Autoimmune Bullous Disorders Research Articles

Case series of cutaneous myiasis in neglected cases of autoimmune bullous disorders.

Myiasis is an infestation of the tissues and organs of living vertebrates and humans by fly larvae, usually those belonging to the Calliphoridae family. The larvae feed on the host's necrotic or living tissue. Preexisting dermatological conditions and poor hygiene have been described as predisposing factors for myiasis, and it is especially common among neglected, dependent patients. Based on our literature search, we could find only a few case reports reported on cutaneous myiasis in autoimmune bullous disorders. It is especially more common in the South Indian Tamil Nadu population due to the false belief that autoimmune blistering disorders like pemphigus and bullous pemphigoid are considered chicken pox and treated with neem and turmeric preparation with poor hygiene. Herein we report a series of eight autoimmune blistering disorders with cutaneous myiasis.

Targeted serum proteome profiling reveals nicotinamide adenine dinucleotide phosphate (NADPH)-related biomarkers to discriminate linear IgA bullous disorder from dermatitis herpetiformis

Linear IgA bullous dermatosis (LABD) and dermatitis herpetiformis (DH) represent the major subtypes of IgA mediated autoimmune bullous disorders. We sought to understand the disease etiology by using serum proteomics. We assessed 92 organ damage biomarkers in LABD, DH, and healthy controls using the Olink high-throughput proteomics. The positive proteomic serum biomarkers were used to correlate with clinical features and HLA type. Targeted proteomic analysis of IgA deposition bullous disorders vs. controls showed elevated biomarkers. Further clustering and enrichment analyses identified distinct clusters between LABD and DH, highlighting the involvement of nicotinamide adenine dinucleotide phosphate (NADPH) oxidase. Comparative analysis revealed biomarkers with distinction between LABD and DH and validated in the skin lesion. Finally, qualitative correlation analysis with DEPs suggested six biomarkers (NBN, NCF2, CAPG, FES, BID, and PXN) have better prognosis in DH patients. These findings provide potential biomarkers to differentiate the disease subtype of IgA deposition bullous disease.

Nail changes in pemphigus: a systematic review and meta-analysis.

Pemphigus is a group of autoimmune mucocutaneous bullous disorders characterized by acantholysis resulting from autoantibodies targeting epithelial cell surface antigens. Studies reflect the presence of nail manifestations in some patients and suggest a potential correlation with clinical severity. This study examines the overall prevalence and characterizes the diverse manifestations of nail changes in pemphigus. We searched Cochrane, MEDLINE, EMBASE, and LILACS from 1990 to June 26, 2023 for studies reporting different nail changes in pemphigus patients. Data were collected and pooled to obtain proportions of the prevalence of nail changes in patients with pemphigus and subgroup analysis for pemphigus foliaceous and pemphigus vulgaris. The risk of bias was assessed with the Joanna Briggs Institute Checklist. Of 321 studies screened, 14 studies with 1,208 patients were included. Paronychia (n = 185) and Beau's lines (n = 104) were the most common nail changes identified. The pooled prevalence of nail disease in pemphigus patients was 0.389 (number of studies; [95% CI]: n = 9; [0.160-0.680], with high heterogeneity between studies (I2 = 95.0%, P < 0.001). Subgroup analysis revealed the highest prevalence in pemphigus foliaceous at 0.342 (n = 3; [0.109-0.688]) and pemphigus vulgaris at 0.396 (n = 5; [0.114-0.769]). Nail changes exhibited varied temporal relationships with disease onset and flares, preceding, concurrent, or following these events. Correlation with disease severity was noted, although discrepancies between studies were reported. Nail changes in pemphigus, particularly pemphigus vulgaris and pemphigus foliaceous, may be underrecognized. Observations regarding temporal associations and potential correlations with disease severity highlight the diagnostic and prognostic implications of nail changes in pemphigus. The limitations of this study include study heterogeneity and possible bias. Further research to establish the correlation of the presence and severity of nail changes on the overall disease course would be helpful.

Current Knowledge on Nail Involvement in Autoimmune Bullous Disorders.

