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BackgroundParaneoplastic 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.ObjectivesThese 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.ResultsChronic 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.ConclusionsThese 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.

BackgroundSeveral European countries recently developed international diagnostic and management guidelines for pemphigus, which have been instrumental in the standardization of pemphigus management.ObjectiveWe now present results from a subsequent Delphi consensus to broaden the generalizability of the recommendations.MethodsA preliminary survey, based on the European Dermatology Forum and the European Academy of Dermatology and Venereology guidelines, was sent to a panel of international experts to determine the level of consensus. The results were discussed at the International Bullous Diseases Consensus Group in March 2016 during the annual American Academy of Dermatology conference. Following the meeting, a second survey was sent to more experts to achieve greater international consensus.ResultsThe 39 experts participated in the first round of the Delphi survey, and 54 experts from 21 countries completed the second round. The number of statements in the survey was reduced from 175 topics in Delphi I to 24 topics in Delphi II on the basis of Delphi results and meeting discussion.LimitationsEach recommendation represents the majority opinion and therefore may not reflect all possible treatment options available.ConclusionsWe present here the recommendations resulting from this Delphi process. This international consensus includes intravenous CD20 inhibitors as a first-line therapy option for moderate-to-severe pemphigus.

Scabies, caused by the Sarcoptes scabiei var hominis mite burrowing into the skin, is a highly contagious disease characterized by intense nocturnal itching. Its global impact is considerable, affecting more than 200 million individuals annually and posing significant challenges to healthcare systems worldwide. Transmission occurs primarily through direct skin‐to‐skin contact, contributing to its widespread prevalence and emergence as a substantial public health concern affecting large populations. This review presents consensus‐based clinical practice guidelines for diagnosing and managing scabies, developed through the fuzzy Delphi method by dermatology, parasitology, pediatrics, pharmacology, and public health experts. The presence of burrows containing adult female mites, their eggs, and excreta is the diagnostic hallmark of scabies. Definitive diagnosis typically involves direct microscopic examination of skin scrapings obtained from these burrows, although dermoscopy has become a diagnostic tool in clinical practice. Treatment modalities encompass topical agents, such as permethrin, balsam of Peru, precipitated sulfur, and benzyl benzoate. In cases where topical therapy proves inadequate or in instances of crusted scabies, oral ivermectin is recommended as a systemic treatment option. This comprehensive approach addresses the diagnostic and therapeutic challenges associated with scabies, optimizing patient care, and management outcomes. [ABSTRACT FROM AUTHOR]

Background: Onychomycosis is a chronic nail infection, and dermatophytes, yeasts, and nondermatophytic molds may be the causative agents. This study aimed to determine the etiological agents of onychomycosis by using conventional and molecular methods. Methods: Between June 2020 and July 2021, 37 patients with a presumptive diagnosis of onychomycosis and mycological evidence (culture and/or EUROArray Dermatomycosis assay) were included in the study. Organisms detected in cultured nail specimens were identified by combined phenotypic characteristics and by matrix-assisted laser desorption/ionization time-of-flight mass spectrometry (MALDI-TOF MS). An EUROarray Dermatomycosis assay was used for molecular detection of fungal pathogens. Results: The EUROArray Dermatomycosis assay was positive for a single fungal target in 23 samples, and 14 samples were positive by culture. The most common pathogen was Trichophyton rubrum in both methods. Coinfection was detected in 14 samples by using molecular methods, and Trichophyton rubrum and Fusarium solani (9 samples) were the most common pathogens detected together. Trichophyton spp., nondermatophyte molds, and Candida spp. were detected in 33 (89.2%), 16 (43.2%), and 6 (16.2%) samples, respectively, when the two methods were evaluated together. Conclusions: Our results revealed that fungal culture allows the diagnosis of onychomycosis, but it is not as sensitive as the EUROArray Dermatomycosis test, especially in patients receiving antifungal therapy. [ABSTRACT FROM AUTHOR]

Byline: Asli. Bilgic, Burcin. Bozca, Gülbin. Subasi, Özlem. Dicle, Soner. Uzun, Ertan. Yilmaz, Erkan. Alpsoy, Ayse. Akman-Karakas Background: Allergic contact dermatitis (ACD) is a type IV hypersensitivity reaction, and The [...]

Byline: Birgül. Kurt, Ceren. ?rican, Betül. Ünal, Mehmet. Çiftçio?lu, Soner. UzunSir,Acrodermatitis enteropathica is a rare disease caused by impaired absorption of zinc from gastrointestinal tract which can either be genetic [...]

Rituximab appears to be effective for treating pemphigus, although there are limited long-term data. This retrospective single-center study evaluated patients with conventional treatment-resistant pemphigus who received rituximab during September 2010–December 2019. The first rituximab cycle was based on the rheumatoid arthritis protocol in all patients except one patient, and additional single doses (500 mg or 1000 mg) were administered after clinical and/or serological relapse. The consensus definitions were used for complete remission off therapy, complete remission on minimal therapy, and clinical relapse. Serological relapse was defined as a progressive ≥2-fold increase in anti-desmoglein titers (vs. previous the measurement). The study included 52 patients with pemphigus vulgaris and 1 patient with pemphigus foliaceus. The mean number of infusions was 5 and the average follow-up after the first infusion was 56 months. The average time to clinical and/or serological relapse was 12 months. Complete remission was achieved in 84.9% of patients, including after the first rituximab cycle in 25 patients (47.1%). Two patients died during the follow-up period. Additional rituximab cycles may help achieve and prolong remission in patients with moderate-to-severe pemphigus resistant to conventional therapies. However, prospective trials are needed to identify the optimal dosing protocol. [ABSTRACT FROM AUTHOR]

Background: Patch testing with dental screening series [dental patch test (DPT)] is used to detect triggers for mucositis and/or oral lichen planus as well as to detect contact sensitization due to substances and restorative materials used mostly in dentistry. Aim: We aimed to retrospectively evaluate the DPT results performed in our clinic in the last 10 years and to assess their clinical relevance. Methods: Data of 127 patients who had DPT in our allergy unit between January 2010 and July 2020 were included in our study. In our clinic, DPTs were applied to patients mostly when they have history of metal allergies, oral lichen planus especially close to dental materials, chronic mucositis, and history of allergy after dental procedures. The forms routinely used in our allergy unit were examined retrospectively. Results: The most common five allergens were nickel (II) sulfate hexahydrate (29.9%), palladium chloride (18.9%), sodium tetrachloropalladate (II) hydrate (18.9%), gold (I) sodium thiosulfate dihydrate (12.6%), and mercury (10.2%). Fifty-eight of 71 patients with positive PT had a current relevance according to the COADEX coding system (P < 0.05). Of the 38 individuals with nickel sensitization, 36 were females and 2 were males, and this result was statistically significant (P = 0.034). Conclusion: Nickel, palladium, sodium tetrachloropalladate, gold, and mercury, which are frequently found in dental prosthesis and materials, were the most common allergens in our study and this is in accordance with the literature. [ABSTRACT FROM AUTHOR]