Tense Blisters Research Articles

Description of a clinical case of bullous scleroderma in a somatically impaired patient

Bullous scleroderma is a rare variant of localized scleroderma characterized by the formation of subepidermal tense blisters. At the moment, the etiology and pathogenesis of dermatosis is not fully understood, but a number of authors consider the pathology of the endocrine system as a trigger for the development of localized and systemic scleroderma. Untimely or erroneous diagnosis of bullous scleroderma leads to the risk of developing a systemic process, irreversible cosmetic defects and even disability. Treatment of bullous scleroderma should be complex and multicourse with the inclusion of systemic glucocorticoids, antibacterial and cytostatic drugs, agents that improve microcirculation, as well as topical glucocorticoids of the 3-4th class of activity, regenerating agents. Physiotherapeutic methods of treatment such as PUVA and UVA therapy are widely used; there are reports on the successful use of a combination of immunosuppressive mycophenolate mofetil and extracorporeal photopheresis, as well as intravenous infusions of N-acetylcysteine. In addition to traditional drug and physiotherapy therapies, the use of biological therapies is currently being considered. The article presents a rare case of scleroderma bullosa on the background of pronounced chronic endocrine pathology. The interest of this clinical case lies not only in the rare form of the disease and the progressive nature of the course of dermatosis, but also in the probable association of dermatosis with the patient's endocrine gland diseases in the form of panhypopituitarism due to adenohypophysis macroadenoma with the development of secondary hypothyroidism and adrenal insufficiency. In our observation, the steady negative dynamics of the skin process — from localised plaque scleroderma with subsequent transformation into the bullous form, and further into a probable systemic process — drew attention. Taking into account the severity of the disease course, the presence of concomitant chronic endocrinopathy, as well as positive specific serological reactions for the systemic form of scleroderma, in the presented clinical case the patient needs an interdisciplinary approach and constant dispensary observation.

Tense blisters and hemorrhagic bullae as the first manifestation of eosinophilic granulomatosis with polyangiitis.

Eosinophilic granulomatosis with polyangiitis (EGPA) poses a significant diagnostic challenge due to its varied clinical presentation. Here, we present a case of a 59-year-old female with a history of asthma and sinusitis, who manifested with an extremely rare presentation of drastic tense blisters and hemorrhagic bullae alongside purpuric lesions and peripheral neuropathy. Examinations revealed eosinophilia, positive anti-neutrophil cytoplasmic antibody, and characteristic pathological findings with small vessel vasculitis in the purpura. Treatment with glucocorticoids and cyclophosphamide led to rapid improvement in peripheral eosinophilia, skin manifestations and motor neuron deficits. Although rare, our case underscores that bullous skin lesions should be recognized as a potential cutaneous hallmark of EGPA to aid timely diagnosis, since prompt treatment initiation is crucial given the potential irreversible organ damage and poor prognosis of EGPA.

Case Report: Bullous Pemphigoid and the Association with Dipeptidyl-peptidase 4 Inhibitors

