Single-cell sequencing reveals distinct immune cell features in cutaneous lesions of pemphigus vulgaris and bullous pemphigoid.

Bullous pemphigoid (BP) and pemphigus vulgaris (PV) are two common subtypes of autoimmune bullous disease (AIBD). The key role of circulating autoreactive immune cells contributing to skin damage of AIBD has been widely recognized. Nevertheless, the immune characteristics in cutaneous lesions remain unclear. Here, we performed single-cell RNA sequencing (scRNA-seq) and single-cell VDJ sequencing (scRNA-seq) to generate transcriptional profiles for cells and T/B cell clonetype in skin lesions of BP and PV. We found that the proportions of NK&T, macrophages/ dendritic cells, B cells, and mast cells increased in BP and PV lesions. Then, BP and PV cells constituted over 75% of all myeloid cell subtypes, CD4+ T cell subtypes and CD8+ T cell subtypes. Strikingly, CD8+ Trm was identified to be expanded in PV, and located in the intermediate state of the pseudotime trajectory from CD8+ Tm to CD8+ Tem. Interestingly, CD8+ Tem and CD4+ Treg highly expressed exhaustion-related genes, especially in BP lesions. Moreover, the enhanced cell communication between stromal cells and immune cells like B cells and macrophages/ dendritic cells was also identified in BP and PV lesions. Finally, clone expansion was observed in T cells of BP and PV compared with HC, while CD8+ Trm represented the highest ratio of hyperexpanded TCR clones among all T cell subtypes. Our study generally depicts a large and comprehensive single-cell landscape of cutaneous lesions and highlights immune cell features in BP and PV. This offers potential research targets for further investigation.

Toxic epidermal necrolysis-like cutaneous toxicity following chimeric antigen receptor T-cell therapy in recurrent large B-cell lymphoma.

Chimeric antigen receptor (CAR) T-cell therapy has demonstrated remarkable success in treating various B-cell malignancies, redirecting T-cell cytotoxicity toward cancer cells. Despite its efficacy, CAR-T therapy is associated with potential risks, including cytokine release syndrome (CRS) and cytopenia. We present a case of a 69-year-old man with diffuse large B-cell lymphoma treated with axicabtagene-ciloleucel CAR-T therapy, who developed a rare and severe cutaneous toxicity resembling toxic epidermal necrolysis (TEN). The patient exhibited persistent fevers, CRS, and subsequent development of a widespread erythematous macular eruption, progressing to vesiculation with bullae. Notably, allopurinol-induced TEN was considered with the patient's recent exposure to allopurinol, although the onset and minimal mucosal involvement did not align with typical presentations of allopurinol-induced cases. The cutaneous reaction, distinct from typical SJS/TEN, showed minimal mucosal involvement and coincided with the cytokine release storm, differing from allopurinol-induced TEN. Despite the absence of guidelines, the patient was managed with systemic steroids, achieving significant improvement. This case expands the spectrum of CAR-T therapy-related cutaneous toxicities, highlighting the need for early recognition of histopathology and tailored management by dermatologists. Further understanding of these reactions is crucial for optimizing the safety profile of this groundbreaking immunotherapy.

Eye and lid involvement as an uncommon feature of pemphigus foliaceus in a pediatric patient.

Pemphigus foliaceus (PF) is an autoimmune blistering disorder which affects the superficial layers of the epidermis with rare mucosal involvement. We present the case of a 12-year-old girl with PF involving the eyes and eyelids. A literature review of pediatric nonendemic PF revealed another two cases with ocular manifestations. Eyelid involvement is an uncommon feature of PF that should be properly identified and treated.

Linear IgA bullous dermatosis of childhood: Retrospective single-center cohort.

Linear IgA bullous dermatosis (LABD) is a rare autoimmune blistering disorder impacting children and adults. In this single-center retrospective chart review of pediatric patients with LABD at a large tertiary referral center, we report the unifying and unique clinical features of 10 pediatric patients. Patients typically presented with the "cluster of jewels" sign (n = 6; 60%), mucous membrane involvement (n = 5; 50%) and had a mean disease duration of 38 months; six patients (60%) required inpatient admission for management of their skin disease, including all five patients who had mucous membrane involvement. Our findings suggest that pediatric LABD may be a disease with high morbidity and may be associated with severe complications when mucous membranes are involved.

Maintenance treatment of pemphigus with rituximab in real life: A single-center study of 50 patients.

