Publications by authors named "Knut Brockow"

160 Publications

Health-related quality of life and influencing factors in adults with non-advanced mastocytosis - a cross-sectional study and qualitative approach.

J Allergy Clin Immunol Pract 2021 May 6. Epub 2021 May 6.

Department of Dermatology and Allergy, Technical University of Munich, School of Medicine, Munich, Germany.

Background: Mastocytosis comprises a heterogeneous group of disorders characterized by an accumulation of mast cells in one or more organs. Symptoms range from mild complaints to severe and life-threatening events. Impact on quality of life seems to vary widely, but influencing factors are poorly understood so far.

Objective: This study examines impairments, psychological burden, health-related quality of life (HRQoL) and possible influencing factors in patients with mastocytosis.

Methods: In semi-structured telephone interviews, patients provided information on impairments in everyday life and psychological burden caused by mastocytosis. HRQoL was measured using the Mastocytosis Quality of Life Questionnaire (MC-QoL). Clinical data were collected from patient files.

Results: A total of 101 adult patients with mastocytosis (74.3% women; mean age: 47.7±13.5 years) were included. Half of the interviewed patients (50.6%) reported disease-related impairments in everyday life and 42.4% stated a psychological burden. MC-QoL scores showed a broad distribution with a mean total score at a 'mild' impairment level (mean total score: 34.7±22.5). One third of patients felt moderately (22.8%) or severely (13.9%) impaired, whereas one third reported no impairment at all (30.7%). Symptoms of mast cell activation and perceived food intolerance had the highest impact on HRQoL. Higher age, higher BMI, higher tryptase level and longer duration of symptoms, as well as current drug therapy and pathological bone density were each associated with reduced HRQoL.

Conclusion: A high level of suffering and strong associations between impairments and symptom-related factors indicate the importance of addressing patients' concerns and adequate symptom management in mastocytosis.
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http://dx.doi.org/10.1016/j.jaip.2021.04.059DOI Listing
May 2021

Practical handling of allergic reactions to COVID-19 vaccines: A position paper from German and Austrian Allergy Societies AeDA, DGAKI, GPA and ÖGAI.

Allergo J Int 2021 Apr 19:1-17. Epub 2021 Apr 19.

Allergology and Immunology, Department of Dermatology, Venereology and Allergology, Charité-University Medicine Berlin, Berlin, Germany.

Background: For the preventive treatment of the 2019 coronavirus disease (COVID-19) an unprecedented global research effort studied the safety and efficacy of new vaccine platforms that have not been previously used in humans. Less than one year after the discovery of the severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) viral sequence, these vaccines were approved for use in the European Union (EU) as well as in numerous other countries and mass vaccination efforts began. The so far in the EU approved mRNA vaccines BNT162b2 and mRNA-1273 are based on similar lipid-based nanoparticle carrier technologies; however, the lipid components differ. Severe allergic reactions and anaphylaxis after COVID-19 vaccination are very rare adverse events but have drawn attention due to potentially lethal outcomes and have triggered a high degree of uncertainty.

Methods: Current knowledge on anaphylactic reactions to vaccines and specifically the new mRNA COVID-19 vaccines was compiled using a literature search in Medline, PubMed, as well as the national and international study and guideline registries, the Cochrane Library, and the Internet, with special reference to official websites of the World Health Organization (WHO), US Centers for Disease Control and Prevention (CDC), Robert Koch Institute (RKI), and Paul Ehrlich Institute (PEI).

Results: Based on the international literature and previous experience, recommendations for prophylaxis, diagnosis and therapy of these allergic reactions are given by a panel of experts.

Conclusion: Allergy testing is not necessary for the vast majority of allergic patients prior to COVID-19 vaccination with currently licensed vaccines. In case of allergic/anaphylactic reactions after vaccination, allergy workup is recommended, as it is for a small potential risk population prior to the first vaccination. Evaluation and approval of diagnostic tests should be done for this purpose.
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http://dx.doi.org/10.1007/s40629-021-00165-7DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC8054127PMC
April 2021

Im Zusammenhang mit COVID-19-Infektionen beschriebene Hautmanifestationen.

J Dtsch Dermatol Ges 2021 04;19(4):530-535

Klinik und Poliklinik für Dermatologie und Allergologie am Biederstein, Fakultät für Medizin, Technische Universität München.

