Systematic Review and Critical Appraisal of Urticaria Clinical Practice Guidelines: A Global Guidelines in Dermatology Mapping Project (GUIDEMAP).

BACKGROUND: Management of urticaria can be optimized with clinical practice guidelines (CPGs). However, the quality of recent urticaria CPGs remains unclear. OBJECTIVE: To identify and appraise urticaria CPGs worldwide published in the last 5 years. METHODS: A search for relevant urticaria CPGs was conducted between January 1, 2017, and May 31, 2022, using the following databases: MEDLINE, Embase, National Institute for Health and Care Excellence (NICE) Evidence Search, Guidelines International Network, ECRI Guidelines Trust, Australian Clinical Practice Guidelines, Trip Medical Database, and DynaMed. The included CPGs were critically appraised using the Appraisal of Guidelines for Research and Evaluation (AGREE) II instrument, Lenzer et al's red flags, and the Institute of Medicine (IOM) criteria of trustworthiness. RESULTS: We included 21 urticaria CPGs. Most guidelines reviewed treatment recommendations of chronic spontaneous urticaria. The majority of guidelines were from European and Asian countries with high and high-middle sociodemographic index, written in English, and openly accessible. Seventeen guidelines (81%) had at least 1 AGREE II domain rated poor quality. Applicability, rigor of development, and stakeholder involvement were the 3 AGREE II domains that scored the lowest across guidelines. Appraisal with Lenzer et al's red flags showed that 18 guidelines (86%) raised at least 1 red flag indicating potential bias. The top 3 domains raising red flags were: no inclusion of nonphysician experts/patient representative/community stakeholders, no or limited involvement of a methodologist in the evaluation of evidence, and lack of external review. Based on IOM's criteria of trustworthiness, 20 guidelines (95%) had 1 or more criteria that did not meet best practice standards. The 3 domains with the highest number of best practice standards not met were updating procedures, rating strength of recommendations, and external review. Guidelines scored highest for the AGREE II domains of defining scope and purpose and clarity of presentation, and had the most fully met IOM's best practice standard for articulation of recommendations. However, only 1 urticaria CPG by NICE was identified as rigorously developed across all 3 appraisal tools. CONCLUSIONS: The quality of urticaria CPGs in the last 5 years varied widely. Only the NICE urticaria guideline consistently demonstrated excellent quality, high trustworthiness, and low risk of bias. Use of a rigorous framework to rate certainty of evidence and grade strength of recommendation, involvement of methodologists, stakeholder engagement with external review, and clear guidance for updating can help improve the quality of future CPGs.

The associations between myositis autoantibodies and clinical presentations in dermatomyositis.

BACKGROUND: The myositis autoantibodies have been widely used clinically in recent years for the identification of an autoantibody-associated clinical phenotype in dermatomyositis (DM) patients. However, correlations between myositis autoantibodies and clinical presentations in different populations are lacking, especially in Taiwan. OBJECTIVES: To investigate the correlations among cutaneous manifestations, myositis autoantibodies, and systemic diseases, including interstitial lung disease (ILD) and internal malignancy. METHODS: A retrospective study of patients with histopathologically confirmed cutaneous manifestations of DM was conducted during 2005 to 2020 in Taiwan. A commercial line blot immunoassay technique was used to detect myositis autoantibodies. RESULTS: A total of 88 DM patients were enrolled, with a mean age of onset of 49.4 years old. The most common systemic features were myositis (56.8%, 50/88), internal malignancy (22.7%, 20/88), dysphagia (19.3%, 17/88), and ILD (17%, 15/88). Among the enrolled patients, 32 patients received serum myositis autoantibodies examination. The most common autoantibodies were ANA (50.7%, 37/73), followed by anti-TIF1-γ (34.4%, 11/32) and anti-MDA5 (31.3%, 10/32) antibodies. Patients with Gottron sign (OR 5.6), arthritis (OR 23.35), or the presence of anti-MDA5 antibody (OR 11.14) were more susceptible to progressing to ILD, whereas patients with pruritus (OR 1.04), dysphagia (OR 6.73), and the presence of ANA (OR 6.29) had significantly higher risks of developing internal malignancies. CONCLUSIONS: Physicians should pay special attention to certain clinical features, which can help with the early detection of systemic diseases. Cancer screening and myositis autoantibodies examination should be conducted in all DM patients if applicable.

