[Paraneoplastic pemphigus with underlying Castleman's disease in a 16-year-old girl]. / Morbus Castleman-assoziierter paraneoplastischer Pemphigus bei einer 16-jährigen Patientin.

Paraneoplastic pemphigus is a rare, life-threatening autoimmune disease that is clinically characterized by mostly extensive and refractory mucosal erosions and polymorphous skin lesions. We report here on a 16-year-old girl with isolated oral erosions, in whom mucosal pemphigoid was initially suspected and after treatment with prednisolone and dapsone marked improvement was achieved. However, a few months later the patient developed massive respiratory insufficiency as a result of bronchiolitis obliterans, so that a lung transplant was planned. As part of the preparatory diagnostic workup, unicentric, abdominally localized Castleman's disease was diagnosed, which ultimately led to the diagnosis of paraneoplastic pemphigus as evidenced by envoplakin autoantibodies. Tumor resection and subsequent lung transplantation achieved good results with sustained mucocutaneous remission.

Telephone follow-up after outpatient nail surgery - a retrospective analysis.

BACKGROUND: One of the areas of care in dermatosurgery is the surgical treatment of diseases of the nail organ. Side effects and complications after nail surgery were investigated by telephone follow-up (TFU), and its suitability for postoperative monitoring and consultation was assessed. PATIENTS AND METHODS: All patients who underwent nail surgery at the Department of Dermatology at the Ludwigshafen City Hospital from October 2019 to December 2021 in outpatient setting were contacted by telephone on the second to third postoperative day and questioned in a standardized manner about postoperative complaints and counselled if necessary. RESULTS: A total of 100 cases were followed up. The most common procedures performed were phenol matricectomy (41%), nail avulsion (16%), and nail matrix biopsies (9%). 50% and 21% of patients reported pain on the day of the procedure and the day after surgery, respectively. After nail avulsion, pain was statistically significantly more frequently reported on the day following the procedure and pain medication was statistically significantly more frequently required (p  =  0.002). Serious adverse events did not occur after nail surgery. 10% of the respondents raised specific questions and needed counseling by TFU. CONCLUSIONS: All nail surgeries were well tolerated in the outpatient setting. Pain was the most common side effect, although only half of all patients reported pain on the day of surgery and only 21% on the day after the procedure. The TFU proved to be an effective and practical as well as easy to establish method for postoperative follow-up and consultation after outpatient nail surgery.

Hydrochlorothiazide and increased risk of atypical fibroxanthoma and pleomorphic dermal sarcoma.

BACKGROUND AND OBJECTIVES: Previous work has demonstrated that hydrochlorothiazide (HCTZ) is a risk factor for squamous cell carcinomas (SCC) and basal cell carcinomas (BCC) due to pro-photocarcinogenic effects. Atypical fibroxanthoma (AFX) and pleomorphic sarcoma (PDS), both ultraviolet-induced cancers, display a rare but rising cutaneous tumor entity. This study aimed to evaluate if the use of HCTZ is higher in patients with AFX/PDS than in patients with SCC/BCC and subsequently may be a risk factor for AFX/PDS-development. PATIENTS AND METHODS: In a retrospective study of four German skin cancer centers, AFX/PDS cases and SCC/BCC controls were sex and age matched (1:3) over a time-period of 7 years (2013-2019) to evaluate the use of HCTZ, immunosuppressive medication, second malignancies, and presence of diabetes mellitus. RESULTS: Overall, 146 AFX/PDS and 438 controls (SCC/BCC) were included in the study. The use of HCTZ was significantly higher in patients with AFX/PDS (44.5%) compared to patients with SCC/BCC (25.3%). Additionally, the presence of diabetes mellitus was significantly higher in AFX/PDS patients. CONCLUSIONS: This study demonstrates a significantly higher use of HCTZ in patients with AFX/PDS compared to SCC/BCC. This result suggests that HCTZ may be a risk factor for AFX/PDS. Additionally, diabetes mellitus or its comorbidities may be associated with an increased risk for AFX/PDS.

