Japanese Clinical Practice Guidelines for Vascular Anomalies 2017

The objective was to prepare guidelines to perform the current optimum treatment by organizing effective and efficient treatments of hemangiomas and vascular malformations, confirming the safety and systematizing treatment, employing evidence-based medicine techniques and aimed at improvement of the outcomes. Clinical questions (CQ) were decided based on the important clinical issues. For document retrieval, key words for published work searches were set for each CQ, and work published from 1980 to the end of September 2014 was searched in PubMed, Cochrane Library and Japana Centra Revuo Medicina databases. The strengths of evidence and recommendations acquired by systematic reviews were determined following the Medical Information Network Distribution System technique. A total of 33 CQ were used to compile recommendations and the subjects included efficacy of resection, sclerotherapy/embolization, drug therapy, laser therapy, radiotherapy and other conservative treatment, differences in appropriate treatment due to the location of lesions and among symptoms, appropriate timing of treatment and tests, and pathological diagnosis deciding the diagnosis. Thus, the Japanese Clinical Practice Guidelines for Vascular Anomalies 2017 have been prepared as the evidence-based guidelines for the management of vascular anomalies.     

Wound,pressure ulcer and burn guidelines – 6: Guidelines for the management of burns,second edition

“Wound, pressure ulcer and burn guidelines – 6: Guidelines for the management of burns, second edition” is revised from the first edition which was published in the Japanese Journal of Dermatology in 2016. The guidelines were drafted by the Wound, Pressure Ulcer and Burn Guidelines Drafting Committee delegated by the Japanese Dermatological Association, and intend to facilitate physicians’ clinical decisions in preventing, diagnosing and treating burn injury. All sections are updated by collecting documents published since the publication of the first edition. Especially, the recommendation levels of dressing materials newly covered by the Japanese national health insurance are mentioned. In addition, the clinical questions (CQ) regarding the initial treatment of electrical (CQ15) and chemical burns (CQ16), and also the use of escharotomy (CQ22), are newly created.     

Clinical practice guide for the treatment of perforating dermatosis

Perforating dermatoses are a heterogeneous skin disease group defined by transepidermal elimination of various skin materials. Four classical forms of primary perforating dermatosis have been described, where the transepidermal elimination mechanism represents the hallmark of the disease: acquired reactive perforating collagenosis, elastosis perforans serpiginosa, Kyrle’s disease and perforating folliculitis. Acquired reactive perforating collagenosis presents with transepidermal elimination of collagen fibers. Elastosis perforans serpiginosum presents with the elimination of elastic fibers. Kyrle’s disease presents with transepidermal elimination of abnormal keratin. In perforating folliculitis, it is the content of the follicle. We established diagnostic criteria and severity classification. In addition, the Japanese guideline for treatment of perforating dermatoses was updated using the Medical Information Network Distribution Service (MINDS) methodology. The guideline is based on a systematic published work review completed from 1989 to 2019, and on a formal consensus and approval process. Most medical published work on the treatment is limited to individual case reports and small series of patients. The guideline covers treatment options considered relevant by the expert panel and approved in Japan at the time of the consensus conference.     

Consensus Update for Systemic Treatment of Atopic Dermatitis

BackgroundIn 2015, the Korean Atopic Dermatitis Association (KADA) working group published consensus guidelines for treating atopic dermatitis (AD).ObjectiveWe aimed to provide updated consensus recommendations for systemic treatment of AD in South Korea based on recent evidence and experience.MethodsWe compiled a database of references from relevant systematic reviews and guidelines on the systemic management of AD. Evidence for each statement was graded and classified based on thestrength of the recommendation. Forty-two council members from the KADA participated in three rounds of voting to establish a consensus on expert recommendations.ResultsWe do not recommend long-term treatment with systemic steroids forpatients with moderate-to-severe AD due to the risk of adverse effects. We recommend treatment with cyclosporine or dupilumab and selective treatment with methotrexate or azathioprine for patients with moderate-to-severe AD. We suggest treatment with antihistamines as an option for alleviating clinical symptoms of AD. We recommend selective treatment with narrowband ultraviolet B for patients with chronic moderate-to-severe AD. We do not recommend treatment with oral antibiotics for patients with moderate-to-severe AD but who have no signs of infection. We did not reach a consensus on recommendations for treatment with allergen-specific immunotherapy, probiotics, evening primrose oil, orvitamin D for patients with moderate-to-severe AD. We also recommend educational interventions and counselling for patients with AD and caregivers to improve the treatment success rate.ConclusionWe look forward to implementing a new and updated consensus of systemic therapy in controlling patients with moderate-to-severe AD.     

