Chronic Bullous Disease Research Articles

The "String of Pearls" Sign of Chronic Bullous Disease of Childhood (CBDC).

The "String of Pearls" Sign of Chronic Bullous Disease of Childhood (CBDC).

Exploring the Potential Advantages of Traditional Therapies in Autoimmune Blistering Illnesses: A Comprehensive Review and Analysis, Research

Autoimmune blistering diseases, such as Linear IgA Bullous Dermatosis (LABD) and Chronic Bullous Disease of Childhood (CBDC), provide considerable therapeutic treatment issues. Samana Aushadhis are regularly prescribed by Ayurvedic practitioners as part of therapy regimens for dermatological diseases including LABD and CBDC. Although anecdotal data and clinical observations point to the potential usefulness of traditional medicines in promoting long-term remission and alleviating symptoms, the precise mechanisms by which they exert their therapeutic benefits in LABD/CBD remain incompletely known in contemporary science. The potential advantages of traditional therapies in autoimmune blistering illnesses are being investigated through clinical trials, observational research, and mechanistic investigations. The therapeutic potential of these herbal remedies in LABD and CBDC is being investigated through clinical trials, observational research, and mechanistic investigations. Clinical trials, observational studies, and mechanistic investigations are being conducted in an effort to better understand the therapeutic potential of conventional medications in autoimmune blistering disorders. The effectiveness of these herbal treatments in causing remission and easing symptoms in LABD and CBDC is being closely examined. Overall, while traditional therapies hold promise in the management of autoimmune blistering diseases like LABD and CBDC, more research is necessary to fully understand their mechanisms of action and therapeutic potential. Through rigorous scientific inquiry, the integration of traditional and contemporary medicine may offer new avenues for managing these difficult conditions. Clinical trials, observational research, and mechanistic investigations are all contributing to the exploration of the therapeutic potential of these herbal remedies in LABD and CBDC.

Management of Linear IgA through Ayurveda -A Case study

Linear IgA bullous dermatosis (LABD)/ Chronic bullous disease of childhood (CBDC) is a rare autoimmune blistering disease characterized by the linear deposition of IgA at the dermoepidermal junction. The disease has an incidence of 0.2 to 2.3 per 1000000 million per year showing diverse clinical manifestations and etiologies1.In this case, a 6-year-old child presented with erythematous vesiculobullous lesions on her perianal region, groins, thighs and in both upper and lower limbs since 1 month.The symptoms were gradually progressive in nature.A biopsy was performed, which was consistent with linear immunoglobulin A bullous dermatosis and later confirmed by direct immunofluorescence studies.The child was advised to consume samana aushadhis (internal medicines)for 6 months, which led to remarkable changes in the lakshanas and the patient had no such symptoms till date.This case shows the significant role of ayurvedic intervention in the management of such diseases.

Juvenile Bullous Pemphigoid with Similarity Clinical Features to Chronic Bullous Disease of Childhood

Introduction: Autoimmune bullous disease (ABD) is a specific autoimmune skin disease characterized by subepidermal vesicles and bullae. In pediatrics, there is an autoimmune bullous disease controversy with clinical clusters of jewel on Chronic bullous disease of childhood (CBDC) but dominant IgG autoantibodies. A similar clinical presentation challenges diagnosis; thus, supporting studies on direct immunofluorescence (DIF) as a specific autoantibody factor are necessary. Case: A 10-year-old boy with clinically tense-walled blisters with or without an erythematous base is itchy all over his body, with a predominance of palmoplantar, neck, inguinal, and axillary, presented by erosions, crusts, and a partial cluster of jewels lesion with mucosal involvement. A complete blood count shows leukocytosis and eosinophilia. Hematoxylin-eosin (HE) staining revealed a subepidermal cleft with eosinophil predominance, while direct immunofluorescence (DIF) revealed IgG deposits in the basal membrana and intercellular parts of the basal stratum. Discussion: Histopathology reveals subepidermal blisters of eosinophil dominance, and direct immunofluorescence (DIF) examination reveals IgG autoantibody deposit dominance, verifying the diagnosis of juvenile bullous pemphigoid. Given that BP180 (BPAG2) is one of the autoantigens involved, there may be similarities between the histology and clinical presentation of Bullous pemphigoid (BP) and CBDC. Conclusion: Autoimmune bullous diseases are difficult to differentiate clinically, histological studies and direct immunofluorescence are necessary to make a diagnosis.