<p>大皰性類天皰瘡(bullous pemphigoid)是一種自體免疫表皮下水泡疾病，表現為全身性瘙癢性蕁麻疹斑塊和緊繃的表皮下水泡。發生率大約每百萬人年為4至22例個案，而且有逐漸增加之趨勢。這種疾病最常影響老年人，除了老年化社會增加罹患風險外，神經疾病增加、與可能誘發類天皰瘡之藥物使用增加皆與該疾病增加的趨勢有關。</p> <p>本個案為一位90歲的女性，過去疾病包括小腦出血性中風、帕金森氏症、失智症、高血壓、及糖尿病。糖尿病於一年前新診斷並開始使用 linagliptin及glimepiride等藥物控制血糖。主訴近半年多來開始會有全身的癢疹，尤其是軀幹及四肢，兩週前開始在皮膚縐摺處出現水泡及破裂傷口，經過臨床檢驗後診斷為大皰性類天皰瘡，搜索相關文獻後懷疑與 Linagliptin 的使用有關，於是改用其他藥物控制血糖，並開始使用外用及口服類固醇治療。經治療後皮膚明顯改善，不再有新水泡產生。本個案報告簡介大皰性類天皰瘡，亦討論該疾病與降血糖藥物dipeptidyl-peptidase 4 inhibitors (DPP4-inhibitor)之關係，研究發現某些類型的DPP4-inhibitor例如linagliptin與大皰性類天皰瘡的發生機率相關。</p> <p> </p><p>Bullous pemphigoid is an autoimmune subepidermal blistering disease characterized by generalized pruritic urticarial plaques and tense subepidermal blisters. The incidence is approximately 4 to 22 cases per million people per year, with a rising trend. This disease predominantly affects the elderly, and the increasing trend is attributed to the aging population, rising incidence of neurological disorders, and the use of medications that may trigger bullous pemphigoid.</p> <p>This case is a 90-year-old female with a medical history of cerebellar hemorrhagic stroke, Parkinson’s disease, dementia, hypertension, and diabetes. Diabetes was newly diagnosed a year ago, and medications like linagliptin and glimepiride were initiated for blood sugar control. The patient complained of generalized itching and rash for the past six months, particularly on the trunk and limbs. Two weeks ago, blisters and ruptured wounds appeared in the skin folds. After clinical examination, the diagnosis of bullous pemphigoid was made. After reviewing relevant literature, it was suspected to have connection with the use of linagliptin and bullous pemphigoid. We shifted linagliptin to other drugs to control blood sugar, and topical and oral corticosteroid treatment was initiated. The skin significantly improved after treatment. This case report not only introduces bullous pemphigoid but also discusses the association between this disease and dipeptidyl-peptidase 4 inhibitors (DPP-4 inhibitors). Research suggests a correlation between certain types of DPP-4 inhibitors, such as linagliptin, and the occurrence of bullous pemphigoid.</p> <p> </p>

Childhood Bullous Pemphigoid with Atypical Immunopathology: A Case Series

Bullous pemphigoid (BP) is a rare autoimmune blistering disorder primarily affecting older adults, with limited occurrences in children. BP in children typically manifests as large, tense blisters on the skin, often on flexural areas. It also more often affects the oromucosal areas and the face in children than in adults. Diagnosis involves histopathological examination revealing eosinophilic spongiosis or subepidermal split, immunofluorescence tests highlighting immunoglobulin G (IgG) and C3 depositions, and immunological assays detecting BP180 and BP230 IgG autoantibodies. This report presents two cases of childhood BP (CBP) with atypical immunopathological findings. Clinically, the two cases had generalized plaques and bullae, including the face. The first case exhibited the characteristic linear deposits of IgG and C3 on the basement membrane through direct immunofluorescence (DIF) and revealed negative anti-BP180 antibodies on enzyme-linked immunosorbent assay (ELISA). In contrast, the second case showed negative DIF results, despite clinical suspicion, but had positive anti-BP180 IgG antibodies on ELISA. It is, therefore, crucial to consider the complete clinical presentation of the patient, in conjunction with the histological findings and immunopathologic assessments to diagnose CBP.

Atypical Presentation of Infantile Bullous Pemphigoid with Isolated C3 Deposits

Bullous pemphigoid is an acquired autoimmune bullous disorder with autoantibodies targeting distinctive antigenic components of the skin and mucous membrane. It occurs mostly in the elderly, with the incidence of disease increasing with age and rare in children and infants. The clinical features are extremely polymorphous characterized by tense blisters and intense itching. The disease in pediatric population usually has a favorable outcome with appropriate treatment. Here, we present a case of infantile bullous pemphigoid with a rare presentation, triggered by vaccination and showing only linear C3 deposits.

The pathological function of neutrophils in pemphigoid diseases

Abstract Pemphigoid diseases (PDs) are a group of autoimmune blistering diseases, including bullous pemphigoid, epidermolysis bullosa acquisita, mucous membrane pemphigoid, linear immunoglobulin A disease, and other rare variants. These diseases are characterized by the presence of autoantibodies that target proteins at the dermal-epidermal junction, resulting in the formation of tense blisters and erosions on the skin and/or mucosa. The current therapeutic approaches, such as systemic corticosteroid, are associated with significant adverse effects, highlighting that safer and more effective treatment options are an urgent clinical need. To address this unmet need, a comprehensive understanding of the detailed mechanisms underlying PDs is essential. Based on their histopathological infiltration in pemphigoid lesions, neutrophils have long been implicated as major contributors to the initiation and progression of the diseases. Numerous in vivo and in vitro studies have investigated the role of neutrophils in the pemphigoid pathology, revealing various pathological mechanisms induced by these cells, including the release of neutrophil elastase and matrix metalloproteinase-9, as well as the formation of neutrophil extracellular traps. The present review provides a comprehensive summary and critical evaluation of the current understanding regarding the role of neutrophils in PDs. In addition, it discusses the potential of targeting neutrophil-associated pathways as a novel therapeutic approach for the diseases.

