A Case of Vesiculobullous Dermatomyositis with Anti-NXP-2 Antibody without Malignancy.

Vesiculobullous dermatomyositis (VD) is a rare manifestation of dermatomyositis (DM) and has been suggested to be associated with malignancy. Although the myositis-specific autoantibodies are associated with distinct clinical presentations of DM, those associated with VD remain unclear. Here, we present the case of a 54-year-old man with VD who tested positive for anti-nuclear matrix protein 2 (NXP-2) antibody, one of the DM-specific autoantibodies. Serological and histopathological findings did not support autoimmune blistering disease. Physical and histological findings suggested that the severe edema in combination with the interface dermatitis of DM contributed to blister formation. Although a systemic examination was performed, no evidence of malignancy was found. Following initiation of immunosuppressive therapy, the patient showed significant improvement in both skin lesions and myositis. This case represents the first report of anti-NXP-2-positive VD without malignancy or autoimmune blistering disease. Subcutaneous edema, a characteristic feature of anti-NXP-2-positive DM, could be related to the formation of VD.

Proposal of a modified technique of Nikolsky's sign in oral autoimmune vesiculobullous diseases.

Nikolsky's sign, originally described for skin lesions, presents challenges when applied to the oral mucosa. To address this, a modified Nikolsky's sign has been proposed specifically for the oral mucosa. In this variant, a gentle breath of air from the air syringe embedded in the dental unit is used to inflate a pre-existing collapsed blister (non-induced technique). Alternatively, in the induced technique, a healthy peri-lesion mucosal site is gently scratched with a blunt dental tool, and after a few minutes, air is blown on the same area to inflate any newly formed blister. The sign is considered positive if a blister is raised from the blown surface. The described modified Nikolsky's sign improves the visualization of oral vesicles and blisters in a cost-effective, easy, and minimally invasive manner. Its elicitation can aid in referring patients to specialized tertiary care units.

Development of bullous systemic lupus erythematosus in patient treated with NB-UVB: A case report and comprehensive review of the literature.

Key Clinical Message: The use of phototherapy is highly effective in treating various skin diseases. In this study, the aim is to present vesicular and blister lesions in patients treated with UVB for psoriasis. It is advisable to consider the possibility of BSLE in cases of vesiculobullous lesions following phototherapy, along with other potential diagnoses. Abstract: Bullous systemic lupus erythematosus (BSLE) is a rare form of cutaneous lupus erythematosus that presents as vesicles and blisters on various parts of the body. The pathological appearance of these lesions often shows subepidermal vesicles with deposits of IgG, IgM, IgA, and complement C3 in granular or linear forms under direct immunofluorescence (DIF) examination. Clinical studies demonstrate the effectiveness of phototherapy in treating various skin conditions. While several studies suggest a correlation between phototherapy and the development of vesiculobullous lesions, most of these reports are related to bullous pemphigoid, with limited research on the occurrence of BSLE following phototherapy. In this case report, vesicular and blistering lesions in a 70-year-old man undergoing UVB treatment for psoriasis are described. Pathological examination confirmed the diagnosis of bullous systemic lupus erythematosus, and the patient experienced significant improvement after treatment with dapsone tablets. A literature review was conducted on the development of vesiculobullous lesions after phototherapy, comparing different approaches presented in previous studies. Our conclusion highlights the importance of considering BSLE as a possible diagnosis in cases of vesiculobullous lesions post-phototherapy, alongside other potential conditions.

A case of disseminated angiosarcoma-like Kaposi sarcoma presenting with unusual vesiculobullous lesions.

Angiosarcoma-like Kaposi sarcoma represents a recently delineated rare histomorphologic variant of Kaposi sarcoma that can be difficult to distinguish from other vasoproliferative lesions. Conventional lesions of Kaposi sarcoma encompass patches, plaques, and nodules; however, rarely vesiculobullous lesions have been described. Angiosarcoma-like Kaposi sarcoma has never been previously reported to present with vesiculobullous lesions. Herein, we describe a unique case of disseminated angiosarcoma-like Kaposi sarcoma with vesiculobullous lesions as the initial manifestation of human immunodeficiency virus infection.

Ocular involvement in oral vesiculobullous diseases: A review on reported cases in the literature.