Systematic review and critical appraisal of psoriasis clinical practice guidelines: a Global Guidelines in Dermatology Mapping Project (GUIDEMAP).

BACKGROUND: Clinical practice guidelines (CPGs) developed with rigorous methods can help optimize clinical care for patients with psoriasis. OBJECTIVES: To conduct an updated systematic review and comprehensive critical appraisal of global psoriasis CPGs. METHODS: A search of MEDLINE and Embase for psoriasis CPGs published between 1 January 2015 and 31 March 2021 was performed. Other guideline repositories were also searched for relevant CPGs. Descriptive analysis was conducted to summarize included guidelines. Three critical appraisal tools were used to assess the quality of included CPGs: the Appraisal of Guidelines for Research and Evaluation (AGREE) II instrument, Lenzer et al.'s red flags, and the US Institute of Medicine's (IOM) criteria of trustworthiness. RESULTS: We included 33 psoriasis CPGs, with 25 openly accessible. Most CPGs were from high sociodemographic index countries in North America and Europe. Five CPGs received 'excellent quality' appraisals across all six AGREE II domains. Stakeholder involvement, rigour of development and applicability were the three domains with the lowest appraisal scores for AGREE II. Twenty-two CPGs raised at least one red flag indicative of potential bias. By the IOM's standards, external review of the guideline draft prior to publication and clear updating procedures were most often not addressed by guidelines, and only three CPGs were assessed as having higher overall trustworthiness. CONCLUSIONS: Most psoriasis guidelines were unable to consistently demonstrate high quality across multiple appraisal tools. The EuroGuiDerm guideline on the systemic treatment of psoriasis vulgaris was the only CPG to receive 'excellent quality' across all six AGREE II domains, to raise no Lenzer's red flags, and to have higher trustworthiness by IOM criteria.

Anti-IL-17A antibody-associated de novo vitiligo: Case report and review of literature.

Interleukin (IL)-17 inhibitor is a biological therapy approved for moderate to severe psoriasis and psoriatic arthritis. The common adverse events of IL-17 inhibitor include injection site reaction, infections, nasopharyngitis, and headache. However, vitiligo associated with the use of IL-17 inhibitors was rarely reported in the previous literature. Here we described a woman who developed de novo vitiligo after 4 months of IL-17A inhibitor treatment for psoriasis and psoriatic arthritis. Upon discontinuation of IL-17A inhibitor and shifting to a broader T cell inhibitor-cyclosporine, our patient had control of both psoriasis and vitiligo and achieved 75% repigmentation after 3 months of oral cyclosporine without phototherapy. Due to the increasing use of anti-IL-17 biologics in psoriasis patients, clinicians should inquire about vitiligo's history before treatment and inform patients of the possible adverse effects.

Successful treatment of corticosteroid-dependent drug reaction with eosinophilia and systemic symptoms with cyclosporine.