Low pH condition impairs BP-IgG binding to the basement membrane zone.

Bullous pemphigoid (BP), an autoimmune subepidermal blistering disease, shows tense blisters associated with urticarial erythema. Tissue-bound Immunoglobulin G (IgG) at the basement membrane zone (BMZ) detected by direct immunofluorescence (DIF) is strong evidence for a diagnosis of BP. The sensitivity of DIF is higher in complement component 3 (C3) than in IgG, but the reason for this different sensitivity is not fully understood. In this study, we performed several exvivo studies to investigate the possible mechanism of IgG negativity and C3 positivity at the BMZ by DIF in some BP cases. First, sera from BP patients showing IgG negativity by DIF were found to clearly react to the BMZ in their own DIF skin samples. Next, indirect immunofluorescence (IIF) was performed using sera diluted with different pH phosphate-buffered saline (PBS), pH 7.4, 6.0, and 3.0. Patients' sera diluted with pH 7.4 PBS showed linear staining at the BMZ, but sera diluted with pH 6.0 PBS and pH 3.0 PBS showed lower fluorescence intensities. Finally, sections of skin from BP patients were pre-incubated with different pH PBS (pH 3.0, 6.0, and 7.4), followed by staining with anti-human IgG and C3. The fluorescence intensities were notably lower for IgG and C3 that had been pre-incubated with pH 3.0 PBS and pH 6.0 PBS than for IgG and C3 that had been pre-incubated with pH 7.4 PBS. These results suggest that a low pH condition hinders the binding of autoantibodies to the BMZ, that is, the drop in tissue pH induced by inflammation inhibits autoantibodies from depositing at the BMZ. Furthermore, the drop in tissue pH causes tissue-bound autoantibodies to detach from the BMZ. Complement fragments are activated not only on IgG but also on the cell surface of cells close to IgG during complement activation. IgG may detach from the BMZ under low pH condition induced by inflammation, but some complement fragments remain at the BMZ. These phenomena may help to explain why C3 is more sensitive than IgG when DIF is used to diagnose BP.

Return to Work Program for a Chef with Pemphigus Vulgaris : a Case Report

Background: Pemphigus vulgaris is an autoimmune, intraepithelial, blistering disease, affecting the skin and mucosae, is defined by IgG antibody deposits in the keratinocyte membranes of the epidermis, leading to acantholysis with consequent generation of blisters. This disease has recurrence characteristics triggering by various risk factors such as medications, physical agents, infectious agents, hormones, and stress. The aim of this case report is to manage a return to work program for pemphigus vulgaris patient with numerous, widespread, blisters especially on both hands.Case presentation: Fourty-three years old woman presents with a painful and itchy blisters appeared in the palms, feet and body surfaces which are widely spreading throughout this week. The patient reported that the lesions first appeared as an ulcer in the upper lip, followed by cutaneus involvement. On physical examination, vital signs are normal and nutritional status is overweight. Dermatology status found numerous, widespread, tense or flaccide blisters, erosions and pruritic erythema on chest, back, abdomen, both arms and both palms accompanied by black crust with a soliter erosions on the upper lip. She was diagnosed with Pemphigus Vulgaris since 10 December 2020 and was controlled very well since February 2021, when suddenly new blisters started to appear on May 2022.She has got a medical and occupational management for her disease, which let her recovered and ready to go back to work again. Conclusion: Pemphigus Vulgaris could affect patient’s work life. Multiple hazard in the work place also can affect the nature of the disease. Occupational management is important to help patients return to their former work, and to prevent reccurence. As a return to work program, the author recommended several changes in the workplace to reduce the triggering factor, promote self care, did an education, and showed the way to work with a good ergonomic style. Work related stress which act as an exogenous factor triggering Pemphigus Vulgaris need to be controlled. With appropriate medications and interventions to control her occupational risk and exposure, patients can return to work with some modifications.