Introduction: Vesiculobullous disorders are a group of autoimmune diseases manifesting as chronic ulcers in the oral cavity. Ocular involvement may accompany oral ulcers and cause various problems for patients. This review summarizes the data regarding ocular involvement in patients with oral vesiculobullous. Methods: Web of Science, Scopus, PubMed/MEDLINE, and Embase electronic databases were searched according to related keywords. Finally, 58 articles were included, all of which were case reports or series. Characteristics such as the age and sex of patients, location and type of oral lesion, type of ophthalmic injury, the interval between oral and ocular lesion, and treatment of oral and ocular disorders were summarized in tables. Results: Eye involvement was 1.6 times more prevalent in women, and most patients were between 30 and 60 years old (67.4 %). Pemphigus vulgaris accounted for almost half of the cases (48.4 %), though lichen planus is more prevalent in the general population. The most frequently affected oral site was the buccal mucosa (17.5 %), and oral ulcers usually presented as erythema, erosion, or inflammation (22.7 %). Conjunctivitis was the most common type of eye involvement (18.4 %), and ophthalmic lesions regularly appeared 12-60 months after the development of oral lesions (30.1 %). Blindness was reported in only one case. Corticosteroids and immunosuppressives were the most frequent oral and ocular lesion therapies. Conclusion: Considering the serious burdens of any ocular injury, monitoring the ocular health of patients with oral vesiculobullous diseases is highly recommended in high-risk cases, especially middle-aged women with oral pemphigus vulgaris.

Juvenile pemphigus vulgaris: Literature review and a rare case report.

Pemphigus vulgaris (PV) is a chronic autoimmune blistering disorder characterized by the loss of intraepithelial adhesion affecting the skin and mucous membranes, predominantly affects females in their fifth and sixth decades of life. Due to its rare occurrence in children and adolescents, there is often a delay in diagnosis and treatment in this age group. PV should always be considered in the differential diagnosis of oral ulcerative and vesiculobullous lesions in both children and adolescents.

A Bibliometric Analysis of Most Cited Papers on Vesiculobullous Oral Lesions.

Aim: A well-known method for quantitatively evaluating scholarly work is bibliometric analysis. Best-cited papers raise awareness of the influential publications and patterns in the literature on a specific subject. The aim was to conduct bibliometric analysis to determine most cited articles on vesiculobullous oral lesions. This is the first study on citation analysis with respect to vesiculobullous oral lesions. Materials and Methods: A retrospective data search was explored on December 2022 using the Scopus database. The articles were evaluated, and fundamental data for bibliometric analysis was reviewed. Standard details about the author, linked organizations, publishing year, and place of origin were noted. Statistical analysis was performed using Chi-square analysis. VOSviewer software was used to determine the bibliometric network analysis for co-occurrence among coauthors and commonly used keywords. Results: A total of 344 articles published from 1971 to 2022 were included in the study. A total of 6680 citations and 19.41 citations per article were observed. The journal Archives of Dermatology received the most citation. There was a significant association between the number of citations and the journal type (open access vs. non-open access) (P < 0.05). Four to five highly related clusters with the help of VOSviewer software were found during co-occurrence network analysis. Conclusions: The top 10 articles on vesiculobullous oral lesions that received the most citations were listed in detail in the present study. This will be a valuable resource for academics, clinicians, and researchers in the fields of dermatology, general pathology, oral pathology, and oral medicine.

A Case Series of Paederus Dermatitis: Understanding Its Varied and Diverse Clinical Presentations.

Introduction  Paederus dermatitis arises from inadvertent skin contact with insects of the genus Paederus, leading to irritant contact dermatitis. This study aims to highlight the diverse clinical presentations and the remarkable ability of the disease to mimic various dermatological conditions.  Methodology  A total of 15 patients diagnosed with Paederus dermatitis in a period of four months from August 2023 to November 2023 were included in this retrospective study. The demographic profile, detailed history, clinical presentation, and site of lesion distribution were documented.  Results  Out of 15 patients, nine were males, and eight were females. All patients exhibited a sudden onset of lesions accompanied by burning and pain, with an average duration of approximately 2.5 days. The most prevalent clinical presentation was the linear type, followed by kissing lesions, an erythematous patch with a central gray area, Nairobi eye, burnt appearance, and post-inflammatory pigmentation.  Conclusion  Paederus dermatitis is common in tropical areas like India but is prone to misdiagnosis due to its varied presentation. Increased awareness can lead to accurate diagnoses and simpler treatment plans, reducing patient confusion.