Allergic Reaction to a Commercially Available Insect Snack Caused by House Cricket (Acheta domesticus) Tropomyosin.

SCOPE: Edible insects contain allergens with potential cross-reactivity to other invertebrates. Here, this study examines IgE-reactive proteins in a house cricket snack (Acheta domesticus) leading to an allergic reaction in a 27-year old man followed by a similar reaction days later after eating shrimps. METHODS AND RESULTS: Prick to prick tests verify the IgE-mediated allergy to crickets and skin prick testing confirms a type I sensitization to house dust mite without any clinical relevance for the patient, and to shrimp extracts, but is negative for several other foods. Serological testing reveals a sensitization to shrimps, shrimp tropomyosin, and house dust mite tropomyosin. IgE-immunodetection shows that the cricket allergic patient is sensitized to two proteins of 45 and >97 kDa using aqueous control cricket extract, but to only one protein at around 45 kDa when using the causative, seasoned insect snack extract. Mass spectrometry data and IgE-inhibition experiments clearly identify this protein belonging to the tropomyosin allergen family. CONCLUSION: This case report suggests that cricket tropomyosin may be an elicitor of allergic reactions even in previously not allergic patients, although it cannot be excluded the patient reacted additionally to other ingredients of the snack.

S2k guidelines on diagnosis and treatment of linear IgA dermatosis initiated by the European Academy of Dermatology and Venereology.

INTRODUCTION: Linear IgA dermatosis (LAD) is a rare subepidermal autoimmune bullous disease (AIBD) defined by predominant or exclusive immune deposits of immunoglobulin A at the basement membrane zone of skin or mucous membranes. This disorder is a rare, clinically and immunologically heterogeneous disease occurring both in children and in adults. The aim of this project is to present the main clinical features of LAD, to propose a diagnostic algorithm and provide management guidelines based primarily on experts' opinion because of the lack of large methodologically sound clinical studies. METHODS: These guidelines were initiated by the European Academy of Dermatology and Venereology (EADV) Task Force Autoimmune Bullous Diseases (AIBD). To achieve a broad consensus for these S2k consensus-based guidelines, a total of 29 experts from different countries, both European and non-European, including dermatologists, paediatric dermatologists and paediatricians were invited. All members of the guidelines committee agreed to develop consensus-based (S2k) guidelines. Prior to a first virtual consensus meeting, each of the invited authors elaborated a section of the present guidelines focusing on a selected topic, based on the relevant literature. All drafts were circulated among members of the writing group, and recommendations were discussed and voted during two hybrid consensus meetings. RESULTS: The guidelines summarizes evidence-based and expert opinion-based recommendations (S2 level) on the diagnosis and treatment of LAD. CONCLUSION: These guidelines will support dermatologists to improve their knowledge on the diagnosis and management of LAD.

[Genitoanal infections caused by Panton-Valentine leukocidin (PVL)-positive Staphylococcus aureus : Smear infection or sexually transmitted disease?] / Genitoanale Infektionen durch Panton-Valentine-Leukozidin(PVL)-positiven Staphylococcus aureus : Schmierinfektion oder sexuell übertragbare Erkrankung?

Panton-Valentine leukocidin (PVL) is a pore-forming exotoxin produced by certain Staphylococcus (S.) aureus strains, which is responsible for the increased virulence of the pathogen. Thus, infections caused by PVL-positive S. aureus tend to recur. Usually, the infection is a smear infection, which can cause folliculitis and purulent lid margin inflammation in addition to the classic mucocutaneous abscesses. Recently, recurrent genitoanal infections caused by PVL-positive S. aureus have also been described. In most cases, this is a sexually transmitted disease. Currently, it is assumed that most infections are imported from abroad. In addition to treatment of these infections, decolonization should be performed for prophylaxis of recurrence.