Mucous membrane pemphigoid involving palmoplantar lesions that developed during adjuvant nivolumab for malignant melanoma: A rare case and literature review

Immune checkpoint inhibitors (ICIs), such as nivolumab, which target anti‐programmed cell death-1 (PD-1), have been applied to a variety of cancers and have caused immune‐related adverse events (irAEs). Although an association between ICIs and bullous pemphigoid and mucosal pemphigoid (MMP) has been reported, the complex mechanisms underlying PD-1 inhibition‐induced autoantibody production and autoimmunity remain unelucidated. In this report, we present a unique case of MMP involving the palmoplantar lesions during adjuvant nivolumab therapy. A Japanese woman in her 70s was treated with nivolumab for postoperative vulvar malignant melanoma; after the 12th cycle of treatment, ulcers were seen in the oral cavity, and 4 months later, tense palmoplantar blisters appeared. Microscopic examination of the palmar blister revealed subepidermal vesicles characterized by eosinophil infiltration. Immunofluorescence analysis revealed linear IgG and C3 deposits along the basement membrane; ELISA testing confirmed the presence of anti‐BP180 NC16A IgG antibody, and MMP was diagnosed. The patient’s condition gradually improved with a therapeutic regimen of corticosteroids and immunoglobulins. A review of 10 cases of ICI‐associated MMP, including the present case, revealed clinical similarities to conventional MMP; in cases of persistent oral erosions due to ICI, it is most important to consider mucositis and MMP in the framework of differential diagnosis. Besides, we suggest that we can be more suspicious of MMP by paying attention to examining the palms and soles when the patient receiving ICIs has refractory oral ulcers. This report underscores the importance of careful observation and prompt management of irAE as cancer immunotherapy evolves.

A Literature Review and Clinical Consensus Guidelines on the Management of Bullous Pemphigoid

Abstract Bullous pemphigoid (BP) is the most common autoimmune blistering disease primarily characterized by tense blisters and occasionally with urticarial plaques, affecting the skin and mucous membranes. These are caused by autoantibodies against BP180 and BP230 which target antigens on the basement membrane zone. The diagnosis relies on the integration of clinical, histopathological, immunopathological, and serological findings. The management depends on the clinical extent and severity. We present in this article a literature review and the clinical consensus guidelines of the Immunodermatology Subspecialty Core Group of the Philippine Dermatological Society in the management of BP.

Pemphigoid diseases in older adults

Pemphigoid diseases are agroup of bullous autoimmune diseases characterized by autoantibodies against structural proteins of the dermal-epidermal junction. With asteadily growing aging population, pemphigoid diseases are emerging as asignificant medical challenge, because they occur primarily in older individuals. The by far most common disease is bullous pemphigoid, which is clinically characterized by tense blisters, erosions, erythema or urticarial plaques, while severe pruritus is the leading subjective symptom. Mucous membrane pemphigoid predominantly affects surface-close mucous membranes with painful erosions and blisters as well as frequently scarring usually in the mouth, nose, and eyes. Anti-p200 pemphigoid clinically resembles bullous pemphigoid but is much less common. Diagnosis of these diseases involves the combination of clinical evaluation, lesional histopathology, direct immunofluorescence microscopy of aperilesional biopsy and serology. Topical and systemic corticosteroids are the mainstay of pemphigoid diseases treatment. Depending on the severity of the disease, various potentially corticosteroid-sparing therapies, such as dapsone, doxycycline, methotrexate, azathioprine and mycophenolate may be used. In severe courses, treatment with rituximab, cyclophosphamide, intravenous immunoglobulins or immunoadsorption are second- or third-line treatment options. Patients are best managed in centers experience with the management of pemphigoid diseases. Updated national and international guidelines for the diagnosis and treatment of bullous pemphigoid and mucous membrane pemphigoid have recently been published.