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http://dx.doi.org/10.1111/ddg.14353_gDOI Listing
April 2021

Reply to: "Include desensitization to radiocontrast media in the diagnostic algorithm".

Authors:
Knut Brockow

Allergy 2021 04;76(4):1304-1305

Department of Dermatology and Allergy Biederstein, School of Medicine, Technical University of Munich, Munich, Germany.

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http://dx.doi.org/10.1111/all.14592DOI Listing
April 2021

COVID-19 Vaccination in Mastocytosis: Recommendations of the European Competence Network on Mastocytosis (ECNM) and American Initiative in Mast Cell Diseases (AIM).

J Allergy Clin Immunol Pract 2021 Apr 5. Epub 2021 Apr 5.

Department of Hematological Biology, Pitié-Salpêtrière Hospital, Paris Sorbonne University, Paris, France.

Mastocytosis is a neoplasm characterized by an accumulation of mast cells in various organs and increased risk for severe anaphylaxis in patients with concomitant allergies. Coronavirus disease 2019 (COVID-19) is a pandemic that is associated with a relatively high rate of severe lung disease and mortality. The mortality is particularly high in those with certain comorbidities and increases with age. Recently, several companies have developed an effective vaccination against COVID-19. Although the reported frequency of severe side effects is low, there is an emerging discussion about the safety of COVID-19 vaccination in patients with severe allergies and mastocytosis. However, even in these patients, severe adverse reactions are rare. We therefore recommend the broad use of COVID-19 vaccination in patients with mastocytosis on a global basis. The only well-established exception is a known or suspected allergy against a constituent of the vaccine. Safety measures, including premedication and postvaccination observation, should be considered in all patients with mastocytosis, depending on the individual personal risk and overall situation in each case. The current article provides a summary of published data, observations, and expert opinion that form the basis of these recommendations.
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http://dx.doi.org/10.1016/j.jaip.2021.03.041DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC8019658PMC
April 2021

Management of anaphylaxis due to COVID-19 vaccines in the elderly.

Allergy 2021 Apr 2. Epub 2021 Apr 2.

Regional Ministry of Health of Andalusia, Seville, Spain.

Older adults, especially men and/or those with diabetes, hypertension and/or obesity, are prone to severe COVID-19. In some countries, older adults, particularly those residing in nursing homes, have been prioritised to receive COVID-19 vaccines due to high risk of death. In very rare instances,the COVID-19 vaccines can induce anaphylaxis, and the management of anaphylaxis in older people should be considered carefully. An ARIA-EAACI-EuGMS (Allergic Rhinitis and its Impact on Asthma, European Academy of Allergy and Clinical Immunology, and European Geriatric Medicine Society)Working Group has proposed some recommendations for older adults receiving the COVID-19 vaccines. Anaphylaxis to COVID-19 vaccines is extremely rare (from 1 per 100,000 to 5 per million injections). Symptoms are similar in younger and older adults but they tend to be more severe in the older patients. Adrenaline is the mainstay treatment and should be readily available. A flowchart is proposed to manage anaphylaxis in the older patients.
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http://dx.doi.org/10.1111/all.14838DOI Listing
April 2021

Molecular Background, Clinical Features and Management of Pediatric Mastocytosis: Status 2021.

Int J Mol Sci 2021 Mar 4;22(5). Epub 2021 Mar 4.

Division of Hematology and Hemostaseology, Department of Internal Medicine I, Medical University of Vienna, 1090 Vienna, Austria.

Pediatric mastocytosis is a heterogeneous disease characterized by accumulation of mast cells in the skin and less frequently in other organs. Somatic or germline mutations in the proto-oncogene are detected in most patients. Cutaneous mastocytosis is the most common form of the disease in children. In the majority of cases, skin lesions regress spontaneously around puberty. However, in few patients, mastocytosis is not a self-limiting disease, but persists into adulthood and can show signs of systemic involvement, especially when skin lesions are small-sized and monomorphic. Children with mastocytosis often suffer from mast cell mediator-related symptoms. Severe hypersensitivity reactions can also occur, mostly in patients with extensive skin lesions and blistering. In a substantial number of these cases, the triggering factor of anaphylaxis remains unidentified. Management of pediatric mastocytosis is mainly based on strict avoidance of triggers, treatment with H1 and H2 histamine receptor blockers, and equipment of patients and their families with epinephrine auto-injectors for use in severe anaphylactic reactions. Advanced systemic mastocytosis occurs occasionally. All children with mastocytosis require follow-up examinations. A bone marrow investigation is performed when advanced systemic mastocytosis is suspected and has an impact on therapy or when cutaneous disease persists into adulthood.
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http://dx.doi.org/10.3390/ijms22052586DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7961542PMC
March 2021

Mediator-Related Symptoms and Anaphylaxis in Children with Mastocytosis.

