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Background: Urticarial vasculitis (UV) should be differentiated from chronic spontaneous urticaria (CSU) in patients initially presenting with recurrent wheals, although criteria for differential diagnosis remain ill-defined., Objectives: To set the goals, define criteria and unmet needs in UV diagnosis and differential diagnosis with CSU, and explore the possibility of coexistence of both diseases., Methods: Thirteen experts experienced in UV research participated in a Delphi survey of European Academy of Allergy and Clinical Immunology taskforce. This Delphi survey involved three rounds of anonymous responses to n = 32 questions with the aim to aggregate the experts' opinions and to achieve consensus. Urticaria specialists (n = 130, most from Urticaria Centers of Reference and Excellence) evaluated the consensus statements and recommendations in the fourth and final round., Results: The panel agreed that essential criteria to guide a skin biopsy in patients with recurrent wheals should include at least one of the following features: wheal duration >24 h, bruising/postinflammatory hyperpigmentation, and systemic symptoms. Leukocytoclasia and fibrin deposits were identified as a minimum set of UV histological criteria. As agreed by the panel members, CSU and normocomplementemic UV (NUV) may coexist in some patients., Conclusions: The use of established criteria for the diagnosis and differential diagnosis of UV in patients with recurrent wheals can help guide the diagnostic approach and prompt earlier treatment. Further studies should investigate whether CSU and NUV are different entities or part of a disease spectrum., (© 2023 The Authors. Clinical and Translational Allergy published by John Wiley & Sons Ltd on behalf of European Academy of Allergy and Clinical Immunology.)

While suboptimal pneumococcal vaccination rates have been reported in immunosuppressed patients with rheumatic diseases, data for patients with psoriasis (PsO) or atopic dermatitis (AD) are scarce. Pneumococcal vaccination in Germany is recommended in patients with certain comorbidities, immunosuppression, and/or aged 60 years or above. The aim of this multicenter cross-sectional study was to investigate the pneumococcal vaccination rate in patients with PsO compared to patients with AD and to evaluate patient perceptions. All patients completed a questionnaire on vaccination status and perceptions, patient and disease characteristics, as well as comorbidity. Medical records and vaccination certificates were reviewed. Over the whole cohort (n = 327 PsO (41.9% female), n = 98 AD (42.9% female)), 83.8% and 42.9% of PsO and AD patients, respectively, had an indication for pneumococcal vaccination due to immunosuppressive treatment. The pneumococcal vaccination rate was 14.4% and 10.2% in PsO and AD patients, respectively. The vaccination rate depended significantly on age, working status and presence of psoriatic arthritis. The most common reason for nonvaccination was lacking recommendation by physicians. Higher awareness, particularly for vaccination indication due to immunosuppression among dermatologists, general physicians, and patients, is warranted.

Eosinophilic fasciitis (EF) and generalized morphea (GM) are rare and difficult-to-treat sclerosing skin diseases which may occur in association with hematologic disorders. We present a 66-year-old man with EF and associated Waldenström macroglobulinemia who received combination therapy with rituximab (375mg/m2 every other week, gradually extended to every eight weeks), prednisolone (1.25-30mg/d), and methotrexate (7.5-15mg/w). Three months after rituximab initiation, his skin condition improved steadily accompanied by a significant improvement in joint mobility with only mild and transitory flares (observation period: 59 months under treatment with rituximab). To date, there are five case reports on rituximab treatment of EF/GM with an association to hypergammaglobulinemia in three of those cases. Therapy effected significant improvement in four patients. Our case adds to the hitherto limited evidence that rituximab may be a promising therapeutic strategy for EF/GM in association with hypergammaglobulinemia.

Background and Objective: Dermatophyte infections of the skin and nails are common worldwide and vary between geographical areas and over time. The aim of this study was to determine the epidemiological profile of dermatophytes in Germany with a focus on comparing children with adults., Patients and Methods: In this retrospective multicenter study, mycological dermatophyte culture results in the period 01/2014 to 12/2016 were analyzed according to identified pathogen, age and gender of patients, and type of disease., Results: Of 1,136 infections (children: n = 88, adults: n = 1,048), 50.8 % were clinically classified as onychomycosis, followed by tinea pedis (34.6 %), tinea corporis (16.2 %), tinea manus (16.2 %), tinea capitis (2.5 %), and tinea faciei (1.2 %). The most frequent pathogen was Trichophyton (T.) rubrum (78.6 %), followed by T. interdigitale (14.3 %), T. benhamiae (3.2 %), T. mentagrophytes (2.1 %), and Microsporum canis (1.7 %). The fungal spectrum differed particularly in tinea corporis and tinea capitis between children and adults with a more diverse pathogen spectrum in children. Trichophyton tonsurans was rarely identified as cause for tinea corporis (2.7 %) or tinea capitis (3.3 %)., Conclusions: Differences in pathogens and frequency of fungal infections between age groups should be considered for optimal selection of the appropriate therapeutic regimen., (© 2021 The Authors. Journal der Deutschen Dermatologischen Gesellschaft published by John Wiley & Sons Ltd on behalf of Deutsche Dermatologische Gesellschaft.)