Paradoxical tralokinumab-induced psoriasis in a patient with atopic dermatitis.

Purpose: Although psoriasis and atopic dermatitis (AD) were for decades considered to be opposing diseases, it is now known that these skin conditions can coexist or even overlap in the same individual. Especially when using modern drugs with targeted IL inhibition, the balance between Th1 and Th2 immunity can be disturbed. In line with it, numerous clinical cases of AD have been induced by antipsoriatic biologics (e.g., TNF-alpha, IL-23, or IL-17 inhibitors), and IL-4-/IL-13 inhibition by dupilumab also resulted in paradoxical psoriasis in patients with AD.Materials and methods: Herein, we describe a case of psoriasis vulgaris in a patient with intrinsic AD after systemic treatment with the anti-IL-13 antibody tralokinumab.Results: We present a 36-years-old male patient with a severe course of an intrinsic atopic dermatitis and dyshidrotic hand eczema. He responded well to the therapy with tralokinumab. However, about 7 months after the start of anti-IL-13 treatment the patient developed psoriasiform lesions. The drug was then discontinued. Currently, the patient is receiving topical therapy with topical corticosteroids and calcineurin inhibitors with stable course of psoriasis and AD.Conclusions: This case suggests, that not only a dual IL-4-/IL-13-blockade, but also a selective IL-13-inhibition is able to skew immune responses toward IL-17 cytokine pathway-related disease. However, no clinical scores exist to predict the development of paradoxical psoriasis in patients with AD during therapy with biologics.

Effects of the COVID-19 pandemic on inpatient skin surgery in Germany: results of the DESSI-survey.

BACKGROUND: The aim of the survey was to investigate a possible impairment of inpatient dermatological and dermatosurgical care in Germany due to the COVID-19 pandemic. METHODS: An online survey on the impact of pandemic-related measures on inpatient care was sent to all German dermatology clinics. Only one person per clinic was asked to participate. Data analysis was mainly descriptive. Differences between university hospitals and non-university hospitals were calculated using the Chi-square test. RESULTS: From the 113 dermatological clinics with inpatient care, we received 45 (39.8%) at least partially completed questionnaires. Of these, 25 (55.6%) came from university hospitals, 18 (40.0%) from teaching hospitals of a university, 1 (2.2%) from a non-teaching hospital, and 1 (2.2%) from a participant who did not provide any information on his facility. More than half of survey participants (57.8%) reported that many elective skin surgeries had to be canceled at their clinics at the beginning of the COVID-19 pandemic. However, most of the clinics (75.6%) were able to perform medically necessary surgeries (such as for malignant melanoma). Only 28.9% (13/45) of participants reported that skin surgery in their clinics returned to full strength after the COVID-19 pandemic. There was no statistically significant difference between university hospitals and non-university hospitals regarding the influence of COVID-19-related restrictions. CONCLUSIONS: Despite their heterogeneity, the results of the survey show an overall clear and long-term pandemic-related impairment of inpatient dermatology and skin surgery in Germany.

[Reasons for allergology consultations prior to COVID-19 vaccination and their outcomes : Two-year experience of an allergy center in North Rhine-Westphalia]. / Gründe für allergologische Diagnostik und deren Ergebnisse im Kontext von COVID-19-Vakzinierungen : 2-jährige Erfahrung eines Allergiezentrums in Nordrhein-Westfalen.

