P.M.L. has received honoraria and/or grants as an investigator, speaker, and/or acted as an advisory board member for AbbVie, Almirall, Amgen, Celgene, Janssen Cilag, Eli Lilly, Pfizer, Sanofi, LEO, UCB Pharma and Novartis. N.J.R. has received travel support, research grants (Newcastle University) and income to Newcastle University for advisory boards/lectures from AbbVie, Almirall, Celgene, Boehringer Ingelheim, Janssen Cilag, Novartis and UCB Pharma. A.B. has received travel bursaries and performed ad hoc consultancy and lecturing roles with AbbVie, Almirall, Galderma, Eli Lilly, Janssen, LEO Pharma, Novartis, UCB Pharma, Pfizer, BMS, MSD and Sanofi. R.B.W. has acted as a consultant and/or speaker for and/or received research grants from AbbVie, Amgen, Almirall, Celgene, Eli Lilly, Pfizer, LEO Pharma, Novartis, Janssen Cilag, Medac, UCB Pharma and Xenoport. C.H.S. reports grants from a Medical Research Council-funded stratified medicine consortium with multiple industry partners, grants from an Innovative Medicines Initiative (Horizon 2020)-funded European consortium with multiple industry partners, and other grants from AbbVie, Novartis, Pfizer, Sanofi, Boehringer Ingelheim and Sobi, outside the submitted work; she is also chair of UK guidelines on biologic therapy in psoriasis. C.E.M.G. has received honoraria and/or research grants from AbbVie, Almirall, Amgen, AnaptysBo, Bristol Myers Squibb, Celgene, Galderma, LEO Pharma, Eli Lilly, GSK-Stiefel, Janssen Cilag, MSD, Novartis, Pfizer, Sandoz and UCB Pharma., (© The Author(s) 2023. Published by Oxford University Press on behalf of British Association of Dermatologists.)

In excess of three quarters of patients with psoriatic arthritis (PsA) have preceding psoriasis (PsO), which offers a clinical biomarker for the recognition of early PsA. Numerous surveys have shown a remarkably high frequency of clinically occult musculoskeletal symptoms in psoriasis patients. Imaging studies, particularly ultrasound, show a high prevalence of subclinical enthesitis and other inflammatory changes in psoriasis subjects. Since a serum biomarker, such as the case of anti-citrullinated protein antibodies (ACPA) in rheumatoid arthritis, neither exists nor seems biologically plausible at this point, this article explores how integration of rheumatological and dermatological assessment can be facilitated for the early recognition of potential PsA. Given that scalp disease is a PsA predictor, but may be managed in the community, then a particular need to access this group is needed. An integrated approach between rheumatology and dermatology can involve joint clinics, parallel clinics with discussion of relevant cases or virtual contact between specialties. Early therapy evaluation and integrated strategies have considerable implications for minimizing suffering and joint damage in PsA., Competing Interests: A.Z. has received honoraria for educational lectures and/or advisory board contribution from Celgene, Jannssen, Novartis, and UCB. P.M.L. has received honoraria and/or grants as an investigator, speaker, and/or advisory board member from AbbVie, Actelion, Celgene, Janssen, Leo, Lilly, Sanofi, UCB, Almirall, and Novartis. M.W. has received honoraria for educational lectures and/or advisory board contribution from Jannssen, Leo, Novartis, Biogen, Sanofi, and UCB.