Int J Mol Sci 2021 Mar 7;22(5). Epub 2021 Mar 7.

Department of Internal Medicine I, Division of Hematology & Hemostaseology, Medical University of Vienna, 1090 Vienna, Austria.

Mastocytosis is characterized by the pathological accumulation of mast cells (MC) in various organs. In these patients, MC may degranulate and thereby contribute to clinical symptoms, especially when a concomitant allergy is present. However, MC activation can not only be induced by high-affinity receptors for IgE, but also by anaphylatoxins, neuropeptides, IgG immune complexes, complement-components, drugs, products of bacteria or parasites, as well as physical factors such as heat, cold, vibration, stress, sun, or physical effort. Symptoms due to mediators released by activated MC may develop in adults suffering from systemic mastocytosis, but also evolve in children who usually have cutaneous mastocytosis (CM). Clinically, CM is otherwise characterized by typical brown, maculopapular skin lesions or mastocytoma associated with a positive Darier's sign. Pruritus and flushing are common and blistering may also be recorded, especially in diffuse CM (DCM). Pediatric patients with mastocytosis may also have gastrointestinal, respiratory, and neurologic complaints. Although anaphylaxis is not a typical finding, pediatric patients with massive skin involvement and high tryptase levels have a relatively high risk to develop anaphylaxis. This paper reviews MC mediator-related symptoms and anaphylaxis in children with mastocytosis, with special emphasis on risk factors, triggers, and management.
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http://dx.doi.org/10.3390/ijms22052684DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7962052PMC
March 2021

Gelatin-Containing Vaccines for Varicella, Zoster, Measles, Mumps, and Rubella Induce Basophil Activation in Patients with Alpha-Gal Syndrome.

Int Arch Allergy Immunol 2021 Mar 18:1-7. Epub 2021 Mar 18.

Department of Dermatology and Allergy Biederstein, School of Medicine, Technical University Munich, Munich, Germany.

Background: The alpha-gal syndrome (AGS) describes a new type I allergy entity to the carbohydrate epitope galactose-α-1,3-galactose (alpha-gal), which is mainly found in mammalian food products (e.g., beef, pork, and venison). Apart from meat products, alpha-gal can also be found in products containing gelatin of bovine or porcine origin. Recent case reports pointed to severe anaphylaxis in patients suffering from AGS after vaccination with vaccines containing hydrolyzed gelatin. It was the objective of this study to evaluate if basophil activation tests (BATs) performed with such vaccines were positive in patients with AGS.

Methods: BAT was performed with different dilutions of a gelatin-containing measles, mumps, and rubella (MMR) live vaccine; an attenuated varicella (V) vaccine; an attenuated V-zoster (VZ) vaccine; a MMR live vaccine not containing gelatin (non-gelatin MMR vaccine) in 2 patients with confirmed AGS, 2 patients highly suspicious for AGS, and 2 healthy individuals without any previous medical history for allergies.

Results: All patients showed strongly positive results for all gelatin-containing vaccines (MMR vaccine, V vaccine, and VZ vaccine). Non-gelatin MMR vaccine was negative. The 2 healthy controls did not show any basophil activation.