BACKGROUND: To counteract the coronavirus disease 2019 (COVID-19) pandemic, several severe acute respiratory syndrome coronavirus type 2 (SARS-CoV-2) vaccines have been licensed since December 2020. Shortly after the start of the vaccination campaigns, occasional allergic reactions related to vaccines were described, thus, leading to concerns in many patients with a history of allergies. The aim of this work was to evaluate which anamnestic events represented a reason for an allergology work-up before COVID-19 vaccination. Furthermore, the results of the allergology diagnostics are described. METHODS: We performed a retrospective data analysis of all patients who presented at the Center for Dermatology, Allergology and Dermatosurgery of the Helios University Hospital Wuppertal during the years 2021 and 2022 for allergology work-up prior to COVID-19 vaccination. Demographic data, allergological history, reason for the consultation in the clinic and results of allergology diagnostic tests including reactions after vaccination were included. RESULTS: A total of 93 patients presented for allergology work-up with COVID-19 vaccines. In about half of the cases, the reasons for the presentation to the clinic were doubts and concerns about allergic reactions and side effects. In all, 26.9% (25/93) of the presented patients had not previously received a COVID-19 vaccine yet and 23.7% (22/93) of patients developed non-allergic reactions after prior COVID-19 vaccine (e.g., headache, chills, fever, malaise). Of the patients, 46.2% (43/93) were successfully vaccinated in the clinic due to a complex allergological history, while the remaining 53.8% (50/93) were subjected to outpatient vaccination in the vaccination practice. Only one patient with known chronic spontaneous urticaria developed a mild angioedema of the lips a few hours after vaccination; however, we do not consider this episode an allergic reaction to the vaccine due to the time delay. CONCLUSION: Allergic reactions after COVID-19 vaccines are rare, but many patients with a positive medical history of allergies are concerned about allergic reactions after COVID-19 vaccination. Thus, public work by practicing allergologists is important during vaccination campaigns to meet the concerns and fears of the population, especially of patients with allergies in their medical history.

S2k guidelines on the management of paraneoplastic pemphigus/paraneoplastic autoimmune multiorgan syndrome initiated by the European Academy of Dermatology and Venereology (EADV).

BACKGROUND: Paraneoplastic pemphigus (PNP), also called paraneoplastic autoimmune multiorgan syndrome (PAMS), is a rare autoimmune disease with mucocutaneous and multi-organ involvement. PNP/PAMS is typically associated with lymphoproliferative or haematological malignancies, and less frequently with solid malignancies. The mortality rate of PNP/PAMS is elevated owing to the increased risk of severe infections and disease-associated complications, such as bronchiolitis obliterans. OBJECTIVES: These guidelines summarize evidence-based and expert-based recommendations (S2k level) for the clinical characterization, diagnosis and management of PNP/PAMS. They have been initiated by the Task Force Autoimmune Blistering Diseases of the European Academy of Dermatology and Venereology with the contribution of physicians from all relevant disciplines. The degree of consent among all task force members was included. RESULTS: Chronic severe mucositis and polymorphic skin lesions are clue clinical characteristics of PNP/PAMS. A complete assessment of the patient with suspected PNP/PAMS, requiring histopathological study and immunopathological investigations, including direct and indirect immunofluorescence, ELISA and, where available, immunoblotting/immunoprecipitation, is recommended to achieve a diagnosis of PNP/PAMS. Detection of anti-envoplakin antibodies and/or circulating antibodies binding to the rat bladder epithelium at indirect immunofluorescence is the most specific tool for the diagnosis of PNP/PAMS in a patient with compatible clinical and anamnestic features. Treatment of PNP/PAMS is highly challenging. Systemic steroids up to 1.5 mg/kg/day are recommended as first-line option. Rituximab is also recommended in patients with PNP/PAMS secondary to lymphoproliferative conditions but might also be considered in cases of PNP/PAMS associated with solid tumours. A multidisciplinary approach involving pneumologists, ophthalmologists and onco-haematologists is recommended for optimal management of the patients. CONCLUSIONS: These are the first European guidelines for the diagnosis and management of PNP/PAMS. Diagnostic criteria and therapeutic recommendations will require further validation by prospective studies.

Medical error analysis in dermatology according to the reports of the North Rhine Medical Association from 2004 to 2018.