There are few studies, mainly case reports, on the involvement of the nail unit in autoimmune bullous disorders. Nail involvement in autoimmune bullous disorders is a significant clinical phenomenon, marked by a range of manifestations, most often not presenting with blisters like on the skin but rather with alterations of the nail unit such as paronychia, onychomadesis, or onycholysis. This involvement is particularly notable due to the unique immunological features of the nail unit, including the expression of various antigens and the presence of Langerhans cells. Conditions like pemphigus vulgaris, bullous pemphigoid, epidermolysis bullosa acquisita, linear IgA disease, and bullous systemic lupus erythematosus can lead to nail abnormalities. The prevalence of nail manifestations varies according to the disorder, and diagnosis often relies on histopathological and immunofluorescence testing. Nail involvement correlates with disease severity and duration, sometimes serving as a herald sign. Further research is needed to guide therapeutic approaches for nail involvement in autoimmune bullous diseases. Nail involvement in autoimmune bullous nail disorders may be confusing as there are almost never bullae. One should keep the diagnosis in mind when facing an atypical paronychia.

A-270 - Autoimmune Bullous Disorders induced by Cyclin-Dependent Kinase 4/6 inhibitors – first case series from the EADV Task Force “Dermatology for Cancer Patients”

A-270 - Autoimmune Bullous Disorders induced by Cyclin-Dependent Kinase 4/6 inhibitors – first case series from the EADV Task Force “Dermatology for Cancer Patients”

Autoimmune bullous disorder flares following severe acute respiratory syndrome coronavirus 2 vaccination: a case series

BackgroundAutoimmune bullous disorders develop due to autoantibodies targeting intercellular adhesion proteins of hemidesmosomes and desmosomes and may be triggered by viral infections and vaccines. Recent reports suggest that the coronavirus disease 2019 vaccination may trigger flares or exacerbations of preexisting autoimmune diseases, including new onset autoimmune bullous disorders. There are less data on whether vaccination against severe acute respiratory syndrome coronavirus 2 may also exacerbate preexisting autoimmune bullous disorders.Case presentationHere we present three cases, two white males (ages 69 years and 88 years) with bullous pemphigoid and one white 50-year-old female with pemphigus foliaceus, wherein all individuals developed minor, tractable flares of their preexisting autoimmune bullous disorders after receiving the coronavirus disease 2019 vaccination, which were readily treatable with topical or low-dose systemic corticosteroids.ConclusionsDermatologists managing patients with autoimmune bullous disorders should be cognizant of the uncommon potential for flares of the disorder following vaccination for severe acute respiratory syndrome coronavirus 2. Flares of bullous pemphigoid and pemphigus foliaceus following vaccination for severe acute respiratory syndrome coronavirus 2 in these cases were mild and tractable.

The Management of Autoimmune Bullous Skin Disorders in the Era of COVID-19 Pandemic: A Single Center, Retrospective, Cross-sectional Study

The Management of Autoimmune Bullous Skin Disorders in the Era of COVID-19 Pandemic: A Single Center, Retrospective, Cross-sectional Study

TZANCK SMEAR: A USEFUL DIAGNOSTIC TOOL IN SPECTRUM OF VESICULOBULLOUS DISEASES OF SKIN.

Introduction: The pemphigus group of autoimmune bullous disorders and mucocutaneous herpesvirus infections are generally diagnosed with the Tzanck smear. Only a few research have looked into its diagnostic value in the literature. To evaluate tzanck smear ndings in patients Objectives: with vesiculobullous skin lesions and histopathological correlation. All recei Material and Methods: ved Samples received between 01/08/2021 to 31/01/2022 were stained with May-Grunwald-Giemsa. In some patients, methylene blue staining and ZN staining were done. Tzanck Results: smear was performed on a total of 25 patients with skin lesions. The sensitivities of multinucleated giant cells and acantholytic cells in herpetic infections, dyskeratotic acantholytic cells,inammatory cells and cocci in bullous impetigo, acantholytic cells with neutrophils and lymphocytes in pemphigus foliaceous, acantholytic cells and plenty of neutrophils in drug-induced pemphigus, no acantholytic cells with neutrophils and lymphocytes in IgA pemphigus, cluster of acantholytic cells in pemphigus vulgaris, only lymphocytes with no acantholytic cells in bullous pemphigoid and dyskeratotic acantholytic cells without inammation in staphylococcal scalded skin syndrome, intracytoplasmic HendersonPatterson bodies and anucleated squamous cells in molluscum contagiosum and broblast, leucocytes with necrotic basal cells in Toxic epidermal necrolysis. The tzanck smear test is an inexpensive, useful, quick, reliable Conclusion: and an easy diagnostic tool for certain vesiculobullous skin diseases.