Conclusions: Gelatin-containing vaccines should be administered with caution or avoided in patients with AGS because of their high potential to activate basophils indicating a risk for anaphylaxis. Also, BAT is a useful additional tool when it comes to screening for potentially high-risk alpha-gal-containing drugs.
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http://dx.doi.org/10.1159/000514263DOI Listing
March 2021

Leitlinie zu Akuttherapie und Management der Anaphylaxie - Update 2021: S2k-Leitlinie der Deutschen Gesellschaft für Allergologie und klinische Immunologie (DGAKI), des Ärzteverbands Deutscher Allergologen (AeDA), der Gesellschaft für Pädiatrische Allergologie und Umweltmedizin (GPA), der Deutschen Akademie für Allergologie und Umweltmedizin (DAAU), des Berufsverbands der Kinder- und Jugendärzte (BVKJ), der Gesellschaft für Neonatologie und Pädiatrische Intensivmedizin (GNPI), der Deutschen Dermatologischen Gesellschaft (DDG), der Österreichischen Gesellschaft für Allergologie und Immunologie (ÖGAI), der Schweizerischen Gesellschaft für Allergologie und Immunologie (SGAI), der Deutschen Gesellschaft für Anästhesiologie und Intensivmedizin (DGAI), der Deutschen Gesellschaft für Pharmakologie (DGP), der Deutschen Gesellschaft für Pneumologie und Beatmungsmedizin (DGP), der Patientenorganisation Deutscher Allergie- und Asthmabund (DAAB) und der Arbeitsgemeinschaft Anaphylaxie - Training und Edukation (AGATE).

Allergo J 2021 12;30(1):20-49. Epub 2021 Feb 12.

Klinik f. Dermatologie und Allergologie am Biederstein, Biedersteiner Str. 29, 80802 München, Germany.

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http://dx.doi.org/10.1007/s15007-020-4750-0DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7878028PMC
February 2021

Anaphylaxie-Leitlinie: Update 2021.

Allergo J 2021 12;30(1). Epub 2021 Feb 12.

Klinik f. Dermatologie und Allergologie am Biederstein, Biedersteiner Str. 29, 80802 München, Germany.

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http://dx.doi.org/10.1007/s15007-021-4757-1DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7878021PMC
February 2021

Clinical Impact of Skin Lesions in Mastocytosis: A Multicenter Study of the European Competence Network on Mastocytosis.

J Invest Dermatol 2021 Feb 11. Epub 2021 Feb 11.

Department of Internal Medicine IV, Oncology, Hematology, Hemostaseology, University Halle (Saale), Halle (Saale), Germany.

Mastocytosis is a rare neoplasm characterized by the expansion and accumulation of mast cells in various organ systems. Systemic mastocytosis (SM) may or may not present with cutaneous lesions. To examine the frequency and clinical impact of cutaneous involvement, data on 1,510 patients with mastocytosis collected in the registry of the European Competence Network on Mastocytosis were analyzed. Cutaneous involvement was found in 1,195 of 1,510 patients (79.1%). Of these, 286 had cutaneous mastocytosis, and 721 had SM with skin involvement. Adult patients with skin involvement who did not have a bone marrow examination (n = 188) were defined as having mastocytosis in the skin. In 315 patients, SM without skin involvement was found. The percentage of cases with cutaneous involvement was higher in indolent SM (100%) and smoldering SM (87.9%) compared to aggressive SM (46.8%) or mast cell leukemia (38.5%). After a median follow-up of 5.6 years, no patient with cutaneous mastocytosis had died, but 2.6% of the patients with mastocytosis in the skin, 5.7% of the patients with SM with skin involvement, and 28.95% of the patients with SM without skin involvement had died. Overall survival was longer in patients with skin involvement (cutaneous mastocytosis and/or mastocytosis in the skin and/or SM with skin involvement) than in patients with SM without skin involvement (P < 0.0001). These data argue for a thorough examination of both the skin and bone marrow in adult patients with mastocytosis.
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http://dx.doi.org/10.1016/j.jid.2020.12.030DOI Listing
February 2021

EAACI Biologicals Guidelines-dupilumab for children and adults with moderate-to-severe atopic dermatitis.

Allergy 2021 04 27;76(4):988-1009. Epub 2020 Dec 27.

Department of Clinical Immunology, University of Wroclaw, Wroclaw, Poland.