BACKGROUND: Drug reaction with eosinophilia and systemic symptoms (DRESS) is an intractable drug hypersensitivity disease with high mortality. The current standard treatment requires high-dose and long-term systemic corticosteroids, which may lead to adverse effects and intolerability of patients. OBJECTIVE: To evaluate the efficacy and safety of cyclosporine in patients with corticosteroid-dependent DRESS or intolerance to systemic corticosteroids. METHODS: A retrospective review of 8 patients with corticosteroid-dependent DRESS who were treated with cyclosporine as an alternative treatment owing to suboptimal response to regular doses of systemic corticosteroids for at least 3 weeks, flare-ups when tapering corticosteroids, or experiencing intolerable adverse effects of corticosteroids. RESULTS: In all 8 patients (4 women and 4 men; age range, 15-75 years), either intractable skin eruptions, persistent eosinophilia, or elevated liver function was noted after at least 3 weeks of treatment with systemic corticosteroids. The patients had marked cutaneous improvement and normalization of liver function and eosinophil count after adding cyclosporine, and the systemic corticosteroid treatment was smoothly tapered down. The mean dosage of cyclosporine was 1.68 ± 0.73 mg/kg/d, and the mean duration of cyclosporine treatment was 76.13 ± 35.64 days. Their serum eosinophil counts, serum alanine aminotransferase levels, and serum thymus and activation-regulated chemokine levels were all elevated at baseline and then significantly decreased during the recovery stage after cyclosporine therapy (P < .05). No adverse events were reported after adding cyclosporine. CONCLUSION: Cyclosporine is an effective and safe therapeutic alternative as a steroid-sparing agent for corticosteroid-dependent DRESS. Further prospective randomized controlled studies are required to confirm these preliminary results.

Dermoscopic features and differential diagnosis of sebaceous carcinoma.

Sebaceous carcinoma is a rare malignant skin neoplasm arising from sebaceous glands. Its clinical diagnosis is difficult and reports of dermoscopic findings have been limited. This study aims to analyze its dermoscopic features and differential diagnosis in dermoscopic examination. The study included patients diagnosed with histologically proven sebaceous carcinomas as well as diagnosed cases of sebaceous hyperplasia, sebaceoma, squamous cell carcinoma and basal cell carcinoma for comparison of dermoscopic findings. The dermoscopic criterion of presence of sebaceous carcinoma was scored only if the two evaluators reached a consensus. Fifteen cases of histologically diagnosed sebaceous carcinoma were included in our study. All cases were extraocular sebaceous carcinoma. A total of 60 (15 basal cell carcinomas, 15 squamous cell carcinomas, 15 sebaceous hyperplasias and 15 sebaceomas) cases were collected for comparing dermoscopic features with sebaceous carcinoma. In dermoscopic analysis of sebaceous carcinoma, the majority of tumors (66.67%) presented polymorphic vessel pattern. Other features included whitish-pink areas (80%), yellowish structures (73.33%) and yellowish structureless areas (60%). Yellowish structures in sebaceous carcinomas are the main dermoscopic findings to differentiate squamous cell and basal cell carcinomas (P < 0.001), whereas purplish globules, shiny white blotches and strands and whitish-pink area distinguish sebaceous carcinomas from other sebaceous tumors (P < 0.05).

Primary scarring alopecia: A retrospective study of 89 patients in Taiwan.

Primary scarring alopecia (PSA) is caused by irreversible damage to the hair epithelial stem cells that reside in hair follicles. There is limited published work regarding PSA amongst the Asian population. The aim of this study was to evaluate the clinical features and to characterize the subtypes of PSA in southern Taiwan. In this retrospective case series, we reviewed 89 patients with pathology-confirmed PSA. The data was collected from National Cheng Kung University Hospital between 1988 through 2016. The clinical and histological data were reviewed, and the patients were characterized into different subtypes of PSA based on the clinical features and histological findings. We noted seven different subtypes of PSA. The most common type was dissecting cellulitis (DC) (30.3%), followed by lichen planopilaris (LPP) (23.5%), central centrifugal cicatricial alopecia (CCCA) (12.4%) and acne keloidalis nuchae (AKN) (12.4%). The other subtypes include folliculitis decalvans (FD), discoid lupus erythematosus (DLE) and pseudopelade of Brocq (PPB). Interestingly, FD, DC and AKN were more common in males, while CCCA, LPP, DLE and PPB had a female predominance. The mean age of patients with DLE, DC and AKN were younger, while patients with CCCA, LPP, PPB and FD tend to be older. The pattern of hair loss was more likely to be unifocal-ragged border in CCCA and DLE, multifocal-interconnected in LPP and FD, and multifocal-separated in DC. The pathogenesis of PSA may be influenced by sex, age and genetic background. It is important to identify the hair loss pattern to differentiate the subtypes of PSA.