Pruritic crusted lesions in an 89‐year‐old woman

Pruritic crusted lesions in an 89‐year‐old woman

B-cell depletion in the treatment of pemphigus: two clinical cases and literature review

Pemphigus presents a group of chronic autoimmune bullous skin disorders with well-known clinical signs, which pathophysiology is mediated by antibodies to various epidermal self-antigens. For a long time, the only therapeutic option for this disease was the lifelong use of non-selective immunosuppressive agents limited by high rate of severe adverse reactions. The article presents two clinical cases of rituximab use (a targeted agent leading to B-cell depletion) in patients with pemphigus (vulgaris and foliaceous) who were previously resistant to high-dose steroid therapy. Treatment with rituximab lead to response and allowed to decrease the prednisone dose in both cases. These results confirm that rituximab can be successfully used in pemphigus as adjuvant therapy if conventional agents for this dermatosis are ineffective.

Acrodermatitis Continua of Hallopeau: A Diagnostic Challenge.

Acrodermatitis Continua of Hallopeau: A Diagnostic Challenge.

Dermatological Manifestations in the Elderly: A Descriptive Cross-Sectional Study

Background: Aging is mainly due to chronic interaction between extrinsic and intrinsic factors. The elderly population is highly vulnerable to skin diseases due to immunosenescence, polypharmacy, chronic exposure to ultraviolet radiation, and inadequate nutrition. Objectives: (1) To estimate the prevalence of dermatological manifestation in the elderly population. (2) Clinico-pathological correlation of the cutaneous tumors seen in the study population. Materials and Methods: A total of 300 elderly patients (above 60 years) who attended the outpatient department and inpatients of a tertiary care hospital, were recruited for the study, during a 1 year period (2019–2020). Patient demographics, brief history, and clinical examination were done. Relevant laboratory investigations, including side laboratory procedures and skin biopsy were done when necessary. Results: Most of the patients were between 65 and 70 years, 175 were male and 125 were female. Type-2 diabetes mellitus and hypertension had equal incidence. Dermatophyte infection was the most common presentation, followed by eczema, papulosquamous, and auto-immune bullous disorders. Nail changes were observed in 50% of the patients. Among those who had drug reactions, 50% had drug hypersensitivity syndrome to anti-tubercular drugs. Malignancies, including basal cell carcinoma, were not uncommon. Alcohol, sunlight, and the male gender were associated with an increased risk of wrinkling. Conclusion: Health education programs regarding skin disorders and preventive measures in the geriatric population will help improve their quality of life.

Role of neutrophil-to-lymphocyte, neutrophil-to-eosinophil and platelet-to-lymphocyte ratios in the diagnosis of bullous pemphigoid and Pemphigus disease.

Bullous pemphigoid (BP) and "Pemphigus diseases" (PD) can have overlapping clinical manifestations and accurate distinction is crucial for appropriate management. The study aimed at analyzing the utility of simple hematological markers of systemic inflammation like neutrophil-to-lymphocyte ratio (NLR), neutrophil-to-eosinophil ratio (NER), and platelet-to-lymphocyte ratio (PLR) in clinical decision making in the setting of clinical differentials of BP and PD in a particular case. This single-centre based retrospective observational analytical study included adult subjects newly diagnosed to have BP (n=66) or PD (n=53), confirmed with direct immune-fluorescence testing, over a period of six years. Blood counts performed using Coulter™ hematology analyser, at the time of their initial presentation, were retrieved from the hospital medical records, and the leucocyte ratios were calculated. The data were compared between the two groups, using Mann-Whitney U test and chi-square test /Fisher's exact test. ROC curve analysis was performed to estimate cut-off values. The BP group had a significantly higher NLR, total leukocyte counts (TLC), absolute eosinophil counts (AEC), and absolute lymphocyte counts (ALC), and lower NER values compared to the PD group (P < 0.05). Areas under ROC for NLR, NER, TLC, AEC, and ALC were between 0.5 and 0.7. NLR ≥ 7, AEC ≥ 2055/cumm, and TLC ≥ 15,000/cumm had a specificity of 90.6, 100, and 100% respectively for identifying BP patients out of the two groups, but with a low sensitivity of 22.7, 21, and 22.7%, respectively. NLR can be a valuable diagnostic adjunct in subtyping autoimmune bullous disorders, albeit in a small proportion of cases.