Introduction: The COVID-19 pandemic dramatically disrupts health care around the globe. The impact of the pandemic on chronic urticaria (CU) and its management are largely unknown., Aim: To understand how CU patients are affected by the COVID-19 pandemic; how specialists alter CU patient management; and the course of CU in patients with COVID-19., Materials and Methods: Our cross-sectional, international, questionnaire-based, multicenter UCARE COVID-CU study assessed the impact of the pandemic on patient consultations, remote treatment, changes in medications, and clinical consequences., Results: The COVID-19 pandemic severely impairs CU patient care, with less than 50% of the weekly numbers of patients treated as compared to before the pandemic. Reduced patient referrals and clinic hours were the major reasons. Almost half of responding UCARE physicians were involved in COVID-19 patient care, which negatively impacted on the care of urticaria patients. The rate of face-to-face consultations decreased by 62%, from 90% to less than half, whereas the rate of remote consultations increased by more than 600%, from one in 10 to more than two thirds. Cyclosporine and systemic corticosteroids, but not antihistamines or omalizumab, are used less during the pandemic. CU does not affect the course of COVID-19, but COVID-19 results in CU exacerbation in one of three patients, with higher rates in patients with severe COVID-19., Conclusions: The COVID-19 pandemic brings major changes and challenges for CU patients and their physicians. The long-term consequences of these changes, especially the increased use of remote consultations, require careful evaluation., (© 2020 The Authors. Allergy published by European Academy of Allergy and Clinical Immunology and John Wiley & Sons Ltd.)