S3 - Guidelines on the treatment of psoriasis vulgaris (English version). Update

Psoriasis vulgaris is a common and often chronic inflammatory skin disease. The incidence of psoriasis in Western industrialized countries ranges from 1.5% to 2%. Patients afflicted with severe psoriasis vulgaris may experience a significant reduction in quality of life. Despite the large variety of treatment options available, surveys have shown that patients still do not received optimal treatments. To optimize the treatment of psoriasis in Germany, the Deutsche Dermatologi sche Gesellschaft (DDG) and the Berufsverband Deutscher Dermatologen (BVDD) have initiated a project to develop evidence-based guidelines for the management of psoriasis. They were first published in 2006 and updated in 2011. The Guidelines focus on induction therapy in cases of mild, moderate and severe plaque-type psoriasis in adults including systemic therapy, UV therapy and topical therapies. The therapeutic recommendations were developed based on the results of a systematic literature search and were finalized during a consensus meeting using structured consensus methods (nominal group process).     

Detection of the levels of neuropeptides, ACTH and cortisol in the blood of patients with polymyositis/dermatomyositis and their significance

Recent research has shown that the neuroendocrine system can regulate the function of the immune system and that ACTH and cortisol play important roles in maintaining the immune homeostasis. Polymyositis and dermatomyositis (PM/DM) are autoimmune diseases with unclear pathogeneses closely related with immune disorders, so we detected the levels of neuropeptide Y (NPY), beta-endorphin (beta-EP), calcitonin gene-related peptide (CGRP), adrenocoricotropic hormone (ACTH), and cortisol in blood of patients with PM/DM to investigate the relationship between these indices and the pathogenesis of PM/DM. The detection of NPY, beta-EP, CGRP, and ACTH concentrations in plasma and cortisol in serum of 28 cases of PM/DM was carried out using radioimmunoassay methods, and the results were compared with those of 20 normal controls. The levels of NPY in the plasma of PM/DM was significantly higher than those of the controls, while beta-EP, CGRP and ACTH were significantly lower than those of the controls, and cortisol was not significantly different before treatment. Linear correlation analysis indicated that NPY was significantly positively correlated with CPK, and beta-EP and CGRP were significantly negatively correlated with CPK. There were no significant correlations among cortisol ACTH, and CPK and no significant correlations between NPY, beta-EP, CGRP, ACTH, cortisol and age or duration of disease before treatment. After treatment for three months, NPY, beta-EP and CGRP tended to become normal and no longer significantly different from the control values. However, ACTH fell further and was significantly lower than the level before treatment. Therefore the increase in NPY and the decreases in beta-EP, CGRP, and ACTH in the plasma of PM/DM patients may be related to the pathogenesis of PM/DM.     

多发性肌炎/皮肌炎治疗研究进展

多发性肌炎/皮肌炎(polymyositis,PM/dermatomyositis,DM)是以侵犯骨骼肌为主,可累及肌肉、肺、肾等多个脏器的自身免疫性疾病,其临床表现复杂,患者多因肺间质病变、肿瘤等并发症死亡,治疗以糖皮质激素为主,以及免疫抑制剂、生物制剂等,但不良反应大,对相关并发症的效果不理想。而干细胞移植治疗是作为当前治疗难治性PM/DM的一种手段。本文从PM/DM的治疗现状出发进行综述,以期为PM/DM临床治疗提供更多参考。     

[Translated article] Practical Update of the Guidelines Published by the Psoriasis Group of the Spanish Academy of Dermatology and Venereology (GPs) on the Treatment of Psoriasis With Biologic Agents: Part 2—Management of Special Populations,Patients With Comorbid Conditions,and Risk