Targeting heat shock proteins 90 and 70: A promising remedy for both autoimmune bullous diseases and COVID-19.

Heat shock proteins (Hsps), including Hsp90 and Hsp70, are intra- and extracellular molecules implicated in cellular homeostasis and immune processes and are induced by cell stress such as inflammation and infection. Autoimmune bullous disorders (AIBDs) and COVID-19 represent potentially life-threatening inflammatory and infectious diseases, respectively. A significant portion of AIBDs remain refractory to currently available immunosuppressive therapies, which may represent a risk factor for COVID-19, and suffer from treatment side-effects. Despite advances in vaccination, there is still a need to develop new therapeutic approaches targeting SARS-CoV-2, especially considering vaccine hesitancy, logistical distribution challenges, and breakthrough infections. In this mini review, we briefly summarize the role of targeting Hsp90/70 as a promising double-edged sword in the therapy of AIBDs and COVID-19.

Messenger RNA-1273 Moderna COVID-19 Vaccination as Potential Trigger for Case of New Onset Pemphigus Vulgaris

As vaccination against Coronavirus 2019 (COVID-19) remains a worldwide initiative, so does describing its associated adverse effects. While there have been many reports detailing various dermatologic adverse events, autoimmune bullous disorders following vaccination are less well described.1 Pemphigus vulgaris (PV) is an autoimmune bullous disease of the skin and mucous membranes that has many known triggers, including vaccination.2 To our knowledge, there are at least six cases of pemphigus vulgaris developing following SARS-Cov-2 vaccination.3 Here we describe a case of new onset PV following COVID-19 vaccination with the mRNA-1273 (Moderna) vaccine. Because this disease carries significant morbidity and mortality, we hope this report serves as a reminder that while vaccination remains a global priority, continued documentation and recognition of novel vaccination side effects by clinicians is essential.

An observational study to determine the role of indirect immunofluorescence and ELISA for desmogleins in the diagnosis and monitoring of autoimmune bullous disorders

BackgroundAutoimmune bullous disorder (AIBD) is a diverse group of blistering dermatoses that affects the skin and mucous membrane, characterized by the formation of autoantibodies against the desmosomal glycoproteins and adhesion molecular components of the basement membrane zone. Various immunoassay techniques for serological diagnosis are Direct Immunofluorescence (DIF), Indirect Immunofluorescence (IIF), Enzyme Linked Immunosorbent Assay (ELISA) and immunoblotting. Quantitative ELISA titer can also be used to monitor the disease activity and response to treatment. The aim of this study was to evaluate the role of IIF in diagnosing various AIBDs and the role of ELISA for desmogleins in monitoring disease activity in the pemphigus group of disorders. MethodsA total of 45 patients with freshly diagnosed AIBD were included in the study. Skin biopsies were done to establish the diagnosis by histopathology and DIF. BIOCHIP-based IIF assay was performed on the sera of the patient. The sensitivity and specificity of the IIF assay were then calculated based on the established diagnosis by Histopathological examination (HPE) and DIF. Quantitative ELISA titer was performed to measure the antibody (anti desmoglein (Dsg) 1 and 3) titre in serum samples of patients with pemphigus at baseline, 1 and 3 months follow-up after initiation of appropriate treatment therapy and results were then compared with disease activity scoring system. ResultsSensitivity and specificity of IIF for Dsg 1 for the pemphigus group were derived to be 85.7% and 98.2% respectively, while that for Dsg 3 were 100% and 98.7%, respectively. Sensitivity (90–100%) of IIF for Bullous pemphigoid was higher than specificity (85–96%). The ELISA titers of anti Dsg1 and 3 were also observed to fall sequentially so was the disease activity score during the follow-up visits at 1 and 3 months. ConclusionBiochip IIF assay can be used as a screening tool for the serological diagnosis of AIBD and quantitative ELISA for monitoring the disease activity and response to treatment.