Direct Immunofluorescence of Skin and Oral Mucosa: Guidelines for Selecting the Optimum Biopsy Site.

Direct immunofluorescence is a vital diagnostic test for assessing vesiculobullous disorders, vasculitides, and connective tissue diseases. It is a robust and valuable technique that offers essential diagnostic information for many critical dermatoses. Dermatopathologists depend heavily on the data obtained from direct immunofluorescence evaluation to confirm final diagnoses. Selecting the most appropriate biopsy site is necessary for maximizing diagnostic accuracy, and the best site may vary depending on the clinical differential diagnosis. Inaccurate biopsy site selection can significantly impact the accuracy of the results. To optimize the use of direct immunofluorescence studies, this review provides helpful guidelines and some practical tips for selecting the best biopsy site.

Epidemiological and clinical study of cases of endemic pemphigus foliaceus and pemphigus vulgaris in a reference center in the state of Minas Gerais, Brazil

Abstract Background: Pemphigus constitutes a group of autoimmune bullous diseases. A reduction in the incidence of endemic pemphigus foliaceus and an increase in pemphigus vulgaris has been described, but there are no studies in Minas Gerais that address the subject. Objective: To describe the epidemiological and clinical profile of patients with pemphigus treated at the Dermatology Service of a public University Hospital in the state of Minas Gerais, Brazil. Methods: An observational, descriptive, and cross-sectional study was carried out of cases of endemic pemphigus foliaceus and pemphigus vulgaris, for a period of six months. A questionnaire was filled out with epidemiological and clinical data on the disease. Results: A total of 122 patients were included in the study, 64 with endemic pemphigus foliaceus and 58 with pemphigus vulgaris. When comparing patients with endemic pemphigus foliaceus and those with pemphigus vulgaris, a statistical difference was observed between the median age of initial disease manifestation (p = 0.001), patient occupation (p = 0.010), area of residence (p = 0.000), forests (p = 0.000) and rivers/streams close to the dwelling (p = 0.001) and the number of systemic medications required to control the disease (p = 0.002). When comparing patients with endemic pemphigus foliaceus to those evaluated in a study carried out at the same service in 2008, there was a statistical difference in the area of residence (p = 0.030). Study limitations: The assessed population comes from a tertiary care service that is not a reference for the entire state. Conclusions: Patients with endemic pemphigus foliaceus and pemphigus vulgaris maintain statistically significant differences regarding their main variables in the literature, such as age and area of residence. Historically, there has been a reduction in cases of endemic pemphigus foliaceus and an increase in cases of pemphigus vulgaris in this population.

Epidemiological and clinical study of cases of endemic pemphigus foliaceus and pemphigus vulgaris in a reference center in the state of Minas Gerais, Brazil.

BACKGROUND: Pemphigus constitutes a group of autoimmune bullous diseases. A reduction in the incidence of endemic pemphigus foliaceus and an increase in pemphigus vulgaris has been described, but there are no studies in Minas Gerais that address the subject. OBJECTIVE: To describe the epidemiological and clinical profile of patients with pemphigus treated at the Dermatology Service of a public University Hospital in the state of Minas Gerais, Brazil. METHODS: An observational, descriptive, and cross-sectional study was carried out of cases of endemic pemphigus foliaceus and pemphigus vulgaris, for a period of six months. A questionnaire was filled out with epidemiological and clinical data on the disease. RESULTS: A total of 122 patients were included in the study, 64 with endemic pemphigus foliaceus and 58 with pemphigus vulgaris. When comparing patients with endemic pemphigus foliaceus and those with pemphigus vulgaris, a statistical difference was observed between the median age of initial disease manifestation (p = 0.001), patient occupation (p = 0.010), area of residence (p = 0.000), forests (p = 0.000) and rivers/streams close to the dwelling (p = 0.001) and the number of systemic medications required to control the disease (p = 0.002). When comparing patients with endemic pemphigus foliaceus to those evaluated in a study carried out at the same service in 2008, there was a statistical difference in the area of residence (p = 0.030). STUDY LIMITATIONS: The assessed population comes from a tertiary care service that is not a reference for the entire state. CONCLUSIONS: Patients with endemic pemphigus foliaceus and pemphigus vulgaris maintain statistically significant differences regarding their main variables in the literature, such as age and area of residence. Historically, there has been a reduction in cases of endemic pemphigus foliaceus and an increase in cases of pemphigus vulgaris in this population.