BACKGROUND: Following the RITUX 3 therapeutic trial, the French national diagnosis and care protocol (NDCP) for the treatment of pemphigus was updated in 2018. The updated protocol recommends initial treatment with rituximab (RTX) followed by maintenance therapy at 12 and 18 months, and potentially at 6 months where there are risk factors for early relapse. We evaluated these recommendations regarding the management of our own patients. PATIENTS AND METHODS: Our single-center retrospective study included all patients with pemphigus diagnosed between 01/2015 and 10/2020 and receiving at least one initial infusion of RTX. We collected the following data: type of pemphigus, severity, levels of anti-desmoglein 1 and 3 antibodies at diagnosis and between 2 and 6 months after initial RTX, presence or absence of maintenance therapy and modalities, time to first relapse and duration of associated systemic corticosteroid therapy ≥5 mg/day. Maintenance treatment modalities were as follows: no maintenance treatment, maintenance "on demand" (MT1) i.e. not performed at the rate imposed by the NDCP, and maintenance "according to NDCP" (MT2). RESULTS: Fifty patients were included (women 54%, median age 58 years, pemphigus vulgaris 68%, moderate to severe 68%). Initial RTX was combined with systemic corticosteroid therapy at 0.5 to 1 mg/kg in 74% of cases. Twenty-seven patients (54%) received no maintenance therapy, 13 were on an MT1 regimen (26%), and 10 were on an MT2 regimen (20%). Median follow-up was 42 months. At the last follow-up, 39 patients (78%) were in complete remission. A total of 25 patients (50%) relapsed: 18/27 (67%) patients without maintenance, 5/13 (38%) with MT1, and 2/10 (20%) with MT2 (p = 0.026). The probability of relapse over time was significantly lower in patients receiving maintenance therapy compared to those who receiving none (p = 0.022). The median time to relapse was 15 months in patients without maintenance, and 30 and 28 in those with maintenance (p = 0.27). The median duration of systemic corticosteroid therapy ≥ 5 mg/day in the no-maintenance group was 10 months, compared to 7 and 9 months respectively in MT1 and MT2 (p = 0.91). CONCLUSION: Our study confirms the value of RTX maintenance therapy in pemphigus in real life.

Utility of the pattern-based approach using BIOCHIP-indirect immunofluorescence in the evaluation of subepidermal bullous disorders.

BACKGROUND: Subepidermal bullous disorders (SEBD) are a heterogeneous group of vesiculobullous diseases because of antibody-mediated destruction of proteins of the dermo-epidermal junction. Direct immunofluorescence (DIF) is the gold standard for diagnosis. BIOCHIP-indirect immunofluorescence (IIF) is a novel serological test that combines multiple target antigens in a single field. The present study aimed to evaluate the utility of the pattern-based approach in BIOCHIP-IIF for the diagnosis of SEBD. METHODS: Seventy cases of BIOCHIP-IIF that showed clinical, histopathological, and/or DIF features favoring SEBD were included in the study. The interpretation in the BIOCHIP was categorized into one of the following patterns. Pattern I: basement membrane zone (BMZ) staining in monkey esophagus (ME), primate salt-split skin (SSS)-roof staining, BP180+ and/or BP230+; Pattern II: roof staining in SSS, BP180- and BP230- with or without BMZ staining in ME; Pattern III: floor staining in SSS, BP180- and BP230-; and pattern IV: negative in SSS and other substrates. The findings were correlated with histopathology and/or DIF. RESULTS: Fifty (71.5%) cases showed pattern I or the typical bullous pemphigoid (BP) pattern. Eight (11.4%) cases showed pattern II. Patterns III and IV were observed in seven (10%) and five (7.1%) cases, respectively. BP was the most common diagnosis in patterns I and II, while anti-p200 pemphigoid was most common in pattern III, as confirmed by immunoblotting. The sensitivity of pattern I in the diagnosis of BP was 96%. CONCLUSION: BIOCHIP-IIF showed a good correlation with DIF and histopathology in the diagnosis of SEBD. This can be used as a first-line investigation in case of bullous disorders.

COVID-19 associated severe mucocutaneous blistering eruptions: A case series.