Background: Expression of vascular endothelial growth factor A (VEGFA) is increased in chronic inflammatory skin diseases, including psoriasis, and loci for two VEGFA single nucleotide polymorphisms are associated with early-onset psoriasis (presenting before the age of 40 years). Studies have suggested that expression of placenta growth factor (PGF) is also upregulated in cutaneous inflammation and that VEGFA-mediated angiogenesis may be dependent on the simultaneous presence of PGF within the skin., Aim: To elucidate the biological importance of PGF in psoriasis., Methods: We investigated whether two commonly occurring PGF polymorphisms were associated with early-onset psoriasis and the genetic interaction between VEGFA and PGF in psoriasis., Results: We observed a significant (P = 0.04) association between rs2268614 TT and rs2268615 AA genotypes of PGF and early-onset psoriasis. In addition, genetic complement, comprising the PGF rs2268615 AA genotype and the VEGFA -460 (rs833061) T allele, was significantly associated with the development of early-onset psoriasis (P < 0.03). We identified that the VEGFA genotype influences PGF expression (P = 0.001) and that mean plasma levels of PGF are lower in patients with severe psoriasis compared with those with mild-moderate disease (P = 0.04)., Conclusion: Our observed genetic interaction between PGF and VEGFA appears relevant to psoriasis, a disease with an angiogenic basis, and may influence development of an antiangiogenic approach to treatment., (© 2019 The Authors. Clinical and Experimental Dermatology published by John Wiley & Sons Ltd on behalf of British Association of Dermatologists.)

Objectives: The objectives of this study were to assess the reliability of a novel objective outcome measure, laser Doppler imaging (LDI), its validity against skin biopsy histology and other clinical instruments, including localized cutaneous lupus disease area and severity index (L-CLASI) and visual analogue scale (VAS) score of photographs, and its responsiveness to clinical change with therapy., Methods: A prospective observational cohort study was conducted in 30 patients with active cutaneous lupus erythematosus (CLE). At baseline and 3 months, disease activity was assessed using L-CLASI and a high resolution LDI system by two assessors. Skin biopsy was scored as 0 = non-active, 1 = mild activity and 2 = active. Photographs were assessed by two clinicians using 100 mm VAS. Inter-rater reliability was analyzed using Bland-Altman limits of agreement. Correlation between histology and LDI, L-CLASI and VAS and sensitivity to change of LDI with physician subjective assessment of change (PSAC) at 3 months were analyzed using Kendall's tau-a., Results: Of 30 patients with CLE, 28 (93%) were female, mean (SD) age 48.4 (11.5) y, 25 (83%) were Caucasians, 25 (83%) had concurrent systemic lupus erythematosus and 16 (53%) were smokers. CLE subtypes were acute = 9, subacute = 8 and chronic = 13. Inter-rater agreement for LDI was fair but for VAS score of photographs was poor. In 20 patients with biopsy, correlation with histology was better for LDI (tau-a = 0.53) than L-CLASI (tau-a = 0.26) (difference = 0.27; 90% CI 0.05-0.49) or VAS score of photographs (tau-a = 0.17) (difference = 0.36; 90% CI 0.04-0.68). There was a moderate correlation between PSAC score and change in LDI (tau-a = 0.56; 90% CI 0.38-0.74; p  < 0.001, n  = 15)., Conclusion: LDI provides a reliable, valid and responsive quantitative measure of inflammation in CLE. It has a better correlation with histology compared to clinical instruments. LDI provides an objective outcome measure for clinical trials.

Primary localized cutaneous amyloidosis is a group of rare conditions where amyloid deposition is limited to the skin without systemic manifestations. Most cases are sporadic; however, mutations in the oncostatin M receptor (OSMR) and interleukin-31 receptor A (IL31RA) genes can cause a familial form of the condition in up to 10% of cases. Here, we describe a family in which 8 female individuals are affected by either macular amyloidosis or amyloidosis cutis dyschromica. To the best of our knowledge, a sex-specific expression or the coexistence of 2 different phenotypes of primary localized cutaneous amyloidosis in 1 pedigree has not yet been reported.

Background: Many patients with psoriasis have undiagnosed psoriatic arthritis. Low specificity is found with many PsA screening tools. A new instrument, the CONTEST questionnaire, was developed utilizing the most discriminative items from existing instruments., Objective: The aim of this study was to compare the CONTEST and PEST screening tools., Methods: People attending secondary care clinics with psoriasis, but not PsA, completed the questionnaires, were assessed for function and quality of life, and had a physical examination. Patients thought to have PsA were compared to those without. The performance of CONTEST and PEST was compared using area under the receiver operating curve (AUC), and sensitivity and specificity at the previously published cut-offs., Results: A total of 451 dermatology patients were approached, 35% were reviewed and 27 (17%, 95% CI 12.3-21.7) had unidentified psoriatic arthritis. The sensitivity and specificity (95% CI) of PEST were 0.60 (0.42-0.78)/0.76 (0.69-0.83) and for CONTEST 0.53 (0.34-0.72)/0.71 (0.63-0.79). The confidence limits for the AUC overlapped (AUC for PEST 0.72 (0.61-0.84), for CONTEST 0.66 (0.54-0.77)., Conclusions: PEST and CONTEST questionnaires performed equally well, with no superiority of the new CONTEST tool., (© 2018 European Academy of Dermatology and Venereology.)