Atopic dermatitis imposes a significant burden on patients, families and healthcare systems. Management is difficult, due to disease heterogeneity, co-morbidities, complexity in care pathways and differences between national or regional healthcare systems. Better understanding of the mechanisms has enabled a stratified approach to the management of atopic dermatitis, supporting the use of targeted treatments with biologicals. However, there are still many issues that require further clarification. These include the definition of response, strategies to enhance the responder rate, the duration of treatment and its regimen (in the clinic or home-based), its cost-effectiveness and long-term safety. The EAACI Guidelines on the use of dupilumab in atopic dermatitis follow the GRADE approach in formulating recommendations for each outcome and age group. In addition, future approaches and research priorities are discussed.
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http://dx.doi.org/10.1111/all.14690DOI Listing
April 2021

A Challenge for Allergologist: Application of Allergy Diagnostic Methods in Mast Cell Disorders.

Int J Mol Sci 2021 Feb 1;22(3). Epub 2021 Feb 1.

Department of Internal Medicine I, Division of Hematology and Hemostaseology, Medical University of Vienna, 1090 Vienna, Austria.

Primary and secondary mast cell activation syndromes (MCAS) can occur in patients with mastocytosis. During the past few years our knowledge about the pathogenesis and disease-triggering mechanisms in MCAS and mastocytosis have increased substantially. Whereas mastocytosis is characterized by an accumulation of neoplastic (clonal) mast cells (MC) in various organ systems, MCAS is defined by a massive and systemic activation of these cells. Mast cells are crucial effector cells in allergic diseases, thus their elevated number and activation can cause severe anaphylactic reactions and MCAS in patients with mastocytosis. However, these cells may also degranulate spontaneously or degranulate in response to non-allergic triggers leading to clinical symptoms. In mastocytosis patients, such symptoms may lead to the diagnosis of a primary MCAS. The diagnosis of a concomitant allergy in mastocytosis patients is challenging. In these patients, a mixed form (primary and secondary) of MCAS may be diagnosed. These patients may also suffer from life-threatening anaphylactic reactions when exposed to allergens. In these cases, the possibility of severe side effects of in vivo provocations can sometimes also limit diagnostic evaluations. In the current article, we discuss the diagnosis and management of patients suffering from mastocytosis and concomitant MCAS, with special emphasis on novel diagnostic tests and management, including allergen microarrays, recombinant allergen analysis, basophil activation tests, optimal prophylaxis, and specific therapies.
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http://dx.doi.org/10.3390/ijms22031454DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7867197PMC
February 2021

Guideline (S2k) on acute therapy and management of anaphylaxis: 2021 update: S2k-Guideline of the German Society for Allergology and Clinical Immunology (DGAKI), the Medical Association of German Allergologists (AeDA), the Society of Pediatric Allergology and Environmental Medicine (GPA), the German Academy of Allergology and Environmental Medicine (DAAU), the German Professional Association of Pediatricians (BVKJ), the Society for Neonatology and Pediatric Intensive Care (GNPI), the German Society of Dermatology (DDG), the Austrian Society for Allergology and Immunology (ÖGAI), the Swiss Society for Allergy and Immunology (SGAI), the German Society of Anaesthesiology and Intensive Care Medicine (DGAI), the German Society of Pharmacology (DGP), the German Respiratory Society (DGP), the patient organization German Allergy and Asthma Association (DAAB), the German Working Group of Anaphylaxis Training and Education (AGATE).

Allergo J Int 2021 28;30(1):1-25. Epub 2021 Jan 28.

Department Dermatology and Allergology Biederstein, Technical University Munich, Biedersteiner Straße 29, 80802 Munich, Germany.

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http://dx.doi.org/10.1007/s40629-020-00158-yDOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7841027PMC
January 2021

Practical recommendations for the allergological risk assessment of the COVID-19 vaccination - a harmonized statement of allergy centers in Germany.

Allergol Select 2021 26;5:72-76. Epub 2021 Jan 26.

Allergy and Asthma Center Westend, Berlin, Germany.

Severe allergic reactions to vaccines are very rare. Single severe reactions have occurred worldwide after vaccination with the new mRNA-based COVID-19 vaccines. PEG2000 is discussed as a possible trigger. We provide guidance on risk assessment regarding COVID-19 vaccination in patients with allergic diseases and suggest a standardized, resource-oriented diagnostic and therapeutic procedure. Reports of severe allergic reactions in the context of COVID-19 vaccination can be made via www.anaphylaxie.net using an online questionnaire.
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http://dx.doi.org/10.5414/ALX02225EDOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7841415PMC
January 2021

Cytogenetic and molecular aberrations and worse outcome for male patients in systemic mastocytosis.