Background and objectivesSince its inception, the Psoriasis Group (GPs) of the Spanish Academy of Dermatology and Venereology (AEDV) has worked to continuously update recommendations for the treatment of psoriasis based on the best available evidence and incorporating proposals arising from and aimed at clinical practice. An updated GPs consensus document on the treatment of moderate to severe psoriasis was needed because of changes in the treatment paradigm and the approval in recent years of a large number of new biologic agents.MethodologyThe consensus document was developed using the nominal group technique complemented by a scoping review. First, a designated coordinator selected a group of GPs members for the panel based on their experience and knowledge of psoriasis. The coordinator defined the objectives and key points for the document and, with the help of a documentalist, conducted a scoping review of articles in Medline, Embase, and the Cochrane Library up to January 2021. The review included systematic reviews and meta-analyses as well as clinical trials not included in those studies and high-quality real-world studies. National and international clinical practice guidelines and consensus documents on the management of moderate to severe psoriasis were also reviewed. The coordinator then drew up a set of proposed recommendations, which were discussed and modified in a nominal group meeting. After several review processes, including external review by other GPs members, the final document was drafted.ResultsThe present guidelines include updated recommendations on assessing the severity of psoriasis and criteria for the indication of systemic treatment. They also include general principles for the treatment of patients with moderate to severe psoriasis and define treatment goals for these patients as well as criteria for the indication and selection of initial and subsequent therapies Practical issues, such as treatment failure and maintenance of response, are also addressed.     

The wound/burn guidelines – 3: Guidelines for the diagnosis and treatment for diabetic ulcer/gangrene

We aimed to prepare guidelines for the management of diabetic ulcer/gangrene with emphasis on the diagnosis and treatment of skin symptoms. They serve as a tool to improve the quality of the diagnosis and treatment in each patient and, further, to improve the level of the care for diabetic ulcer in Japan by systematically presenting evidence‐based recommendations for clinical judgments by incorporating various viewpoints.     

Guidelines for diagnosis,prevention and treatment of hand eczema – short version

The guidelines aim to provide advice on the management of hand eczema (HE), using an evidence‐ and consensus‐based approach. The guidelines consider a systematic Cochrane review on interventions for HE, which is based on a systematic search of the published literature (including hand‐searching). In addition to the evidence‐ and consensus‐based recommendation on the treatment of HE, the guidelines cover mainly consensus‐based diagnostic aspects and preventive measures (primary and secondary prevention). Treatment recommendations include non‐pharmacological interventions, topical, physical and systemic treatments. Topical corticosteroids are recommended as first line treatment in the management of HE, however continuous long‐term treatment beyond six weeks only when necessary and under careful medical supervision. Alitretinoin is recommended as a second line treatment (relative to topical corticosteroids) for patients with severe chronic HE. Randomized control trials (RCT) are missing for other used systemic treatments and comparison of systemic drugs in “head‐to‐head” RCTs are needed. The guidelines development group is a working group of the European Society of Contact Dermatitis (ESCD) and has carefully tried to reconcile opposite views, define current optimal practice and provide specific recommendations, and meetings have been chaired by a professional moderator of the AWMF (Arbeitsgemeinschaft der Wissenschaftlichen Medizinischen Fachgesellschaften; Association of the Scientific Medical Societies in Germany). No financial support was given by any medical company. The guidelines are expected to be valid until December 2017 at the latest.     

Actualización práctica de las recomendaciones del Grupo de Psoriasis de la Academia Española de Dermatología y Venereología (GPS) para el tratamiento de la psoriasis con terapia biológica. Parte 2 «Manejo de poblaciones especiales,pacientes con comorbilidad y gestión del riesgo»