Mucocutaneous eruptions are associated with numerous infectious processes and can present as erythema multiforme (EM), reactive infectious mucocutaneous eruption (RIME), Stevens Johnson syndrome (SJS), or toxic epidermal necrolysis (TEN). Limited reports have detailed the association of these eruptions with SARS-CoV-2 infection. We present a series of eight cases of severe mucocutaneous blistering eruptions associated with SARS-CoV-2 infection. A retrospective case series was performed at six tertiary medical centers from March 1, 2020 to August 1, 2022. Inclusion criteria were met with a clinical diagnosis of EM, RIME, SJS, or TEN and a positive SARS-CoV-2 test (rapid antigen or polymerase chain reaction) less than 4 weeks prior to onset of dermatologic manifestation. Data was collected at time of each patient encounter. Eight patients met criteria with six pediatric patients (<18 years of age) having a median age of 15 years and two adult patients (>18 years of age) having a median age of 36 years. Patients were found to have a clinical diagnosis of RIME in 85.7% of cases. Oral mucosal involvement was the most common clinical finding (100%), followed by ocular (50.0%), urogenital (50.0%), and skin (37.5%) involvement. Evaluation did not reveal any additional infectious triggers in four patients. Evidence of possible concurrent or previous infectious triggers were identified in four patients. This case series highlights the development of severe mucocutaneous eruptions in association with COVID-19 infection, as well as the potential contributing role of concurrent or prior infections.

Role of Human Leukocyte Antigen Class II in Antibody-Mediated Skin Disorders.

HLA class II molecules are key factors determining susceptibility to autoimmune disorders, and their role in immune-mediated skin conditions such as psoriasis has been extensively investigated. However, there is currently little understanding of their role in antibody-mediated skin diseases such as autoimmune blistering disorders. We researched the available literature using PubMed to narratively review the current knowledge on HLA associations in antibody-mediated blistering skin pathologies. Our results summarized the risk alleles that are identified in the literature, together with certain known protective alleles: in the pemphigus group, alleles HLA-DQB1*0503 and HLA-DRB1*0402 are most commonly associated with disease; in the pemphigoid group, the most studied allele is HLA-DQB1*0301; in epidermolysis bullosa acquisita, few genetic studies are available; in dermatitis herpetiformis, the association with haplotypes HLA-DQ2 and HLA-DQ8 is strongly established; finally, in linear IgA bullous disease, specific HLA alleles may be responsible for pediatric presentations. Our current pathogenic understanding of this group of disorders assigns a key role to predisposing HLA class II alleles that are able to bind disease autoantigens and therefore stimulate antigen-specific autoreactive T cells. The latter engage B lymphocytes that will produce pathogenic autoantibodies. The distribution of HLA alleles and their disease associations are variable across demographics, and an in-depth pathogenetic understanding is needed to support associations between HLA alleles and disease phenotypes. Additionally, in a personalized medicine approach, the identification of HLA alleles associated with the risk of disease may become clinically relevant in identifying susceptible subjects that should avoid exposure to known triggers, such as medication, when possible.

Ultra-high frequency ultrasound detection of the dermo-epidermal junction: Its potential role in dermatology.

Traditional high-frequency ultrasound (HFUS; 20 MHz) is a non-invasive method used to study skin in vivo but is not able to measure skin thickness accurately and to identify the dermal-epidermal junction (DEJ). Ultra-high frequency ultrasound (UHFUS; 70-100 MHz) has sub-millimetre resolution comparable to histology. The aim of this study was to identify, by UHFUS, the DEJ and to describe skin differences in healthy individuals by providing a measure of skin thickness, based on age and gender. We also described the bullous pemphigoid lesion. We enrolled 42 patients divided into 2 groups: A and B. Group A included 32 healthy volunteers aged 22-74 years. Group B consisted of 10 patients with bullous pemphigoid. For each patient in group A, 8 ultrasound (US) clips by 70 MHz probe were performed at forehead, cheek, nose, forearm, abdomen, chest, back and leg. For each patient in group B, 5 US images were acquired at blisters roofs and edges. In each US image, we measured thickness of stratum corneum (α-ß), epidermis (α-γ) and epidermis plus dermis (α-δ). In both groups, we found the presence of 4 lines delimiting: the stratum corneum (the layer between α-line and ß-line), the epidermis (distance between α- and γ-line), and the boundary between dermis and subcutis (δ-line). The γ-line corresponds to the point of detachment of the bullae. The abdominal α-ß layer was thicker in males (p = 0.019) and α-δ thickness at cheeks (p < 0.001), chest (p = 0.007), back (p = 0.025) and forearm (p < 0.001). In females, α-γ thickness of the back was greater (p = 0.005). In old people compared to young, we noticed an increase of α-ß layer at forehead and chest (p = 0.014), an increase of α-γ layer at forearm (p = 0.001), back (p = 0.024) and leg (p = 0.010) and an increase of α-δ layer at forehead (p = 0.001) and nose (p = 0.049). UHFUS is an advanced imaging technique that can detect both the DEJ and the boundary between dermis and subcutaneous tissue so that epidermal and dermal thickness can be measured with good accuracy. Regarding gender and age, skin differences obtained with UHFUS were comparable to other non-invasive methods.