CD08 Double bubble trouble

Abstract A 29-year-old woman of Lithuanian heritage was referred to dermatology with a 3-year history of recurrent blistering to the hands and, occasionally, feet. The patient’s own photographs revealed small, tense blisters on the sides of her fingers and one on the foot. In the general dermatology clinic, she was noted to have acrylic nails, which she had been applying at home with her own gel nail kit for over 5 years. In recent years, the patient had noticed that use of the acrylic nail kit exacerbated her blistering. There was no prior history of skin disease or atopy. She was otherwise well, with no known allergies. She worked in the food-packing industry where she wore nonlatex gloves. In light of her frequent, prolonged use of acrylic nails and exacerbation of blistering on use of the home kit, she was suspected to have allergic contact dermatitis (ACD) to acrylates and was referred to the patch test clinic. Patch testing was performed to the British standard, facial, rubber and acrylate series. She had positive reactions to five different acrylates, confirming a clinical diagnosis of ACD relevant to her use of acrylic nails and gel polish. However, she was noted to have several unusual features. Clinically, she had small round scars on the dorsum of both hands. On questioning, she described scars occurring after each blister. Blisters occurred all year round but were significantly more frequent in the summer months. Exposure of her gel nails to ultraviolet (UV) light was felt to trigger the blistering. This prompted additional tests to investigate possible photosensitivity. Both urine and plasma porphyrin levels were significantly elevated, confirming cutaneous porphyria. This is an unusual case of a dual pathology causing blistering of the hands: ACD to acrylates and cutaneous porphyria. In most circumstances, it is ACD that is the overlooked diagnosis, but here, an important general medical diagnosis may have been missed. It is important to remember that blistering caused by gel nail kits may be multifactorial, with acrylates causing ACD and UV light possibly triggering cutaneous porphyria.

A case of bullous pemphigoid developing under treatment with benralizumab for bronchial asthma.

Bullous pemphigoid (BP) is an autoimmune disease characterized by itchy erythema and tense blisters on the whole body. Recent reports have unveiled the pathogenic roles of eosinophils in BP (e.g., dermal-epidermal separation, generation of pruritus). Thus, eosinophils are considered a therapeutic target. Benralizumab is an anti-IL-5 receptor alpha (IL-5Rα) monoclonal antibody (mAb) that is widely used to treat severe eosinophilic asthma. By affecting IL-5Rα, benralizumab depletes eosinophils and basophils due to apoptosis through antibody-dependent cell-mediated cytotoxicity. The efficacies of benralizumab and other biologics, including bertilimumab (anti-eotaxin-1 mAb) and mepolizumab (anti-IL-5 mAb), were evaluated in several clinical trials. Also, reslizumab, an anti-IL-5 mAb, was reported as a successful treatment option in a case of BP. We present a case of severe asthma treated with benralizumab at 8-week intervals for 3 years before BP developed. Histologically, subepidermal blisters without eosinophilic infiltration were observed. Methylprednisolone pulse therapy followed by 40 mg/day (1 mg/kg/day) of oral prednisolone (PSL) was initiated, but the skin lesions worsened. Additional intravenous immunoglobulin and oral azathioprine enabled the oral PSL to be tapered. The benralizumab was discontinued after the onset of BP because the asthma did not worsen. To the best of our knowledge, there have been no reports of BP developing during anti-eosinophil therapy. BP may occur paradoxically via various pathways during treatment with drugs that are typically effective against BP.

Bart Syndrome

Background: Bart syndrome is one of the rarest forms of aplasia cutis congenita (ACC). Clinical Description: We report a sporadic case of a neonate presenting with the absence of skin on the lower limb, tense blisters on fingers, and nail dystrophy. Management: Clinical features and skin biopsy confirmed the diagnosis of Bart syndrome. The baby was given symptomatic treatment with regular antimicrobial dressing. Within 2 weeks, the defect healed with the formation of hypopigmented scar and milia. The bullous lesions also disappeared completely. Conclusion: Bart syndrome, which is a type of ACC, is an extremely rare occurrence. Although it has an extensive involvement, the lesions if superficial, limited to the epidermis, have a good prognosis. Conservative management is sufficient to cause healing within a short span of time.

Analysis of the clinical characteristics of pembrolizumab-induced bullous pemphigoid.