Theranostics 2021 1;11(1):292-303. Epub 2021 Jan 1.

Department of Dermatology and Venereology, Allergy Centrr, Kepler University Hospital, Linz, Austria.

In systemic mastocytosis (SM), the clinical features and survival vary greatly. Patient-related factors determining the outcome in SM are largely unknown. We examined the impact of sex on the clinical features, progression-free survival (PFS), and overall survival (OS) in 3403 patients with mastocytosis collected in the registry of the European Competence Network on Mastocytosis (ECNM). The impact of cytogenetic and molecular genetic aberrations on sex differences was analyzed in a subset of patients. Of all patients enrolled, 55.3% were females. However, a male predominance was found in a subset of advanced SM (AdvSM) patients, namely SM with an associated hematologic neoplasm (SM-AHN, 70%; 0.001). Correspondingly, organomegaly (male: 23% female: 13%, 0.007) was more, whereas skin involvement (male: 71% female: 86%, 0.001) was less frequent in males. In all patients together, OS ( 0.0001) was significantly inferior in males, and also within the WHO sub-categories indolent SM, aggressive SM (ASM) and SM-AHN. PFS was significantly ( 0.0002) worse in males when all patients were grouped together; due to low numbers of events, this significance persisted only in the subcategory smoldering SM. Finally, prognostically relevant cytogenetic abnormalities (10% 5%, 0.006) or molecular aberrations (// profile; 63% 40%, 0.003) were more frequently present in males. Male sex has a major impact on clinical features, disease progression, and survival in mastocytosis. Male patients have an inferior survival, which seems related to the fact that they more frequently develop a multi-mutated AdvSM associated with a high-risk molecular background.
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http://dx.doi.org/10.7150/thno.51872DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7681091PMC
January 2021

Skin manifestations reported in association with COVID-19 infection.

J Dtsch Dermatol Ges 2021 04 30;19(4):530-534. Epub 2020 Dec 30.

Department of Dermatology and Allergy Biederstein, School of Medicine, Technical University of Munich, Munich, Germany.

The prevalence of all cutaneous manifestations directly associated with COVID-19 infection is unknown, but the number of reports is rapidly increasing and provisional knowledge is rapidly evolving. Skin manifestations reported can be classified into (1) manifestations unspecifically indicating possible infectious diseases, i.e. maculopapular exanthem, urticaria and erythema multiforme, and (2) manifestations more specifically indicating COVID-19 infection, i.e. varicella-like, livedo reticularis or chilblain-like eruptions. The latter appear to be associated with thrombovascular events and vascular pathologies.
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http://dx.doi.org/10.1111/ddg.14353DOI Listing
April 2021

Scoring the Risk of Having Systemic Mastocytosis in Adult Patients with Mastocytosis in the Skin.

J Allergy Clin Immunol Pract 2021 Apr 23;9(4):1705-1712.e4. Epub 2020 Dec 23.

Division of Allergy and Clinical Immunology, Department of Medicine, University of Salerno, Salerno, Italy.

Background: Mastocytosis in adults often presents with skin lesions. A bone marrow biopsy is necessary to confirm or exclude the presence of systemic mastocytosis (SM) in these cases. When a bone marrow biopsy is not performed, the provisional diagnosis is mastocytosis in the skin (MIS). No generally accepted scoring system has been established to estimate the risk of SM in these patients.

Objective: To develop a risk score to predict SM in adults with MIS.

Methods: We examined 1145 patients with MIS from the European Competence Network on Mastocytosis Registry who underwent a bone marrow biopsy. A total of 944 patients had SM and 201 patients had cutaneous mastocytosis; 63.7% were female, and 36.3% were male. Median age was 44 ± 13.3 years. The median serum tryptase level amounted to 29.3 ± 81.9 ng/mL. We established a multivariate regression model using the whole population of patients as a training and validation set (bootstrapping). A risk score was developed and validated with receiver-operating curves.

Results: In the multivariate model, the tryptase level (P < .001), constitutional/cardiovascular symptoms (P = .014), and bone symptoms/osteoporosis (P < .001) were independent predictors of SM (P < .001; sensitivity, 90.7%; specificity, 69.1%). A 6-point risk score was established (risk, 10.7%-98.0%) and validated.

