Acquired haemophilia A (AHA) is a rare condition due to neutralizing antibodies against Factor VIII and can result in severe bleeding manifestations. The association of AHA with bullous pemphigoid (BP) is rare, most frequently observed in the elderly, and has been attributed to cross-reactive antibodies targeting Factor VIII and BPAG2 protein. The diagnosis of AHA requires specific investigations, and treatment involves urgent haemostasis and intensive immunosuppression. Managing both conditions simultaneously is challenging due to the risk of severe bleeding and treatment-related complications including infections. Early diagnosis and treatment are crucial to improve outcomes in these patients.

Bullous pemphigoid is a chronic, subepidermal autoimmune blistering disease characterized by tense blisters on erythematous or normal skin that predominantly affects the older population. The disease arises from autoantibodies targeting hemidesmosomal proteins BP180 and BP230, which are crucial for dermal–epidermal adhesion. The incidence of bullous pemphigoid is increasing, attributed to an ageing population and improved diagnostic recognition. Genetic predisposition, environmental triggers and associations with other autoimmune disorders underline its multifactorial nature. Diagnosis involves clinical presentation, histopathology, direct immunofluorescence and serological tests. Treatment aims to reduce symptoms and prevent new blister formation, using corticosteroids, immunosuppressive agents and biologics such as rituximab and omalizumab. Despite therapeutic advancements, challenges persist in long-term management, especially in older patients with comorbidities. Ongoing research into molecular mechanisms and novel therapeutic targets and clinical trials are crucial for the development of safer and more effective treatments. Bullous pemphigoid is an autoimmune skin disease characterized by tense blisters and erosions. In this Primer, Akbarialiabad et al. review the epidemiology, pathophysiology, diagnosis and management of this disease, and highlight the quality of life of patients and future research areas.

Bullous pemphigoid is a chronic, subepidermal autoimmune blistering disease characterized by tense blisters on erythematous or normal skin that predominantly affects the older population. The disease arises from autoantibodies targeting hemidesmosomal proteins BP180 and BP230, which are crucial for dermal–epidermal adhesion. The incidence of bullous pemphigoid is increasing, attributed to an ageing population and improved diagnostic recognition. Genetic predisposition, environmental triggers and associations with other autoimmune disorders underline its multifactorial nature. Diagnosis involves clinical presentation, histopathology, direct immunofluorescence and serological tests. Treatment aims to reduce symptoms and prevent new blister formation, using corticosteroids, immunosuppressive agents and biologics such as rituximab and omalizumab. Despite therapeutic advancements, challenges persist in long-term management, especially in older patients with comorbidities. Ongoing research into molecular mechanisms and novel therapeutic targets and clinical trials are crucial for the development of safer and more effective treatments. Bullous pemphigoid is an autoimmune skin disease characterized by tense blisters and erosions. In this Primer, Akbarialiabad et al. review the epidemiology, pathophysiology, diagnosis and management of this disease, and highlight the quality of life of patients and future research areas.

Introduction: The relevance of autoantibodies to clinical phenotypes and underlying disease in dermatomyositis has been studied.

EDITORIAL article Front. Med., 24 October 2023Sec. Dermatology Volume 10 - 2023 | https://doi.org/10.3389/fmed.2023.1305765

We report 2 patients who first developed cutaneous manifestations, followed by autoimmune phenomena, infections, and hypogammaglobulinemia. They were initially diagnosed with common variable immunodeficiency; however, the diagnosis was revised to cytotoxic T-lymphocyte antigen 4 haploinsufficiency after genetic and functional testing.

Coronavirus disease (COVID-19) vaccines prevent severe disease, hospitalisation and death. However, as vaccination drives commenced, there have been multiple reports of cutaneous reactions following COVID-19 vaccination and resultant vaccine hesitancy. This chapter provides evidence-based guidance on the evaluation of patients with cutaneous reactions following COVID-19 vaccination. An accurate evaluation will enable physicians to risk stratify patients based on their reaction pattern and guide patients to complete recommended COVID-19 vaccinations, where possible. Evaluation of a patient with a possible COVID-19 vaccine cutaneous reaction involves determining causality to the COVID-19 vaccination. Other possible aetiologies such as new medications and unrelated exacerbations of pre-existing dermatoses should be excluded. For cutaneous reactions that are potentially life-threatening such as anaphylaxis, severe cutaneous adverse reactions and systemic vasculitis, it would be prudent to avoid further doses of the same type of COVID-19 vaccine. Patients with urticaria/angio-oedema without features of anaphylaxis, delayed large location reactions, pemphigoid/pemphigus, vaccine-related eruption of papules and plaques, varicella-zoster virus (VZV) reactivation, flares of eczema and psoriasis may continue to receive further doses of the same COVID-19 vaccine.

BACKGROUND:The coronavirus-19 pandemic has impacted the delivery of medical education in dermatology, leading to decreased patient contact. There arose a need to pioneer innovative teaching tools to augment current methods for now and beyond the pandemic.OBJECTIVES:We aimed to assess the utility of three-dimensional (3D) images in the learning and teaching of dermatology by analysing the perceptions of medical undergraduates and faculty members in a qualitative and quantitative study.METHODS:Medical undergraduates (n = 119) and dermatology faculty members (n = 20) were recruited on a voluntary basis to watch a showcase session using a portable 3D imaging system allowing 3D images of skin lesions to be examined and digitally manipulated. After the session, participants filled in an anonymous questionnaire evaluating their perceptions.RESULTS:Of the 119 learners, most (> 84%) strongly agreed/agreed that (i) they would have more confidence in the field of dermatology; (ii) their ability to describe skin lesions would increase; (iii) their understanding of common dermatological conditions would increase; (iv) 3D images allow a greater approximation to real-life encounters than 2D images; and (v) learning with this modality would be useful. Of the 20 faculty members, most (> 84%) strongly agreed/agreed that (i) it is easier to teach with the aid of 3D images, and (ii) they would want access to 3D images during teaching sessions. Skin tumours were perceived to be learnt best via this modality in terms of showcasing topography (P < 0.01) and close approximation to real-life (P < 0.001). Overall, thematic analysis from qualitative analysis revealed that conditions learnt better with 3D images were those with surface changes and characteristic topography.CONCLUSIONS:Our results show that the greatest utility of 3D images lies in conditions where lesions have skin surface changes in the form of protrusions or depressions, such as in skin tumours or ulcers. As such, 3D images can be useful teaching tools in dermatology, especially in conditions where appreciation of surface changes and topography is important.

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.

Lichenoid drug eruption is an uncommon condition which can be difficult to distinguish from classical lichen planus clinically and histologically. Clinicians face further difficulties in the wide range of drug causes and long latency period of exposure before rash appears. Novel drugs such as biologics and immune checkpoint inhibitors are now increasingly implicated and pose new clinical challenges.

Bullous pemphigoid (BP) is the most common autoimmune subepidermal blistering disease of the skin and mucous membranes. This disease typically affects the elderly and presents with itch and localized or, most frequently, generalized bullous lesions. A subset of patients only develops excoriations, prurigo‐like lesions, and eczematous and/or urticarial erythematous lesions. The disease, which is significantly associated with neurological disorders, has high morbidity and severely impacts the quality of life.