Background and objectivesSince its inception, the Psoriasis Group (GPs) of the Spanish Academy of Dermatology and Venereology (AEDV) has worked to continuously update recommendations for the treatment of psoriasis based on the best available evidence and incorporating proposals arising from and aimed at clinical practice. An updated GPs consensus document on the treatment of moderate to severe psoriasis was needed because of changes in the treatment paradigm and the approval in recent years of a large number of new biologic agents.MethodologyThe consensus document was developed using the nominal group technique complemented by a scoping review. First, a designated coordinator selected a group of GPs members for the panel based on their experience and knowledge of psoriasis. The coordinator defined the objectives and key points for the document and, with the help of a documentalist, conducted a scoping review of articles in Medline, Embase, and the Cochrane Library up to January 2021. The review included systematic reviews and meta-analyses as well as clinical trials not included in those studies and high-quality real-world studies. National and international clinical practice guidelines and consensus documents on the management of moderate to severe psoriasis were also reviewed. The coordinator then drew up a set of proposed recommendations, which were discussed and modified in a nominal group meeting. After several review processes, including external review by other GPs members, the final document was drafted.ResultsThe present guidelines include updated recommendations on assessing the severity of psoriasis and criteria for the indication of systemic treatment. They also include general principles for the treatment of patients with moderate to severe psoriasis and define treatment goals for these patients as well as criteria for the indication and selection of initial and subsequent therapies Practical issues, such as treatment failure and maintenance of response, are also addressed.     

Malignancies associated with dermatomyositis and polymyositis in Taiwan: a nationwide population-based study

Background  Previous studies showed that idiopathic inflammatory myopathies (IIM) carried an increased risk of cancers. However, no large-scale study of IIM has been conducted in the Chinese population.
Objectives  We sought to delineate the association of IIM and various cancer types from a nationwide database in Taiwan.
Methods  We analysed the published national data from records of National Health Insurance claims. Cases of dermatomyositis (DM) and polymyositis (PM) from 2000 to 2005 and cancers registered in the catastrophic illness profile from 1997 to 2006 were collected. A nationally representative cohort of 1 000 000 enrollees was included for comparison.
Results  In total, 136 patients (12·8%) among 1059 cases of DM and 46 persons (7·0%) among 661 cases of PM carried internal malignancies. Patients with DM tended to have cancers of nasopharynx, lung and breast. On the other hand, patients with PM tended to have breast, uterine cervix and lung cancers. Compared with the general population, DM gave a 10-fold increased risk for cancers, in which a 66-fold increased risk for nasopharyngeal carcinoma and a 31-fold increased risk for lung cancer were the two most significant. For patients with PM, a 6-fold increased risk for cancer was observed. Juvenile DM had a 16-fold increased risk for haematopoietic or lymphoid malignancy. Two thirds of comorbid malignancies were detected shortly after the diagnoses of IIM, within a mean of 1–2 years. Overall, younger patients with IIM carried the highest risk for malignancies, especially those in their twenties and thirties.
Conclusions  This is the first large-scale study to report the associated malignancies and the cancer risk of IIM in Taiwan.     

Incidence, cancer risk and mortality of dermatomyositis and polymyositis in Taiwan: a nationwide population study

Background Nationwide data on the epidemiology of dermatomyositis (DM) and polymyositis (PM) were limited. Objectives This study was to estimate the incidence, occurrence of cancer and mortality of DM and PM in Taiwan. Methods Both the register of critical illness of the Taiwan National Health Insurance Research Dataset and the National Death Registry of Taiwan were used to calculate estimates of the incidence, cancer association, and mortality of DM and PM between 2003 and 2007. Results A total of 803 DM and 500 PM cases were identified between 2003 and 2007. Mean age at diagnosis was 44·0 ± 18·3 years for DM and 49·2 ± 15·9 years for PM. The overall annual incidences of DM and PM were 7·1 (95% CI 6·6–7·6) and 4·4 (95% CI 4·0–4·8) cases per million population. The incidence of both DM and PM increased with age and reached a peak at age 50–59 years. One hundred and eleven (13·8%) patients with DM and 31 (6·2%) patients with PM had cancers. The diagnosis of most cancers was made after the diagnoses of DM (n = 71; 64·0%) and PM (n = 21; 67·7%). Overall, the standardized incidence ratios (SIR) for cancer were 5·36 (4·12–6·87) and 1·80 (1·10–2·79) among patients with DM and PM; however, during the first year, SIRs for cancer were 24·55 (95% CI 18·62–31·79) and 9·17 (95% CI 14·82–15·93) in patients with DM and PM, respectively. The most common types of cancer were nasopharyngeal cancer for men and breast cancer for women. Patients with DM and PM had standardized mortality ratios of 7·68 (6·41–9·01) and 5·29 (4·28–6·48). Conclusion This study reports robust estimates of important aspects of the epidemiology of both DM and PM in Taiwan. This highlights the rarity of these diseases, and their associated cancer risks and increased mortality.     