Background: Chronic urticaria (CU) is a common disease which represents a considerable burden for many patients. The current urticaria guideline describes the evidence-based diagnosis and treatment of CU. In addition, however, questions often arise in everyday practice that are not addressed by the guideline., Methods: In May 2020, a digital meeting with German urticaria experts was held, in which practical aspects of CU treatment were discussed and supporting aids for everyday clinical treatment formulated. The resulting advice in this document focus on practical questions and the available literature and experiences of the participants., Results: The diagnosis of CU can be made in a short time by means of a thorough anamnesis, a physical examination, and a basic laboratory chemical diagnosis. For this purpose, practical recommendations for everyday practice are given in this paper. An extended diagnosis is only indicated in a few cases and should always be carried out in parallel with an effective therapy. In general, CU should always be treated in the same way, regardless of whether wheals, angioedema or both occur. Symptomatic therapy should be carried out according to the treatment steps recommended by the guidelines. This publication provides practical advice on issues in everyday practice, such as the procedure in the current coronavirus disease 2019 (COVID-19) pandemic, the cardiac risk under higher dosed H1 antihistamines, the self-administration of omalizumab as well as vaccination under omalizumab therapy. In addition to treatment recommendations, topics such as documentation in the practice and family planning with urticaria will be discussed., Discussion: These supporting treatment recommendations serve as an addendum to the current CU guideline and provide support in dealing with CU patients in everyday practice. The aim is to ensure that patients suffering from CU achieve complete freedom of symptoms with the help of an optimal therapy., Supplementary Information: The online version of this article (10.1007/s40629-021-00162-w) contains supplementary material, which is available to authorized users., Competing Interests: Conflict of interestA. Bauer received fees for lectures and advisory boards of Novartis, Biofrontera, Shire/Takeda and Leo Pharma as well as research support from Novartis during the last three years. She has been involved in clinical trials with drugs from Novartis, Lilly, Regeneron, Sanofi, Leo Pharma, and Genetec. H. Dickel received fees from Novartis for lectures and advisory boards. He is also involved as PI in clinical studies with preparations from Novartis and Leo Pharma. T. Jakob has received fees for lectures and advisory boards from Novartis, ALK-Abéllo, Allergopharma, Bencard, Thermo Fischer and Celegene, as well as research support from Novartis and ALK-Abéllo. He is also a member of the extended board of the German Society for Allergology and Clinical Immunology and editor of the Allergo Journal and Allergo Journal International. A. Kleinheinz received financial support from Novartis, LEO, Sanofi, Galderma, Abbvie, Janssen, Medac, Almirall, Amgen, Celgene, Genentech, GSK, Lilly, Parexel, Pfizer and proinnovera as a speaker and for participation in clinicial studies. U. Lippert received fees for lectures or advisory boards lasting several hours in the last three years from Novartis, Shire/Takeda, ALK-Abéllo, Meda Pharma GmbH and Sanofi. She received research grants and consulting fees from Novartis and travel expenses from Novartis and Shire/Takeda. She has also been involved as LKP and PI in clinical trials with preparations from Regeneron, Sanofi and Novartis. M. Metz received fees as speaker and/or consultant for Amgen, Aralez, argenx, Moxie, Novartis, Roche, Sanofi and Uriach. S. Schliemann is an investigator in studies by Novartis Pharma GmbH, Sanofi GmbH, Eli Lilly and Company Limited, LEO Pharma GmbH, Pierre Fabre Dermo-Cosmetique, has received lecture fees from Novartis Pharma GmbH, Sanofi GmbH, LEO Pharma GmbH and HAL Allergy GmbH and research support from Novartis Pharma GmbH. U. Schwichtenberg: AbbVie Deutschland GmbH, Almirall Hermal GmbH, Amgen, Beiersdorf Derma Medical GmbH, Celgene GmbH, Dermapharm GmbH, Janssen Cilag GmbH, Johnson & Johnson GmbH, LEO Pharma GmbH, L’Oréal GmbH, MEDA Pharma GmbH, Merz Pharmaceuticals GmbH, MSD SHARP & DOHME GmbH, Novartis Pharma GmbH, and Pfizer GmbH, Sanofi-Aventis Deutschland GmbH—fees for lectures, advisory boards, study participation, Medical Project Design GmbH Shareholder and Managing Director, Professional Association of German Dermatologists Bremen State Chairman, Association of Statutory Health Insurance Physicians Bremen Member of the Assembly of Representatives and Chairman of the Honorarium Distribution Scale Committee, Member of the Finance Committee. P. Staubach is or recently was a speaker and/or advisor for and/or has received research funding from AbbVie, Allergika, Almirall, Amgen, Beiersdorf, Biocryst, Biogen Idec, BMS, Boehringer-Ingelheim, Celgene, CSL-Behring, Eli-Lilly, Galderma, Hexal, Janssen, Klosterfrau, LEO-Pharma, LETI-Pharma, L’Oreal, Novartis, Octapharma, Pfizer, Pflüger, Pharming, Regeneron, Shire, Takeda, Regeneron, Sanofi-Genzyme und UCB Pharma. E. Valesky has received honours for lectures and consultancy work for the companies Novartis, Bencard, Alk-Abello, JUZO, Kreussler and Sigvaris over the last three years. N. Wagner received fees for lectures or advisory boards lasting several hours in the last three years from Novartis, Shire/Takeda, ALK-Abéllo, Allergopharma, Abbvie, as well as research support from Novartis. In addition, she was involved as PI in clinical trials with Novartis products. B. Wedi received fees for lectures or advisory boards lasting several hours over the last three years from Novartis, Shire/Takeda, ALK-Abéllo, Bencard, CSL-Behring, HAL-Allergy and Leo Pharma as well as research support from Shire/Takeda. She has also been involved as LKP or PI in clinical trials with Novartis products. M. Maurer is or recently was a speaker and/or advisor for and/or has received research funding from Allakos, Amgen, Aralez, ArgenX, AstraZeneca, Celldex, Centogene, CSL Behring, FAES, Genentech, GIInnovation, Innate Pharma, Kyowa Kirin, Leo Pharma, Lilly, Menarini, Moxie, Novartis, Roche, Sanofi/Regeneron, Third HarmonicBio, UCB, and Uriach., (© The Author(s) 2021.)