Assessing the use of methotrexate as an alternate therapy for pemphigus vulgaris and pemphigus foliaceus.

Methotrexate is historically recognized as an effective treatment of pemphigus but its utility as a single or alternate steroid-sparing agent was not recognized in recent consensus recommendations in pemphigus management. We aimed to evaluate the efficacy and safety of a treatment course for pemphigus that involves methotrexate as a single or steroid-sparing agent. In a retrospective cohort study, we examined patients with pemphigus vulgaris or pemphigus foliaceus who were on ≥3 months of methotrexate therapy. Efficacy and safety were evaluated by established pemphigus disease endpoints. Of the 34 patients who met inclusion criteria, 25 (73.5%) were on glucocorticoids at time of methotrexate initiation (median follow-up: 5.4 years; median time on methotrexate: 3.7 years). An appreciable proportion achieved disease control (91.2%), with some achieving clinical remission off all systemic therapies (23.5%). For patients on glucocorticoids, median time to control was 42 days, median time to minimal steroid dose tapering (5 mg prednisone) was 161 days, and median time to complete steroid tapering was 308 days. For patients on methotrexate as a single agent, median time to control was 119 days. Among all patients, relapse commonly occurred (88.2%). At last follow-up, 26.5% were managed on topical therapies alone and 11.8% required systemic steroid therapy. Methotrexate was largely tolerated with a low incidence of adverse events leading to treatment discontinuation (2.9%). Methotrexate has the potential to be an effective and well-tolerated option for patients and may be considered for use as an alternate single or steroid-sparing agent for pemphigus.

Multiple cycles of rituximab therapy for pemphigus: A group of patients with difficult- to-treat disease or a consequence of late rituximab initiation?

Pemphigus is a serious autoimmune disease with few appropriate therapeutic options. Although rituximab (RTX) has recently shown great promise in this regard, the best protocol of its administration is remains to be elucidated. This study aimed to evaluate the patients who need at least 3 cycles of treatment with RTX to identify hard-to-treat patients' characteristics, which might lead to consider more prompt protocols for treatment of them. A retrospective cross-sectional study was conducted on 45 patients with pemphigus vulgaris who received at least 3 cycles of RTX. Their demographic, clinical, and laboratory data as well as details of treatment protocol and final clinical situation of patients were evaluated. Totally, 45 patients (21 men and 24 women) with mean age of 44.33 years were included in this paper. Women were about 8 years older than men (mean age: 48.1 years versus 40.1 years, p: 0.011) and needed RTX approximately 2 years later in their course of disease (gap: 41.04 months vs. 14.85 months, p: 0.003). Buccal, truncal, and scalp regions were the most frequent sites of involvement respectively. A significant decrease in both anti-Dsg1, 3 was seen at last visit compared to baseline. However, the amount of this decrement was not significantly different between them (p: 0.083). Partial remission in 31.11%, complete remission in 24.44%, relapse in 15.56%, partial remission on treatment in 15.56% and complete remission on treatment in 13.33% were seen at the last follow-up session. RTX is an effective medication for PV even in patients with refractory disease and its therapeutic effect is increased with each subsequent cycle. Male gender, severe oral mucosal involvement on disease onset and extensive scalp and truncal lesions as first cutaneous manifestation of disease are more likely to be signs of refractory PV. Hence, it is reasonable to consider more prompt protocols for treatment of these cases. Moreover, late prescription of RTX during the course of disease might play a role in presence of more resistant form of disease.

ELISA detection of anti-desmoglein 1 and anti-desmoglein 3 and indirect immunofluorescence in oral pemphigus: A retrospective study.