Polymyositis and dermatomyositis: Disease spectrum and classification

Muscle inflammation and weakness are the key features of idiopathic inflammatory myopathies (IIMs). In addition IIMs are frequently associated with cutaneous and pulmonary involvement. In clinical practice the three common inflammatory myopathies we come across are polymyositis (PM), dermatomyositis (DM) and inclusion body myositis (IBM). The Bohan and Peter criteria combine clinical, laboratory, and pathologic features to define PM and DM. They did not recognize inclusion body myositis (IBM) or other inflammatory myopathies, such as granulomatous and eosinophilic myositis. Thus the disease spectrum is wide and IIMs are a heterogeneous group of autoimmune disorders. To address these issues in this article we have discussed the currently developing newer classifications of IIMs.     

Japanese Dermatological Association Guidelines: Outlines of guidelines for cutaneous melanoma 2019

With consideration of the ongoing developments in treatment options for cutaneous melanoma, the Japanese Skin Cancer Society published the first guidelines for cutaneous melanoma in 2007 and later revised them in 2015. Here, we report on an English version of the 2019 Japanese Melanoma Guidelines. In this latest edition, all processes were carried out according to the Grading of Recommendations, Assessment, Development and Evaluation system. A comprehensive published work search, systematic review and determination of recommendations in each clinical question were performed by a multidisciplinary expert panel consisting of dermatologists, a plastic and reconstructive surgeon, and a radiation oncologist. The advent of novel agents, such as immune checkpoint inhibitors and molecular-targeted agents, has drastically changed the nature of treatment for adjuvant and advanced-stage diseases among melanoma patients worldwide. Additionally, recent reports of clinical trials regarding surgical procedures and a better understanding of molecular biology and tumor immunology in clinical types of melanoma have had an impact on clinical practise. Based on these viewpoints, eight relevant clinical questions were raised in this report that aim to help clinicians select the appropriate therapeutic approach.     

Predicting factors of malignancy in dermatomyositis and polymyositis: a case-control study

BACKGROUND: An association between dermatomyositis (DM)/polymyositis (PM) and malignancies has been widely reported in the literature. The validity of extensive evaluation for malignancies in those patients has also been questioned for decades. Only limited papers regarding the signs of malignancy and the prognostic factors in DM/PM have been reported. OBJECTIVES: To define the potential risk factors of concomitant neoplastic diseases in patients diagnosed as having DM/PM. METHODS: From 1 April 1983 to 30 June 1999, 147 patients were diagnosed as having probable or definite DM/PM at the Veterans General Hospital, Taichung, Taiwan. We excluded four patients who had preceding neoplastic diseases diagnosed before DM/PM, then retrospectively reviewed the data of the remaining 143 patients and subgrouped the cases as four main types: primary idiopathic DM, primary idiopathic PM, juvenile DM/PM and amyopathic DM (ADM). We next performed univariate analysis using logistic regression to evaluate the possible predictive factors for malignancies, such as mean age at onset, gender, manifestations at onset, association with other connective tissue diseases, initial skin presentations, complications and laboratory data. Then we chose the significant factors for multivariate analysis by logistic regression, to determine the independent risk factors of malignancies in DM/PM patients. RESULTS: Among the 143 patients, DM was the most common type (64%), followed by ADM (14%), juvenile DM/PM (13%) and PM (10%). The mean age at onset overall was 42.4 years. Other connective tissue diseases were present in 22% of all patients, especially PM (50%) and juvenile DM/PM patients (28%). Internal malignancies were present in 13% of patients, and most were associated with DM. Nasopharyngeal carcinomas (NPCs) were the most common tumours. Patients with primary idiopathic DM, with an older age at onset, higher serum creatine phosphokinase levels and male gender, had more chance of developing concomitant malignancies. Those associated with complications, especially interstitial lung diseases, had a lower risk of associated neoplasia. In multivariate analysis, an older age at onset (odds ratio 9.10) and male gender (odds ratio 4.06) were associated with greater risk of developing malignancies. CONCLUSIONS: The two independent predictive factors for malignancy (P < 0.05) in patients with DM/PM were an older age at onset (> 45 years) and male gender. The primary idiopathic DM group was shown to have higher risk of developing internal malignancies, especially NPC. However, this was not identified as an independent predictive factor for concomitant neoplastic diseases in multivariate analysis. In addition, patients who had the complication of interstitial lung disease had a significantly lower frequency of malignancies (P < 0.001).     