Pemfigus Vulgaris

Pemphigus vulgaris (PV) is a chronic intraepidermal bullous disease that causes extensive skin lesions. Pemphigus vulgaris initially appears in the form of vesicles and loose-walled bullae that break easily on normal skin, which then develop into bullae with an erythematous base, then rupture, causing erosion, excoriation, which is usually accompanied by crusting and pain. Bullae occur due to damage or damage to intercellular adhesion due to IgG autoantibodies to desmoglein 3, causing epithelial cells called acantholysis (epidermal cell separation reaction). The hallmark of pemphigus is erosion that extends into normal skin by pulling on remnants of the blister wall or rubbing on the periphery of an active lesion. Erosion can be induced on normal-appearing skin away from the active lesion by mechanical stress or shear, a phenomenon known as the Nikolsky sign. Prognosis before corticosteroids are used, death occurs in 50% of patients within the first year. Treatment with corticosteroids makes the prognosis better. Pemphigus vulgaris in the early stages will be easier to control than those with widespread manifestations, the mortality rate will increase if there is a delay in therapy.

Cicatricial alopecia associated with pemphigus.

Pemphigus is a chronic autoimmune bullous disease associated with the production of autoantibodies directed against desmosomal proteins, such as desmogleins 1 and 3. Here, we present the case of an 83-year-old woman who was referred to us with suspicious cicatricial alopecia of the scalp and a small, eroded lesion on the forehead, previously labeled as atrophic actinic keratosis after a skin biopsy. In our clinic, after a careful examination of the case, we decided to perform two new skin biopsies of the scalp on suspicion of an inflammatory disease.

Annular bullous lesions in a child from Uganda: chronic bullous disease of childhood.

Annular bullous lesions in a child from Uganda: chronic bullous disease of childhood.

Chronic Bullous Disease of Childhood: A Case Report and Review of Literature of Bullous Diseases in Children

We describe a case of a 5-year-old boy who presented with multiple mild itchy and painful blisters predominantly over his trunk and limbs for one year with multiple tense bullae with hypopyon signs resulting in a half-and-half appearance of the blisters. A skin biopsy showed subepidermal separation with predominantly neutrophils and eosinophils in the bulla cavity and mixed infiltrate in the papillary dermis. We would like to report a case of chronic bullous disease of childhood and review its differentials for diagnostic approaches to blistering disease in children.

Annular bullous diseases.

Annular bullous dermatoses represent an etiologically diverse group of cutaneous phenomena that present with a figurate morphology in association with vesicles and bullae. This group of diverse conditions consists of bullous pemphigoid; pemphigoid gestationis; epidermolysis bullosa simplex, Dowling-Meara type; linear immunoglobulin A bullous dermatosis; chronic bullous disease of childhood; anti-p200 pemphigoid; subcorneal pustular dermatosis; and immunoglobulin A pemphigus. Astute examination of clinical, histopathologic, and serologic features is crucial in distinguishing these bullous dermatoses. We review the clinical presentation, pathophysiology, histopathology, and treatments for each bullous annular disease to aid physicians in their recognition, diagnosis, and management.

Hypertension as Comorbidity in Pemphigus Vulgaris Patients: A Case Series

Background: Pemphigus Vulgaris (PV) is a chronic autoimmune bullous disease characterized by flaccid blisters on the skin and mucous membrane. This study aimed to report a serial case of PV followed by hypertension as comorbidity.
 Case presentation: Four patients, 2 males, and 2 females were hospitalized due to PV. All of these patients also suffer from hypertension, but only 2 patients take antihypertension. Drugs relatively, histopathology features of all patients revealed a cleft above the basal epidermal layer with a tombstone appearance. Pemphigus vulgaris can be accompanied by hypertension as comorbidity, but its pathogenesis is unclear. A low level of programmed cell death 1 (PD1) in PV patients promotes downregulating protective factors, including T regulator (Treg) cells. In addition, decreasing the PD1 level may cause apoptosis and inflammation process in the vascular endothelial, which may lead to hypertension. 
 Conclusion: Pemphigus vulgaris is significantly associated with hypertension.