OBJECTIVE: The aim of this study was to test the efficacy of autoantibodies to desmoglein 1 and desmoglein 3 detected by ELISA and indirect immunofluorescence in the diagnosis of oral pemphigus and to correlate the antibody titres with the severity of the disease. MATERIALS AND METHODS: We report a retrospective cohort study of 22 patients with oral pemphigus and 64 controls from a single tertiary centre. Data about histopathological examination, direct immunofluorescence, indirect immunofluorescence and ELISA were analysed. Global validation of ELISA and IIF both alone and combined was established by calculating sensitivity, specificity, accuracy and both positive predictive value and negative predictive value. The relationship between Oral Disease Severity Score values and ELISA titres was analysed using Pearson's coefficient. RESULTS: The best diagnostic performance was observed for anti-desmoglein 3 ELISA. The sensitivity was 75% and specificity 100% and positive predictive value and negative predictive value were 92.5% and accuracy 93.9%. The level of agreement with histopathology + direct immunofluorescence was substantial (k = .758). Anti-desmoglein 3 titres showed a significant correlation with Oral Disease Severity Score (p < .05). CONCLUSIONS: Serological tests are commonly employed during clinical practice as adjunctive tools. Anti-desmoglein 3 ELISA should be considered as a first-instance diagnostic test for oral pemphigus early detection.

Dermoscopy, confocal microscopy and optical coherence tomography features of main inflammatory and autoimmune skin diseases: A systematic review.

BACKGROUND/OBJECTIVES: Non-invasive skin imaging features of main skin inflammatory and autoimmune diseases have been reported, although a comprehensive review of their correlation with histopathologic features is currently lacking. Therefore, the aim of this paper was to review the correlation of dermoscopic, reflectance confocal microscopy (RCM) and optical coherence tomography (OCT) criteria of main inflammatory and autoimmune skin diseases with their corresponding histopathologic criteria correlation. METHODS: Studies on human subjects affected by main inflammatory and autoimmune diseases, defining the correlation of dermoscopic, RCM or OCT with histopathologic criteria, were included in the review. Five groups of diseases were identified and described: psoriasiform, spongiotic and interface dermatitis, bullous diseases and scleroderma. RESULTS: Psoriasiform dermatitis was typified by white scales, corresponding to hyperkeratosis, and vessels, observed with RCM and OCT. Spongiosis, corresponding to dark areas within the epidermis with RCM and OCT, was the main feature of spongiotic dermatitis. Interface dermatitis was characterised by dermoepidermal junction obscuration. Blisters, typical of bullous diseases, were visualised as dark areas with RCM and OCT while scleroderma lesions were characterised by dermoscopic fibrotic beams, related to dermal thickness variations, with specific OCT and histopathologic correlations. CONCLUSIONS: Although the role of RCM and OCT has yet to be defined in clinical practice, non-invasive skin imaging shows promising results on inflammatory and autoimmune skin diseases, due to the correlation with histopathologic features.

Immunophenotyping in pemphigus reveals a TH17/TFH17 cell-dominated immune response promoting desmoglein1/3-specific autoantibody production.

BACKGROUND: TH2 cells were thought to be a pivotal factor for initiation of the autoimmune blistering disease pemphigus. However, the role of other T-cell subsets in pemphigus pathogenesis remained unclear. OBJECTIVE: We aimed to characterize the exact phenotype of T cells responsible for the development of pemphigus. METHODS: Whole transcriptome shotgun sequencing was performed to determine differential gene expression in pemphigus lesions and skin of healthy individuals. The cutaneous cytokine signature was further evaluated by real-time quantitative PCR. In peripheral blood, the distribution of TH cell and folliclular helper (TFH) cell subsets was analyzed by flow cytometry. Finally, the capacity of TH and TFH cell subsets to induce desmoglein (Dsg)-specific autoantibodies by memory B cells was evaluated in coculture experiments. RESULTS: Transcriptome analysis of skin samples identified an IL-17A-dominated immune signature in patients with pemphigus, and Kyoto Encyclopedia of Genes and Genomes pathway analysis confirmed the dominance of the IL-17A signaling pathway. Increased expression of IL17A and associated cytokines was also detected by real-time quantitative PCR comparing lesional with perilesional or healthy skin. Interestingly, utilization of flow cytometry showed that patients with active pemphigus had elevated levels of circulating IL-17+, TH17, TFH17, and TFH17.1 cells. Notably, levels of TH17 and TFH17 cells correlated with levels of Dsg-specific CD19+CD27+ memory B cells, and patients with acute pemphigus showed higher levels of Dsg3-autoreactive TFH17 cells. Coculture experiments revealed TFH17 cells as primarily responsible for inducing Dsg-specific autoantibody production by B cells. CONCLUSION: Our findings show that TFH17 cells are critically involved in the pathogenesis of pemphigus and offer novel targets for therapeutic intervention.