Pediatric autoimmune blistering disorders - a five-year demographic profile and therapy experience.

There is a paucity of literature on pediatric autoimmune bullous disorders (AIBD) in the Indian population. We aimed to retrospectively evaluate the clinico-demographic profile of pediatric AIBDs in our patients and their response to various therapeutic modalities. This was a retrospective chart review of patients enrolled in our immunobullous disease clinic from November 2013 to August 2019. The clinical records of all the patients aged less than 18 years old with a definitive diagnosis of AIBD were reviewed based on clinical, histopathological, and immunological features. Forty out of 1209 patients with AIBD (3.3%) belonged to the pediatric age group. Pemphigus vulgaris (PV) was the most common AIBD (24, 60%) followed by chronic bullous disease of childhood (CBDC) at 15% (6) and pemphigus foliaceus (PV) at 12.5% (5). Subepidermal blistering disorders had a significantly younger age of onset (P = 0.04) compared to intraepidermal blistering disorders but higher frequency of achieving complete remission off therapy (P = 0.02). The mean time to achieve remission was significantly shorter in PV patients treated with a combination of rituximab and corticosteroids compared to those treated with oral prednisolone and oral immunosuppressive adjuvants (P = 0.001). Rituximab was tolerated well in all 12 pemphigus patients. Oral lesions in PV patients took significantly longer time to achieve remission compared to the cutaneous lesions (P = 0.001). PV was the most common pediatric AIBD in Indian patients. Rituximab was a safe and effective modality of treatment in moderate to severe pediatric pemphigus.

A 72-year-old man with nonhealing facial erosions and bullae

A 72-year-old man with nonhealing facial erosions and bullae

Current and Innovated Managements for Autoimmune Bullous Skin Disorders: An Overview.

Autoimmune bullous skin disorders are a group of disorders characterized by the formation of numerous blisters and erosions on the skin and/or the mucosal membrane, arising from autoantibodies against the intercellular adhesion molecules and the structural proteins. They can be classified into intraepithelial or subepithelial autoimmune bullous dermatoses based on the location of the targeted antigens. These dermatoses are extremely debilitating and fatal in certain cases, depending on the degree of cutaneous and mucosal involvement. Effective treatments should be implemented promptly. Glucocorticoids serve as the first-line approach due to their rapid onset of therapeutic effects and remission of the acute phase. Nonetheless, long-term applications may lead to major adverse effects that outweigh the benefits. Hence, other adjuvant therapies are mandatory to minimize the potential harm and ameliorate the quality of life. Herein, we summarize the current therapeutic strategies and introduce promising therapies for intractable autoimmune bullous diseases.

Comorbidities in Patients with Autoimmune Bullous Disorders: Hospital-Based Registry Study.

The incidence of autoimmune bullous disorders has increased over the years, especially in elderly patients with multiple comorbidities, which has stimulated research into their association with other diseases. We performed a retrospective observational study used the Minimum Basic Data Set of hospital discharges to review records of patients admitted to Spanish public hospitals between 2016 and 2019 with a diagnosis of any autoimmune bullous disorder. The objectives were to describe the comorbidity profile and the clinical-epidemiological characteristics of patients with pemphigus and pemphigoid, and analyze the evolution of the incidence of these diseases. The study included 1950 patients with pemphigus and 5424 patients with pemphigoid. Incidence increased from 2016 to 2019. The main comorbidities were hypertension (40.19%) and diabetes mellitus (28.57%). Compared to patients with pemphigoid, those with pemphigus had a higher prevalence of neoplasms, osteoporosis, solid metastases and malignant lymphoma, while the prevalence of hypertension, kidney disease, diabetes, heart failure, dementia, chronic obstructive pulmonary disease and Parkinson’s disease was higher in the pemphigoid group (p < 0.05). Therefore, since autoimmune bullous disorders are associated with diverse comorbidities and their incidence has risen in recent years, the establishment of strategies to prevent the main comorbidities in these patients is justified.