Comparison of guidelines and consensus articles on the management of patients with acne with oral isotretinoin

Guidelines and consensus on the management of patients with acne aim to give evidence-based, expert-group recommendations. This review compares current guidelines and consensus articles to provide a compilation of recommendations on the treatment of acne with oral isotretinoin. Ten common, relevant, clinical questions are addressed, based on published recommendations, including the indications of isotretinoin, the proposed daily dose, the cumulative isotretinoin dose and the laboratory monitoring needed. Recommendations on special considerations are also addressed, including the timing of procedures and the question of an association of depression or inflammatory bowel disease with isotretinoin. A major limitation is the use of different classification systems for acne across guidelines. The recommended daily dose ranges from 0.3 to 0.5 mg/kg in the European guidelines to up to 1 mg/kg in the US guidelines. A specific duration of treatment of at least 6 months is only recommended in the European guidelines. All guidelines report the need of strict pregnancy prevention measures. The European, French and US guidelines recommend to monitor for symptoms of depression. Important clinical questions that are inconsistently addressed in guidelines include the age indication, the recommendation for a cumulative dose, the timing of procedures, the association of isotretinoin with IBD, the recommendation for preventing acne flares and for appropriate laboratory monitoring. These topics should be clearly included in the recommendations of guidelines as they are often raised in everyday clinical practice.     

Atopic dermatitis management: comparing the treatment patterns of dermatologists in Japan,U.S.A. and U.K

BACKGROUND: The incidence of atopic dermatitis (AD) is increasing worldwide. No large-scale study has previously compared the therapeutic management of this condition in different countries. OBJECTIVES: The purpose of this study was to determine the treatment preferences of dermatologists in Japan, the U.S.A. and the U.K., and investigate their relationship with certain factors pertaining to the physician and his practice. METHODS: A questionnaire was sent to all registered members of dermatological societies in Japan, the U.S.A. and the U.K. Responses were collated and statistical analysis performed using chi2, Mantel-Haenszel and Breslow heterogeneity tests. RESULTS: Three thousand six hundred and eighty-eight completed surveys were returned. U.S.A. and U.K. physicians were significantly more aggressive in prescribing systemic medications, such as steroids, antibiotics and immunosuppressants, compared with those in Japan. Japanese dermatologists also utilized topical steroids significantly less. The use of alternative remedies was highest in Japan. All three countries had a relatively high degree of optimism for topical immunosuppressants, but less so for other emerging therapies. CONCLUSIONS: Both similarities and differences in the therapy of AD exist in the three countries studied. Factors related to the physician, patient population and culture may influence these observations.     

皮肌炎/多发性肌炎患者伴发恶性肿瘤危险因素的研究与Logistic回归分析

目的探讨皮肌炎/多发性肌炎(DM/PM)患者伴发肿瘤的危险因素及两者间的关系。方法对DM/PM伴发恶性肿瘤者的临床表现、实验室辅助检查指标和治疗情况进行单因素和Logistic回归分析。结果恶性肿瘤年龄偏大者多见,好发于DM后1~2年,PM后1~5年。DM伴发恶性肿瘤的危险因素有中度日光性皮炎和/或皮肤瘙痒、中度咽喉部肌群受累(如吞咽困难、声嘶等)、重度颈部肌群受累(如抬头困难)、消瘦等;而PM则为轻度呼吸肌受累(如呼吸困难)。结论对出现中度日光性皮炎和/或皮肤瘙痒、消瘦、中度咽喉部肌群、重度颈部肌群和轻度呼吸肌受累的中老年DM/PM者尤其在发病2~5年内应警惕伴发的恶性肿瘤。