Background: Pemphigus herpetiformis (PH) is a rare subtype of pemphigus that presents challenges in diagnosis., Objective: To review the presentation, diagnosis, and management of PH., Methods: We reviewed the charts of all patients diagnosed and treated for PH in an immunobullous referral center between September 2007 and June 2013., Results: Eight patients were identified with a diagnosis of PH. All presented initially with pruritus. Clinical disease was manifest as either urticated erythematous plaques or a vesiculobullous eruption. Histological evaluation demonstrated eosinophilic spongiosis in all patients with acantholysis in half of cases (n = 4/8). Peripheral eosinophilia was noted in three of eight (37.5%) patients. In all cases, direct immunofluorescence showed intercellular deposition of immunoglobulin G in the epidermis. All patients required high-dose corticosteroid initially. All patients treated with dapsone or sulfasalazine (n = 4) achieved at least partial control. Other effective treatments included intravenous immunoglobulin (n = 2), azathioprine (n = 2), and leflunomide (n = 1). Rituximab was ineffective in two patients., Conclusion: The clinical and histological features of PH develop over time and with treatment, making distinction between pemphigus subtypes challenging and delay in diagnosis common. Diagnosis of PH requires a high index of suspicion and is made on clinical grounds (urticated erythema) in the context of compatible histology and immunofluorescence findings. Treatment may be challenging, although efficacy of sulfonamide derivatives appears to offer a therapeutic effect., (© 2015 The International Society of Dermatology.)

Mycosis fungoides (MF), the most common cutaneous T-cell lymphoma (CTCL), is rare in childhood. The prognosis and response to treatment are poorly described in children. The objective of the current study was to evaluate the response to phototherapy in a pediatric cohort. A retrospective cohort study of all patients diagnosed with MF before the age of 18 years and referred to the regional CTCL phototherapy service was performed between January 1990 and April 2012. Twenty-eight patients were identified (13 boys, 15 girls). The mean age at presentation was 11.6 ± 3.9 years. The hypopigmented variant was noted in 79% of patients. All patients had stage I disease (IA = 10, IB = 17, unknown = 1). The median follow-up after diagnosis was 43 months (range 6-274 mos). Narrowband ultraviolet B (NbUVB; 311 nm) was used as first-line phototherapy in 18 patients and psoralen (bath) plus ultraviolet A (PUVA) was used in 8 patients. Complete or partial response was observed in 19 of 22 patients (86%). A further course of phototherapy was required in 7 of 12 patients (58%) treated with NbUVB after a median of 4 months (range 4-29 mos). A further course of phototherapy was required in four of eight patients (50%) successfully treated with PUVA after a median of 45.5 months (range 30-87 mos). No disease progression was noted over the follow-up (median 43 mos). The majority of patients in our cohort had hypopigmented MF. Phototherapy offers an effective option for treatment of childhood MF, although the period of remission may be greater in patients treated with PUVA., (© 2014 Wiley Periodicals, Inc.)

Importance: Dermatologists are using an increasing range of immunomodulatory therapies to treat an expanding number of skin diseases. Complications of therapy are broad and include infection. Cytomegalovirus (CMV) retinitis has not been reported in association with dermatologic disease., Observations: We report two cases of CMV retinitis associated with immunosuppression for eczema and pemphigus vulgaris. In both cases, patients were receiving corticosteroid and a second-line immunosuppressive agent (cyclosporine or mycophenolate mofetil). Disease presented in both patients with painless visual loss., Conclusions and Relevance: Patients receiving immunosuppressive therapy should be monitored for blurred vision, floaters, or visual loss and referred for urgent assessment to ensure accurate diagnosis and prompt treatment of possible CMV retinitis.