BACKGROUND: Assessment of complaints addressed to the Expert Committee for Medical Malpractice Claims of the North Rhine Medical Association helps to identify quality assurance problems in dermatology. METHODS: 333 dermatological reports (years 2004-2018) were allocated to three five-year intervals in order to show changes of total number of reports and with regard to indications, types of care, defined diagnosis categories, and therapeutic interventions. Statistical analysis was mainly descriptive (SPSS 27). Representative cases are explained in more detail. RESULTS: The number of dermatological reports fell from 119 in the first to 92 reports in the last five-year interval, along with a decline in treatment error rates (43 % and 29 %) despite an increasing number of procedures across disciplines. 104 reports dealt with (supposed) diagnostic errors, 156 with physical or pharmacological therapies, and 73 reports with surgery. The latter had a low treatment error rate (29 %). CONCLUSIONS: In comparison to the number of dermatological treatments in Germany, patient allegations are raised only rarely indicating greater patient satisfaction and/or a lower risk potential in dermatology. Incorrect dosages or device settings, diagnostic errors, and deficits in obtaining informed consent are relevant sources of error as underlined by the presented case reports.

Zunahme des atypischen Fibroxanthoms und pleomorphen dermalen Sarkoms: eine retrospektive Analyse vier deutscher Hauttumorzentren.

HINTERGRUND UND ZIELE: In den letzten Jahren konnten umfassende Erkenntnisse über die Pathogenese, Diagnostik und Behandlung von kutanen Sarkomen, insbesondere des atypischen Fibroxanthoms (AFX) und pleomorphen dermalen Sarkoms (PDS) gesammelt werden. Beide Entitäten zeigten innerhalb der letzten Dekade steigende Inzidenzraten. Die vorliegende Studie diente der Untersuchung, welchen Einfluss die neuen Erkenntnisse auf die Fallzahlen von AFX/PDS im Vergleich zu anderen Sarkom-Entitäten haben. PATIENTEN UND METHODIK: Diese retrospektive Studie wurde an vier deutschen Hauttumorzentren durchgeführt und alle von zertifizierten Dermatopathologen bestätigten histopathologischen Befunde von kutanen Sarkomen (AFX, PDS, Dermatofibrosarcoma protuberans, kutanes Leiomyosarkom, Angiosarkom und Kaposi-Sarkom) in einem Zeitraum von sieben Jahren (2013-2019) evaluiert. Zusätzlich wurde der Einsatz von immunhistochemischen Markern als diagnostische Hilfe (Panzytokeratin, S100, Desmin, CD34, CD10, Prokollagen-1, CD99, CD14 und CD68) erfasst. ERGEBNISSE: Insgesamt konnten 255 kutane Sarkome in die vorliegende Studie eingeschlossen werden. Die Zahl der kutanen Sarkome nahm kontinuierlich von 2013 bis 2019 zu (von 16 auf 52 Fälle im Jahr). Die Diagnose eines AFX/PDS konnte in 2019 4,6-mal häufiger als in 2013 gestellt werden. Das AFX stellte mit 49,3 % aller kutanen Sarkome den häufigsten Sarkom-Subtypen dar. Zusätzlich war der Anstieg von AFX/PDS mit dem Einsatz von Immunhistochemie assoziiert. Der Einsatz von spezifischen Immunhistochemischen Markern stieg von 57,1 % im Jahr 2013 auf 100 % in 2019. SCHLUSSFOLGERUNGEN: Diese retrospektive Studie von vier deutschen Hauttumorzentren demonstriert eine substanzielle Zunahme von AFX/PDS, wahrscheinlich infolge kürzlich etablierter beziehungsweise verbesserter diagnostischer und terminologischer Standards. Dieser Anstieg ist vermutlich mit dem vermehrten Einsatz von bestimmten immunhistochemischen Markern assoziiert. AFX/PDS treten wahrscheinlich häufiger auf als bisher vermutet und repräsentieren möglicherweise den häufigsten kutanen Sarkom-Subtyp.