Background/objectives: Para-phenylenediamine, a dye frequently added to henna tattoos to create the black color, is a potent contact allergen. Severe contact dermatitis may arise within days even after the first application. Our objective was to develop a method for rapid and complete removal of para-phenylenediamine-containing black henna tattoos from the skin, an important problem many physicians are confronted with, but for which no simple method exists., Methods: A database search revealed polyethylene glycol 400, described in removal of ortho-phenylenediamine from contaminated skin. We therefore investigated its use in removal of the structurally related ortholog para-phenylenediamine present in black henna tattoos., Results: A protocol was established involving repeated cycles of rinsing of the skin with polyethylene glycol 400 solution. In 5 patients, one of whom had already developed a severe blistering contact dermatitis and another a mild erosive dermatitis, black henna tattoos were successfully removed. Removal was completed in a single session of 1 hour or less, depending on tattoo size, with a maximum of 6 rinse cycles., Conclusions: We provide a simple and safe method for rapid and effective removal of black henna tattoos. This procedure requires no special equipment and can be applied in virtually any setting., (© 2020 Wiley Periodicals LLC.)

Background: Pityriasis rubra pilaris (PRP) is a rare chronic inflammatory dermatosis. Treatment is challenging and mostly based on scarce empirical evidence from large case series., Objective: To investigate patient characteristics and therapy outcomes in patients with PRP., Materials & Methods: In this retrospective study, patients with PRP treated at the Department of Dermatology, University Medical Center Göttingen, between 01/2005 and 10/2018, were analysed according to patient and disease characteristics and treatment experience., Results: PRP was diagnosed in 28 patients with a mean age of 57.3 years at onset; 20 were men (71.4%) and eight women (28.6%). Of all the patients, 89.3% had comorbid diseases; metabolic disorders were particularly frequent. Seventy-eight treatment courses were identified with acitretin as the most common therapy (28.2%). Excellent response was noted in 71.4% of treatment courses with methotrexate, followed by cyclosporine (27.3%) and acitretin (19.0%). Among biologics, ustekinumab (excellent response: 75.0%) and infliximab (50.0%) were particularly effective., Conclusion: This study is one of the few comprehensive larger case series of patients with PRP. Acitretin may be effective, particularly for type I PRP, and other retinoids, methotrexate or cyclosporine might also be considered early in the therapeutic algorithm. Moreover, our data adds to the growing evidence that biologics are effective and safe for the treatment of PRP.

Background: Hereditary angioedema (HAE) is a group of genetic diseases characterized by recurrent, painful and potentially lethal tissue swelling. The most common form results from mutations in the SERPING1 gene, leading to reduced function of complement 1 inhibitor (C1-INH). Rarer forms with normal C1-INH may arise from mutations in the coagulation factor F12 gene, but mostly the genetic background is unknown. Recently, a novel HAE mutation in the plasminogen (PLG) gene was shown., Patients and Methods: We analyzed the various clinical manifestations of HAE in 14 related patients using clinical data, biochemical analysis for C1-INH and C4 as well as gene sequencing., Results: Patients' symptoms were assigned to two different forms of HAE. In ten patients suffering from swelling of the lips or tongue but not of the extremities, a mutation in the PLG gene (c.988A>G) was found whereas in the only four patients with swelling of the gastrointestinal tract and extremities, a mutation in the SERPING1 gene (c.1480C>T) was identified. In two cases this was additional to PLG c.988A>G., Conclusions: This unique finding of two different HAE-specific mutations in a large family not only explains the divergent phenotypes but also supports a genotype-phenotype correlation showing that abdominal attacks and swelling of the extremities are common with HAE-C1-INH but unusual with HAE-PLG., (© 2020 Deutsche Dermatologische Gesellschaft (DDG). Published by John Wiley & Sons Ltd.)

Background and Objectives: Proper management of infantile scabies is indispensable in order to prevent prolonged duration and spread of the disease. Data are still lacking that support topical treatment with permethrin 5 % cream in infants under two months of age, and application remains off-label for this age group., Patients and Methods: We identified infants younger than two months who suffered from scabies in order to evaluate the safety and efficacy of topical treatment with permethrin cream in this age group. Diagnosis of scabies was based on the typical symptoms and pathognomonic features as determined with dermoscopy. We analyzed the efficacy and safety of the therapies that were applied., Results: Seven scabies patients under two months of age were treated with permethrin 5 % cream. Topical therapy was repeated up to three times in four patients due to incomplete resolution or recurrence of skin lesions., Conclusions: Permethrin therapy was well tolerated in all seven infants, even when conducted several times. Our results confirm that the use of permethrin 5 % cream in children younger than two months of age is safe., (© 2019 Deutsche Dermatologische Gesellschaft (DDG). Published by John Wiley & Sons Ltd.)