Epidemiological studies of severe drug hypersensitivities are important to understanding the morbidity and mortality of this heterogeneous group of disorders. These insights also allow greater identification of at-risk patient groups. However, epidemiological studies of drug hypersensitivity reactions are challenging due to the variable diagnostic criteria applied and incomplete data sets studied. We review the epidemiology of severe drug hypersensitivity reactions with a particular focus on severe cutaneous adverse reactions (SCARs). SCAR diseases include: Stevens-Johnson syndrome, toxic epidermal necrolysis, drug rash eosinophilia and systemic symptoms, serum-sickness-like reaction and acute generalized exanthematous pustulosis.

Psoriasis is a common chronic inflammatory disease of the skin that has a significant impact on quality of life. A small number of systemic therapies are well established in psoriasis management. These have immunosuppressive and/or anti-proliferative effects on the skin and immune system. As understanding of the pathogenesis of psoriasis has advanced over the last 2 decades, there has been clearer appreciation of the genetic, cellular and immunological components of disease expression, which has provided new insight into potential therapeutic targets, including the development of biological therapies. Biologics offer a unique opportunity to block or inhibit specific key components of psoriasis pathogenesis. The introduction of tumour necrosis factor (TNF).α and interleukin (IL)-12/-23 inhibitors has resulted in remarkable clinical responses in patients with severe psoriasis and has led to the development of a range of other cytokine modulators currently undergoing investigation. More recently, research in keratinocyte biology and immune cell function, particularly intracellular signalling, has afforded additional opportunities to develop a range of small-molecule oral preparations that may prove effective in disease control. This paper reviews current and emerging systemic treatments in the management of psoriasis.

Background: UK medical graduates are required to reach standards defined by the General Medical Council (in Tomorrows Doctors) in all specialties, including dermatology., Aims: We assessed the self-reported competence of Foundation Year 1 (FY1) doctors in the diagnosis and management of skin disease correlated against their undergraduate dermatology experience., Methods: The FY1 doctors attending a teaching programme were surveyed at six hospital sites throughout the North Western Deanery. Questionnaires were completed on self-reported competence in learning outcomes defined by the British Association of Dermatologists (BAD). Information recorded previous undergraduate teaching (duration and delivery), and whether they felt sufficiently prepared to diagnose and manage patients with skin disease in their clinical practice., Results: Of 174 FY1 doctors, 118 attended the teaching sessions. All of the attendees completed a questionnaire. Trainees who undertook longitudinal placements in dermatology (defined as 10 or more half days of clinical experience) regarded themselves significantly more prepared to practise for their stage of training: 61 per cent compared with 8.9 per cent (χ(2) = 32.8, df = 1, p < 0.05). Furthermore, these trainees rated their abilities in basic history taking, clinical examination, management of dermatological emergencies and diagnosis of skin malignancy as being greater than those who had not experienced longitudinal placements in the specialty., Conclusions: Longitudinal placements in dermatology offer undergraduates experience in the diagnosis and management of skin disease that develops confidence for foundation practice., (© Blackwell Publishing Ltd 2012.)

Background: There are limited data on the use of ustekinumab outside of clinical trials., Objectives: To assess the efficacy and safety of ustekinumab in patients with severe psoriasis attending 10 dermatology centres in the U.K. and Ireland., Methods: A retrospective case-note review of 129 patients with psoriasis treated with ustekinumab., Results: Baseline Psoriasis Area and Severity Index (PASI) was 22·9±10·1 (mean±SD). After 16weeks of treatment with ustekinumab PASI 75 (75% reduction in PASI) was observed in 63·0% (n=80/127) of patients, although four patients required concomitant therapy at the 16-week time point. Previous biologic use did show a small, non-significant trend towards treatment failure. A PASI 75 response was seen in 29·4% (n=5/17) of individuals weighing 90-100kg and treated with the standard 45mg ustekinumab dose compared with PASI 75 of 70·3%, 71·4%, 75·0% and 55·6% for weight groups <80, 80-90, 100-110 and >110kg, respectively (P=0·024). Ustekinumab therapy was well tolerated; serious adverse events were observed in 2·3% (n=3/129) of patients., Conclusions: Ustekinumab is a novel biologic agent for psoriasis. When used in everyday clinical practice it demonstrates high levels of short-term therapeutic efficacy with an acceptable short-term safety profile., (© 2011 The Authors. BJD © 2011 British Association of Dermatologists.)