Conclusions: Using a large data set of the European Competence Network on Mastocytosis Registry, we created a risk score to predict the presence of SM in patients with MIS. Although the score will need further validation in independent cohorts, our score seems to discriminate safely between patients with SM and with pure cutaneous mastocytosis.
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http://dx.doi.org/10.1016/j.jaip.2020.12.022DOI Listing
April 2021

Predominant patterns of β-lactam hypersensitivity in a single German Allergy Center: exanthem induced by aminopenicillins, anaphylaxis by cephalosporins.

Allergy Asthma Clin Immunol 2020 Nov 17;16(1):102. Epub 2020 Nov 17.

Department of Dermatology and Allergy, Allergy Center Mainfranken, University Hospital Würzburg, 97080, Würzburg, Germany.

Background: Penicillins and other β-lactam antibiotics are the most common elicitors of allergic drug reaction. However, data on the pattern of clinical reaction types elicited by specific β-lactams are scarce and inconsistent. We aimed to determine patterns of β-latam allergy, i.e. the association of a clinical reaction type with a specific β-lactam antibiotic.

Methods: We retrospectively evaluated data from 800 consecutive patients with suspected β-lactam hypersensitivity over a period of 11 years in a single German Allergy Center.

Results: β-lactam hypersensitivity was definitely excluded in 595 patients, immediate-type (presumably IgE-mediated) hypersensitivity was diagnosed in 70 and delayed-type hypersensitivity in 135 cases. Most (59 out of 70, 84.3%) immediate-type anaphylactic reactions were induced by a limited number of cephalosporins. Delayed reactions were regularly caused by an aminopenicillin (127 out of 135, 94.1%) and usually manifested as a measles-like exanthem (117 out of 135, 86.7%). Intradermal testing proved to be the most useful method for diagnosing β-lactam allergy, but prick testing was already positive in 24 out of 70 patients with immediate-type hypersensitivity (34.3%). Patch testing in addition to intradermal testing did not provide additional information for the diagnosis of delayed-type hypersensitivity. Almost all β-lactam allergic patients tolerated at least one, usually several alternative substances out of the β-lactam group.

Conclusions: We identified two patterns of β-lactam hypersensitivity: aminopenicillin-induced exanthem and anaphylaxis triggered by certain cephalosporins. Intradermal skin testing was the most useful method to detect both IgE-mediated and delayed-type β-lactam hypersensitivity.
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http://dx.doi.org/10.1186/s13223-020-00488-0DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7672956PMC
November 2020

Diagnosis, Classification and Management of Mast Cell Activation Syndromes (MCAS) in the Era of Personalized Medicine.

Int J Mol Sci 2020 Nov 27;21(23). Epub 2020 Nov 27.

Laboratory of Allergic Diseases, NIAID, NIH, Bethesda, MD 20852, USA.

Mast cell activation (MCA) is seen in a variety of clinical contexts and pathologies, including IgE-dependent allergic inflammation, other immunologic and inflammatory reactions, primary mast cell (MC) disorders, and hereditary alpha tryptasemia (HAT). MCA-related symptoms range from mild to severe to life-threatening. The severity of MCA-related symptoms depends on a number of factors, including genetic predisposition, the number and releasability of MCs, organs affected, and the type and consequences of comorbid conditions. In severe systemic reactions, MCA is demonstrable by a substantial increase of basal serum tryptase levels above the individual's baseline. When, in addition, the symptoms are recurrent, involve more than one organ system, and are responsive to therapy with MC-stabilizing or mediator-targeting drugs, the consensus criteria for the diagnosis of MCA syndrome (MCAS) are met. Based on the etiology of MCA, patients can further be classified as having i) primary MCAS where -mutated, clonal, MCs are detected; ii) secondary MCAS where an underlying IgE-dependent allergy or other reactive MCA-triggering pathology is found; or iii) idiopathic MCAS, where neither a triggering reactive state nor -mutated MCs are identified. Most severe MCA events occur in combined forms of MCAS, where -mutated MCs, IgE-dependent allergies and sometimes HAT are detected. These patients may suffer from life-threatening anaphylaxis and are candidates for combined treatment with various types of drugs, including IgE-blocking antibodies, anti-mediator-type drugs and MC-targeting therapy. In conclusion, detailed knowledge about the etiology, underlying pathologies and co-morbidities is important to establish the diagnosis and develop an optimal management plan for MCAS, following the principles of personalized medicine.
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http://dx.doi.org/10.3390/ijms21239030DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7731385PMC
November 2020

"Idiopathic" anaphylaxis caused by carboxymethylcellulose in ice cream.