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.

Immunotherapy for Pemphigus: Present and Future.

Pemphigus is a chronic and severe autoimmune bullous disease caused by autoantibodies targeting adhesion molecules between keratinocytes. It requires 2–3 years on average to manage the disease. To date, although Rituximab combined with short-term systemic glucocorticoids was accepted as first-line therapy, systemic glucocorticoids remain the primary therapeutic option for pemphigus patients, successfully decreasing morbidity and mortality from pemphigus. However, novel therapeutic strategies are desirable due to the low efficacy in some subset of patients and the long-term severe adverse effects of traditional therapies. Recently, immunotherapy has proved to be encouraging for disease control or cure. Based on the current understanding of the immune mechanisms of pemphigus, we review the immune targets and corresponding agents applied in practice or under clinical trials. The goals of the novel treatments are to improve the quality of life of pemphigus patients by improving efficacy and safety, minimizing side effects, achieving fast disease control, or curing the disease.

Not all that blisters is infectious.

A child who presents with blistering that is not due to an infectious cause can represent a diagnostic challenge for primary care physicians. Pediatric autoimmune bullous diseases encompass several very uncommon blistering skin conditions, including chronic bullous disease of childhood (CBDC),

IL-13 Genetic Susceptibility to Bullous Pemphigoid: A Potential Target for Treatment and a Prognostic Marker.

BackgroundBullous pemphigoid (BP) is a senile chronic autoimmune bullous skin disease with a high relapse rate, which significantly impairs patients’ quality of life and contributes to disease mortality. This observational case-control study explores the gene polymorphisms of cytokines and their clinical significance in Chinese patients with BP.MethodsIL-1α (rs1800587), IL-1β (rs16944, rs1143627, rs1143634), IL-4 (rs2243250), IL-6 (rs1800795), IL-10 (rs1800896, rs1800871, rs1800872), IL-13 (rs1800925, rs20541), TNF-α (rs1799964, rs1800630, rs1799724, rs361525), IFN-γ (rs1799964, rs1800630, rs361525, rs1800629, rs4248160, rs1800750), and TGF-β1 (rs2317130, rs1800469, rs4803457) genes were genotyped in the healthy controls and BP patients, respectively. Expression of these cytokines in serum was measured. Medical profiles of patients, including baseline characteristics and prognosis, were statistically analyzed.ResultsWe found that IL-1 β and IL-13 concentrations were higher in the BP patients’ sera compared to those in the controls. For IL-13, significant differences were found in the nucleotide ratio/genotype/haploid frequency/haplotype, respectively. IL-13 (rs20541, rs1800925) is related to gender, and the IL-13 genotype was significantly associated with recurrence.ConclusionsBP is associated with IL-13 gene polymorphism and IL-13 concentration is elevated in blood circulation in patients with BP. Our results support that IL-13 is relevant in the pathogenesis of BP, suggesting that IL-13 could potentially represent a promising target for BP therapy and a prognostic marker.

Isolated oral pemphigus vulgaris: Case series of 15 patients

Pemphigus is a chronic inflammatory autoimmune bullous disease that affects the oral mucosa and is sometimes difficult to diagnose when only mucosal involvement is present. We performed a retrospective descriptive study including 15 cases of isolated mucosal pemphigus (mPV). We noticed that this rare form is characterized by it slow healing, a female predominance with frequent relapses.

007 Comorbidities and Complications among Pemphigus patients: a retrospective cohort study

Pemphigus is a chronic bullous disease with a poor prognosis, especially in case of a mucocutaneous involvement. Due to immune disturbance and skin barrier deficiency, complications are frequent. We performed a retrospective cohort study based on medical records of 303 hospitalized pemphigus patients in the Dermatology department of Ibn Sina University Hospital between 1990-2020. Data collected include: age, history, mean duration of disease before consultation, bedridden duration, clinical phenotype, death.