Management of psoriasis over the last decade has changed significantly with the introduction of biological therapies. Ustekinumab is a first-in-class biological agent, inhibiting the action of IL-12 and IL-23, and has provided further evidence for the role of Th1 and Th17 lymphocytes in the pathogenesis of psoriasis. Efficacy has been clearly demonstrated in three Phase III clinical trials. Week 12 Psoriasis Area and Severity Index (PASI) 75 was observed in 66.4-75.7% of patients with PASI 90 achieved in 36.7-50.9%. This marked clinical response is also reflected in a significant improvement in quality of life. The most recent Phase III clinical trial has demonstrated the superior efficacy of ustekinumab (regardless of dosing regimen) compared with high-dose etanercept at week 12. Long-term efficacy has been demonstrated over 148 weeks with 64-76% of patients maintaining PASI 75. The role of ustekinumab in the treatment of psoriatic arthritis has shown some benefit in Phase II clinical trials. Phase III clinical trials are pending and will provide further guidance on management of concurrent disease. The currently available safety data are on the whole reassuring, although ongoing vigilance remains central to the detection of rare or late sequelae.

Tuberculosis verrucosa cutis is an uncommon form of tuberculosis that typically presents as a chronic warty plaque. It develops in individuals with moderate to high immunity to Mycobacterium tuberculosis due to inoculation of an open wound. We present the case of a Somali man born in the United Kingdom who presented with a nonhealing ulcer on the right hand of 10 years' duration. The patient was diagnosed with tuberculosis verrucosa cutis based on clinical suspicion, which was confirmed by several investigations including strongly positive results of a Mantoux test, IFN-gamma release assay, typical histology on skin biopsy, and polymerase chain reaction (PCR) analysis positive for mycobacterial DNA. Treatment with quadruple antituberculous therapy produced rapid resolution of the ulcer. This unusual condition often is overlooked in the differential diagnosis of nonhealing ulcers, yet it has an excellent prognosis with treatment. A high index of suspicion is required.

Importance of the Field: The majority of patients with psoriasis can be safely and effectively treated with topical therapy alone, either under the supervision of a family physician or dermatologist. For those requiring systemic agents, topical therapies can provide additional benefit. Optimal use of topical therapy requires an awareness of the range and efficacy of all products., Areas Covered in This Review: The review covers the efficacy and role of topical therapies including emollients, corticosteroids, vitamin D analogs, calcineurin inhibitors, dithranol, coal tar, retinoids, keratolyics and combination therapy. The report was prepared following a PubMed and Embase literature search up to April 2010., What the Reader Will Gain: The paper provides a broad review of the relevant topical therapeutic options available in routine clinical practice for the management of psoriasis and a recommendation for selection of treatment., Take Home Message: Topical therapies used appropriately provide a safe and effective option for the management of psoriasis. An awareness of the available products and their efficacy is key to treatment selection and patient satisfaction.

Psoriasis is a common, chronic inflammatory skin disease which is associated with a number of significant co-morbidities including: impairment of quality of life; cardiovascular disease; and a seronegative arthritis known as psoriatic arthritis. Our understanding of the pathogenesis of psoriasis has developed at a remarkable rate in recent years. These new insights have significantly changed our perception of the condition and have led to the development of several new treatment strategies. Biological agents have proved a major step forward in therapeutic options for psoriasis. The ability to clear, or almost clear, cutaneous disease has changed the outcomes and expectations of many patients with this disease. The impact on both physical and psychological health may be great. This review covers the clinical features and management of psoriasis with specific reference to new therapeutic options.