Palliative care in dermatology: A clinical primer, review of the literature, and needs assessment.

Palliative care has been shown to improve quality of life, symptoms, and caregiver burden for a range of life-limiting diseases. Palliative care use among patients with severe dermatologic disease remains relatively unexplored, but the limited available data suggest significant unmet care needs and low rates of palliative care use. This review summarizes current palliative care patterns in dermatology, identifying areas for improvement and future investigation.

Subepithelial autoimmune bullous dermatoses disease activity assessment and therapy.

Subepidermal (subepithelial) autoimmune blistering dermatoses are a group of rare skin disorders characterized by the disruption of the dermal-epidermal junction through the action of autoantibodies. The fourth article in this continuing medical education series presents the current validated disease activity scoring systems, serologic parameters, treatments, and clinical trials for bullous pemphigoid, mucous membrane pemphigoid, epidermolysis bullosa acquisita, bullous systemic lupus erythematosus, anti-p200 pemphigoid, linear IgA bullous dermatosis, and dermatitis herpetiformis.

Therapeutic approaches and targets for treatment of autoimmune bullous diseases.

Autoimmune bullous diseases are a heterogeneous group of diseases characterized by the development of cutaneous and mucosal vesicles, blisters, and finally erosions. The common pathogenetic mechanism is the presence of autoantibodies targeting structural proteins of the skin and mucous membranes (demosomes and hemidesmosomes): in the case of pemphigus, the antigens are intraepidermal, whereas in the case of pemphigoid, dermatitis herpetiformis, and epidermolysis bullosa acquisita they are subepidermal. Mucosal involvement typically affects the oral and ocular mucosa, but in some cases, the upper airways or the upper digestive tract are affected. The burden on patients' lives could be severe due to the impairment of normal feeding or breathing. In other cases, they may represent paraneoplastic syndromes. Since autoimmune bullous diseases may result in significant morbidity and mortality, depending on the grade of cutaneous and mucosal involvement, a prompt therapeutic approach is mandatory and, in recalcitrant cases, may be challenging. The first line therapy consists of corticosteroids, both topical and systemic. Once remission or control of the acute phase is obtained, adjuvant therapies need to be introduced in order to spare the corticosteroid load and minimize side effects such as iatrogenic diabetes or osteoporosis. Herein, we describe all current therapeutic approaches to autoimmune bullous diseases, also including emerging therapies.

Therapy of autoimmune mouth bullous disease: 2020 review.

Most diseases of oral mucosa are either autoimmune in nature or are the results of immunologically mediated events. The diseases with autoimmune pathogenesis are namely pemphigus and pemphigoid; the oral involvement is frequent or regularly observed in these diseases. The treatments with traditional drugs or biologic agents or combinations of these molecules are employed in clinical practice. New therapeutic targets aim to provide new treatment strategies that may go beyond nonspecific immunosuppression.

Neonatal pemphigoid gestationis: An atypical presentation of a rare disease.

Bullous pemphigoid (BP) is an autoimmune blistering disease characterized by urticarial plaques and/or vesicles and tense bullae. A unique presentation of BP can occur during pregnancy, the postpartum period after delivery, or with the initiation of contraception, in which case it is referred to as pemphigoid gestationis (PG). In rare instances, newborns born to mothers with PG may also present with blisters due to transplacental passage of maternal anti-bullous pemphigoid 180 (BP180) or 230 (BP230) immunoglobulin G (IgG). In this report, we present an unusual case of neonatal PG in an infant born to an asymptomatic mother without a previous diagnosis of PG.

Treatment concerns for bullous pemphigoid in the COVID-19 pandemic era.

Bullous pemphigoid (BP) is the most common autoimmune blistering disease with subepidermal involvement, typically affecting the elderly. It has spontaneous remissions and exacerbations with significant morbidity. A novel coronavirus called severe acute respiratory syndrome coronavirus-2 (SARS-CoV-2) is responsible for the new universal coronavirus disease 2019 (COVID-19) pandemic. The pandemic made concerns, especially about immunosuppressive therapy. In this article, we reviewed the management of BP in the COVID-19 pandemic era. The data about the best management of autoimmune bullous diseases like BP, during the outbreak of COVID-19, are evolving and updated every day.