Quality of systematic reviews on the treatment of vesiculobullous skin diseases. A meta-epidemiological study.

BACKGROUND: Systematic reviews of Randomized Controlled Trials (RCTs) are considered high-level evidence to support a decision on therapeutic interventions, and their methodological quality is essential to provide reliable and applicable results. OBJECTIVE: This meta-epidemiological study aimed to map and critically appraise systematic reviews assessing treatments for vesiculobullous skin diseases. METHODS: We conducted a comprehensive search strategy on MEDLINE (via Pubmed) in December 2022 without restrictions to find systematic reviews evaluating pharmacological interventions for vesiculobullous skin diseases. The methodological quality was assessed using the AMSTAR-2 tool, and additional information was extracted. We identified nine systematic reviews published between 2002 and 2021, seven assessing pemphigus. RESULTS: According to the AMSTAR-2 tool, 55.6% were classified as critically low quality, 22.2% as moderate quality, 11.1% as low and 11.1% as high quality. No review assessed the certainty of the evidence (GRADE); 86% of pemphigus reviews had at least two overlapping RCTs. There were some limitations regarding methodological flaws and the AMSTAR-2 tool use CONCLUSIONS: These findings reveal a frail methodological quality of systematic reviews about vesiculobullous diseases treatment that may impact the results. Therefore, methodological rigor is mandatory for future systematic reviews to avoid duplication of effort and increase the certainty of the evidence supporting decision-making.

Quality of systematic reviews on the treatment of vesiculobullous skin diseases. A meta-epidemiological study

Abstract Background Systematic reviews of Randomized Controlled Trials (RCTs) are considered high-level evidence to support a decision on therapeutic interventions, and their methodological quality is essential to provide reliable and applicable results. Objective This meta-epidemiological study aimed to map and critically appraise systematic reviews assessing treatments for vesiculobullous skin diseases. Methods We conducted a comprehensive search strategy on MEDLINE (via Pubmed) in December 2022 without restrictions to find systematic reviews evaluating pharmacological interventions for vesiculobullous skin diseases. The methodological quality was assessed using the AMSTAR-2 tool, and additional information was extracted. We identified nine systematic reviews published between 2002 and 2021, seven assessing pemphigus. Results According to the AMSTAR-2 tool, 55.6% were classified as critically low quality, 22.2% as moderate quality, 11.1% as low and 11.1% as high quality. No review assessed the certainty of the evidence (GRADE); 86% of pemphigus reviews had at least two overlapping RCTs. There were some limitations regarding methodological flaws and the AMSTAR-2 tool use Conclusions These findings reveal a frail methodological quality of systematic reviews about vesiculobullous diseases treatment that may impact the results. Therefore, methodological rigor is mandatory for future systematic reviews to avoid duplication of effort and increase the certainty of the evidence supporting decision-making.

Dental implants for patients with oral mucosal diseases: A narrative review and clinical guidance.

Oral mucosal diseases are a group of conditions that affect the oral mucosa with variable severity and include recurrent aphthous stomatitis (RAS), oral lichen planus (OLP), pemphigus vulgaris (PV), mucous membrane pemphigoid (MMP), and systemic lupus erythematosus (SLE). These may manifest clinically as painful oral ulcerations, reticulations and/or erosions, with differences between each. Management protocols often include initial topical and/or systemic corticosteroid (CS) therapy to control the patient's acute symptoms, followed by CS-sparing agents for long-term maintenance therapy. Patients with oral mucosal diseases often require dental implants to replace missing teeth. However, data on potential complications and success rates for these cases is still lacking. Considering the steady increase in the incidence of immune-related systemic conditions in the general population globally, dentists are expected to have the needed knowledge and ability to safely place dental implants in this group of patients. Therefore, this review aims to discuss the underlying pathogeneses of common oral mucosal diseases, clinical presentations, best practice approaches, and recommendations for the placement of dental implants in patients with similar conditions.