Pembrolizumab, a programmed cell death protein 1 checkpoint inhibitor, is a novel drug used to treat a variety of advanced malignancies. However, it can also result in many immune-related adverse events, with cutaneous toxicities being the most frequent. Regarding pembrolizumab-induced skin adverse reactions, bullous pemphigoid (BP) has the worst effects on quality of life. Recently, there have been more and more reports of BP incidents resulting from pembrolizumab therapy in patients with cancer. This study aimed to define the clinical characteristics, diagnosis and management of pembrolizumab-induced BP and identify potential differences between classical BP and pembrolizumab-induced BP. Case reports, case series, and case analyses of pembrolizumab-induced BP up to 10 December 2022 were collected for retrospective analysis. Our study included 47 patients (33 males and 14 females) from 40 studies. The median age was 72 years (range 42-86 years). The median time to cutaneous toxicity was 4 months (range 0.7-28 months), and the median time to bullae formation was 7.35 months (range 0.7-32 months). The most common clinical features were tense bullae and blisters (85.11%), pruritus (72.34%), and erythema (63.83%) on the limbs and trunk. In 20 of the 22 cases tested, the serum anti-BP180 autoantibodies were positive. However, in 10 cases (91.90%, 10/11) the circulating autoantibodies of anti-BP230 were negative. 40 patients had skin biopsies and the skin biopsy revealed subepidermal bullae or blister eosinophil infiltration in 75.00% of patients with pembrolizumab-induced BP, 10.00% of patients with lymphocyte infiltration and 20.00% of patients with neutrophil infiltration. There were 20 patients (50%) with eosinophilic infiltration around the superficial dermis vessels, 8 patients (20.00%) with lymphocyte infiltration around the superficial dermis vessels, and 4 patients (10.00%) with neutrophil infiltration around the superficial dermis vessels. Direct immunofluorescence detected linear immunoglobulin G (IgG) IgG and/or complement C3 along the dermo-epidermal junction in 36 patients (94.74%) with BP. IgG positivity was detected by indirect immunofluorescence in 81.82% of patients with BP. All patients were in complete remission (95.65%,44/46) or partial remission (4.35%, 2/46) of BP, whereas 9/46 patients had a relapse or refractory. The majority of patients achieved BP remission after discontinuation of pembrolizumab with a combination of topically and systemically administered steroid treatments, or other medications. The median duration of BP remission was 2 months (range 0.3-15 months). A thorough diagnosis of pembrolizumab-induced BP should be made using clinical signs, biochemical markers, histopathological and immunopathological tests. Pembrolizumab-induced BP had similar clinical characteristics to classic BP. Temporary or permanent discontinuation of pembrolizumab therapy may be required in patients with perbolizumab-induced BP depending on the severity of BP and the response to medication. Pembrolizumab-induced BP may be effectively treated using topical and systemic steroid treatments in combination with other medications (e.g., doxycycline, niacinamide, dapsone, rituximab, intravenous immunoglobulins, dupilumab, cyclophosphamide, methotrexate, mycophenolate mofetil, and infliximab). Clinicians should provide better management to patients with BP receiving pembrolizumab to prevent progression and ensure continuous cancer treatment.

Bullous Pemphigoid in a 37-Year Old Female: A Case Report and Literature Review

Bullous pemphigoid is a blistering disorder which mainly affects the geriatric population predominantly older than 70 years. It is caused by an autoimmune reaction to the hemidesmosomal proteins in basal keratinocytes, causing an inflammatory cascade and subsequent bullae formation. It is rarely encountered in infants, children, and middle-aged adults. Herein, a case of Bullous pemphigoid in a 37-year old female patient is reported. The patient presented with a three-month history of multiple serous fluid-filled tense blisters on the face, neck, trunk, flexor and extensor surfaces of the extremities up to the lower thigh, with areas of excoriation, peeling, erosion and crusting. No involvement of the mucous membranes noted. The lesions were associated with intense pruritus and pain upon rupture. Patient had no other subjective complaints and had an unremarkable past medical history. Patient was initially treated with antibiotics but noted no improvement in her condition. Histologic evaluation of the skin was done and revealed a subepidermal vesicular dermatitis with prominent neutrophilic infiltrates. No hyphae or spores were seen. With these findings, patient was admitted and treated with systemic steroids, antibiotics, and antipruritic medications. Direct Immunofluorescence was done and yielded findings consistent with the diagnosis of Bullous Pemphigoid. The overall response of the patient to therapy was satisfactory. The differentiation of Bullous pemphigoid from other subepidermal bullous diseases is important due to the potential of systemic manifestations and complications of the other diseases. The importance of clinical, histopathologic and immunologic findings in confirming the diagnosis of Bullous Pemphigoid is highlighted in this case report.