J Allergy Clin Immunol Pract 2021 Jan 10;9(1):555-557.e1. Epub 2020 Nov 10.

Department of Dermatology and Allergy Biederstein, School of Medicine, Technical University of Munich, Munich, Germany.

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http://dx.doi.org/10.1016/j.jaip.2020.10.051DOI Listing
January 2021

Noninvasive and minimally invasive techniques for the diagnosis and management of allergic diseases.

Allergy 2021 04;76(4):1010-1023

Center of Allergy and Environment (ZAUM), Technical University and Helmholtz Zentrum München, München, Germany.

Allergic diseases of the (upper and lower) airways, the skin and the gastrointestinal tract, are on the rise, resulting in impaired quality of life, decreased productivity, and increased healthcare costs. As allergic diseases are mostly tissue-specific, local sampling methods for respective biomarkers offer the potential for increased sensitivity and specificity. Additionally, local sampling using noninvasive or minimally invasive methods can be cost-effective and well tolerated, which may even be suitable for primary or home care sampling. Non- or minimally invasive local sampling and diagnostics may enable a more thorough endotyping, may help to avoid under- or overdiagnosis, and may provide the possibility to approach precision prevention, due to early diagnosis of these local diseases even before they get systemically manifested and detectable. At the same time, dried blood samples may help to facilitate minimal-invasive primary or home care sampling for classical systemic diagnostic approaches. This EAACI position paper contains a thorough review of the various technologies in allergy diagnosis available on the market, which analytes or biomarkers are employed, and which samples or matrices can be used. Based on this assessment, EAACI position is to drive these developments to efficiently identify allergy and possibly later also viral epidemics and take advantage of comprehensive knowledge to initiate preventions and treatments.
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http://dx.doi.org/10.1111/all.14645DOI Listing
April 2021

Systemic treatments in the management of atopic dermatitis: A systematic review and meta-analysis.

Allergy 2021 04 4;76(4):1053-1076. Epub 2020 Nov 4.

Center for Evidence-Based Healthcare, University Hospital Dresden, Dresden, Germany.

Background: As an evidence resource for the currently planned European Academy of Allergy and Clinical Immunology (EAACI) clinical practice guideline "systemic treatment of atopic dermatitis (AD)," we critically appraised evidence on systemic treatments for moderate-to-severe AD.

Methods: We systematically identified randomized controlled trials (RCTs) investigating the safety and efficacy of systemic treatments for AD up to February 2020. Primary efficacy outcomes were clinical signs, AD symptoms and health-related quality of life. Primary safety outcomes included cumulative incidence rates for (serious) adverse events. Trial quality was assessed applying the Cochrane Risk of Bias Tool 2.0. Meta-analyses were conducted where appropriate.

Results: 50 RCTs totalling 6681 patients were included. Trial evidence was identified for apremilast, azathioprine (AZA), baricitinib, ciclosporin A (CSA), corticosteroids, dupilumab, interferon-gamma, intravenous immunoglobulins (IVIG), mepolizumab, methotrexate (MTX), omalizumab, upadacitinib and ustekinumab. Meta-analyses were indicated for the efficacy of baricitinib [EASI75 RD 0.16, 95% CI (0.10;0.23)] and dupilumab [EASI75, RD 0.37, 95% CI (0.32;0.42)] indicating short-term (ie 16-week treatment) superiority over placebo. Furthermore, efficacy analyses of AZA and CSA indicated short-term superiority over placebo; however, nonvalidated scores were used and can therefore not be compared to EASI.

Conclusion: The most robust, replicated high-quality trial evidence is present for the efficacy and safety of dupilumab for up to 1 year in adults. Robust trial evidence was further revealed for AZA, baricitinib and CSA. Methodological restrictions led to limited evidence-based conclusions for all other systemic treatments. Head-to-head trials with novel systemic treatments are required to clarify the future role of conventional therapies.
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http://dx.doi.org/10.1111/all.14631DOI Listing
April 2021