One Case Suspected Chronic Bullous Disease in Childhood

Penyakit bula pada anak sangat beraneka ragam, mulai dari yang paling sering adalah impetigo bulosa dan epidermolysis bulosa. Sedangkan penyakit bulosa lain pada anak yang lebih jarang dijumpai adalah penyakit bulosa linear IgA pada anak, pemfigoid bulosa dan sistemik lupus eritematosus bulosa. Salah satu varian dari penyakit bulosa linear Ig A pada anak adalah Chronic bullous disease in childhood (CBDC) yaitu suatu penyakit autoimun yang jarang dijumpai dan muncul pada usia prepubertas. Ciri khas dari penyakit ini adalah timbulnya lepuh yang muncul secara mendadak dan biasanya lesi yang baru muncul di sekitar lesi yang mulai sembuh dengan membentuk pola lingkaran (clusters of jewels) dan seringnya pasien disertai dengan rasa gatal. Untuk diagnosa pasti dari penyakit ini adalah dengan melakukan biopsi dan pemeriksaan imunofloresen direk. Terapi utamanya sendiri yaitu dapson dan sulfapiridin, sedangkan prednison dengan dosis rendah sebagai terapi tambahan lini pertama. Tujuan dari laporan kasus ini adalah ingin mengetahui efikasi dan efek samping dari pemberian oral steroid pada kasus dengan lesi bula yang diduga CBDC. Seorang anak laki-laki berusia 5 bulan, suku Jawa, datang ke poliklinik kulit dan kelamin Rumah Sakit Kandou dengan keluhan adanya bercak kemerahan yang disertai lenting berisi cairan jernih di hampir seluruh tubuh sejak kurang lebih 1 bulan yang lalu. Diagnosis pasti pada pasien ini masih belum dapat diketahui , namun diduga penyakit bula pada anak CBDC, dikarenakan berdasarkan anamnesis dan pemeriksaan fisik saja, karena keterbatasan alat dan keluarga pasien menolak untuk dilakukan pemeriksaan penunjang. Terapi diberikan dengan tujuan agar keluhan dari pasien dapat berkurang dan dengan efek samping yang minimal. Pemberian prednison oral yang diberikan secara tapering-off, ditambah dengan oral antihistamin dan topikal antibiotik dan steroid memberikan hasil yang cukup signifikan pada pasien. Kesimpulan walaupun sebenarnya penyakit ini dapat sembuh sendiri dalam hitungan bulan, namun dengan pemberian oral steroid dan antihistamin dengan kombinasi obat topikal dapat mempercepat proses penyembuhan pada pasien.

PEMPHIGOID OF MUCOUS MEMBRANES REPORT OF A CASE: MANAGEMENT AND FOLLOW-UP EVALUATION

Pemphigoid of the mucous membranes is a chronic autochthonous mucocutaneous bullous disease, in which the production of tissue-bound autoantibodies is directed against 1 or more components of the basement membrane hemidesmosomes. This is a report of case of a 56-year-old male patient, with a symptomatic lesion on gingival mucosa, reddish and scaly. Clinical diagnosis was pemphigoid of the mucous membranes and the lesion was submitted to incisional biopsy. Microscopic examination revealed a fragment of oral mucosa coated with parakeratinized pavement epithelium, exhibiting subepithelial cleft areas. The underlying lamina propria consisted of dense fibrous connective tissue with moderate chronic inflammatory infiltrate. The clinical and histologic characteristics led to the diagnosis of pemphigoid of mucous membranes. The patient was successfully treated with 0.05% clobetasol mouthwash, showing an improvement in the presented clinical picture. The patient’s condition remains stable after 1 year of follow-up. Pemphigoid of the mucous membranes is a chronic autochthonous mucocutaneous bullous disease, in which the production of tissue-bound autoantibodies is directed against 1 or more components of the basement membrane hemidesmosomes. This is a report of case of a 56-year-old male patient, with a symptomatic lesion on gingival mucosa, reddish and scaly. Clinical diagnosis was pemphigoid of the mucous membranes and the lesion was submitted to incisional biopsy. Microscopic examination revealed a fragment of oral mucosa coated with parakeratinized pavement epithelium, exhibiting subepithelial cleft areas. The underlying lamina propria consisted of dense fibrous connective tissue with moderate chronic inflammatory infiltrate. The clinical and histologic characteristics led to the diagnosis of pemphigoid of mucous membranes. The patient was successfully treated with 0.05% clobetasol mouthwash, showing an improvement in the presented clinical picture. The patient’s condition remains stable after 1 year of follow-up.