Bullous pemphigoid in a patient with psoriasis after mRNA COVID‐19 vaccination

AbstractBullous pemphigoid and psoriasis are two well‐characterised chronic autoimmune diseases that may be triggered by drugs, infections or vaccines. The coexistence of the two is a rare event but is more extraordinary if incited by messenger RNA (mRNA) COVID‐19 vaccination. A 62‐year‐old Filipino male experiencing a flare‐up of his long‐standing psoriasis vulgaris presented with new onset tense blisters on the legs 2 days after his first booster dose of mRNA (Pfizer‐BioNTech) vaccination. The clinical, skin punch biopsy, direct immunofluorescence and enzyme‐linked immunosorbent assay were consistent with bullous pemphigoid. Complete resolution of the vesicles and bullae with no new lesions and flattening of psoriasis plaques were observed after 1 month of treatment of systemic corticosteroids and corticosteroid‐sparing agents. An association may exist between bullous pemphigoid development and mRNA COVID‐19 vaccination.

Biologic treatment outcomes in refractory bullous pemphigoid: An evidence-based review

To the Editor: Bullous pemphigoid (BP) is an autoimmune disorder characterized by tense blisters and intense pruritus.1 Initial treatment typically involves the use of topical and systemic corticosteroids, with refractory cases often requiring systemic immunosuppressives or biologics. This systematic review examines treatment outcomes of systemic biologics for BP refractory to other systemic therapies.

The significance of preclinical anti-BP180 autoantibodies.

Bullous pemphigoid (BP) is the most common autoimmune subepidermal blistering disease. Although the pathomechanism of BP onset has yet to be elucidated in detail, BP autoantibodies targeting two hemidesmosomal components, BP180 and BP230, are known to play a pivotal role in BP pathogenesis. Thus, the detection and measurement of BP autoantibodies are necessary for diagnosing BP and monitoring the disease activity. Immune assays such as immunofluorescence microscopy, immunoblotting, and ELISAs using BP180 and BP230 detect BP autoantibodies in most BP cases with high specificity; however, BP autoantibodies are sometimes detected in BP patients before the onset of this disease. BP autoantibodies that are detected in patients without typical tense blisters are defined as “preclinical BP autoantibodies”. These preclinical BP autoantibodies are detected even in a low percentage of normal healthy individuals. Although the importance of preclinical BP autoantibodies remains elusive, these autoantibodies might be a potential risk factor for subsequent BP development. Therefore, previous comparative epidemiological studies have focused on the prevalence of preclinical BP autoantibodies in populations susceptible to BP (e.g., the elderly) or in diseases with a higher risk of comorbid BP. This mini-review summarizes the literature on the prevalence of preclinical BP autoantibodies in patients with various conditions and diseases, and we discuss the significance of preclinical BP autoantibody detection.

Bullous systemic lupus erythematosus in females.

Bullous systemic lupus erythematosus (BSLE) is a rare blistering presentation of systemic lupus erythematosus, typically affecting women with the highest incidence in those of African descent. The key pathogenic insult includes the formation of autoantibodies against type VII collagen, which weaken the basement membrane zone and lead to the formation of subepidermal blisters. The acute vesiculobullous eruptions in BSLE generally tend to affect photo-distributed areas, although they can arise unrelated to sun exposure (eg, mucous membranes, axillae). The bullae can arise from erythematous macules, inflammatory plaques, or previously normal skin. Their appearance can range from small, grouped vesicles reminiscent of lesions in dermatitis herpetiformis to large, tense blisters, similar to bullous pemphigoid. Internal organ involvement occurs in up to 90% of those affected. This mostly includes lupus nephritis (classes III–V, lifetime prevalence of up to 90%), arthralgias/arthritis, and cytopenias, while serositis and neuropsychiatric involvement are rare. First-line management with dapsone should be considered in mild disease with stable underlying systemic lupus erythematosus. As discussed in this review, the off-label use of rituximab (an anti-CD20 B-cell depleting agent) has been shown to be safe and effective in several refractory cases of BSLE unresponsive to dapsone, glucocorticoids, or steroid-sparing immunosuppressants.