CHIMERIC ANTIGEN RECEPTOR (CAR) T-CELLS THERAPY UNTUK PENYAKIT AUTOIMUN

Introduction: Pemphigus vulgaris (PV) is a rare and potentially life-threatening chronic bullous autoimmune disease caused by the presence of desmoglein 3 adhesion protein autoantibodies. The most commonly used PV treatment remains immunosuppressants which have adverse effects such as increased immune responses and relapse later on. Therefore, innovative therapeutic strategy that more targeted and can prevent relapse in the long term are needed for autoimmune diseases treatment. eview current trends of CAR T-cell applications in PV. The objectives of this study is to review current trends of CAR T-cell applications in PV.Discussion: The presence of autoantibodies against desmoglein 3 (?Dsg3) is commonly found in patients with PV. ?Dsg3 is localized to the mucous membrane and mucocutaneous. Autoantibodies appear due to overactivation of CD4+ Th2 cells. CAR T-cell is the result of the engineering of vector-assisted T cells that carry the chimera receptor according to what is targeted. This treatment with CAR T-cell is increasingly promising after FDA approval of two drugs for haematological cancer. Researchers are interested in developing CAR T-cell as a treatment for autoimmune disease. In vitro results from CAAR T-cell (which is modification of CAR T-cell) showed specific cytotoxicity activity against ?Dsg3 BCR. The results of the study in vivo discussed the potential for specific elimination of B cells Dsg3 based on serum and succeeded in maintaining epidermal integrity in mice.Conclusion: CAR T-cell shows the potential to be applied as a choice for PV therapy in the future. Nevertheless, further development of the application of CAR T-cell is needed for PV therapy so that specific treatment options can be obtained with minimal adverse effects for patients

Secondary spontaneous pneumothorax and bullous lung disease in cannabis and tobacco smokers: A case-control study.

BackgroundThe notion that smoking cannabis may damage the respiratory tract has been introduced in recent years but there is still a paucity of studies on this subject. The aim of this study was to investigate the relationship between cannabis smoking, pneumothorax and bullous lung disease in a population of operated patients.Methods and findingsWe performed a retrospective study on patients operated on for spontaneous pneumothorax. Patients were divided into three groups according to their smoking habit: cannabis smokers, only-tobacco smokers and nonsmokers. Cannabis lifetime exposure was expressed in dose-years (1d/y = 1 gram of cannabis/week for one year). Clinical, radiological and perioperative variables were collected. The variables were analyzed to find associations with smoking habit. The impact of the amount of cannabis consumption was also investigated by ROC curves analysis.Of 112 patients, 39 smoked cannabis, 23 smoked only tobacco and 50 were nonsmokers. Median cannabis consumption was 28 dose/years, median tobacco consumption was 6 pack/years. Cannabis smokers presented with more severe chronic respiratory symptoms and bullous lung disease and with a higher incidence of tension pneumothorax than both tobacco smokers and nonsmokers. Cannabis smokers also developed a larger pneumothorax, experienced prolonged postoperative stay and demonstrated a higher incidence of pneumothorax recurrence after the operation than nonsmokers did. The risk of occurrence of chronic respiratory symptoms and bullous lung disease in cannabis smokers was dose-related.ConclusionsCannabis smoking seems to increase the risk of suffering from respiratory complaints and can have detrimental effects on lung parenchyma, in a dose-dependent manner. Cannabis smoking also negatively affected the outcome of patients operated for spontaneous pneumothorax. A history of cannabis abuse should always be taken in patients with pneumothorax. There may be need for a specific treatment for pneumothorax in cannabis smokers.