Cases Of Bullous Pemphigoid Research Articles

A case series of ocular involvement in bullous pemphigoid: clinical features, management, and outcomes

Ocular involvement in cases of bullous pemphigoid is rare and when present, the signs are usually subtle and in the form of fine tarsal scarring and dry eye disease. The current report aims to describe the clinical features and management protocols in a series of cases with aggressive ocular manifestations at presentation. All cases of bullous pemphigoid seen between 2017 and 2020 were included in this retrospective case series. Data regarding the clinical features, treatment administered, and outcomes was collected. Five cases (n=10 eyes) of bullous pemphigoid disease with ocular involvement were included. All eyes had significant cicatricial conjunctival changes in the form of symblephara, inferior forniceal shortening, and tarsal conjunctival scarring. Conjunctival granulomas were present in 3/10 eyes. Corneal involvement in the form of punctate keratitis was present in all eyes while 4/10 eyes had an epithelial defect as well. The management of these cases involved topical therapy with corticosteroids and lubricants (n=10 eyes) while pulse doses of intravenous methyl prednisolone were administered in 5/5 cases. Pulse intravenous cyclophosphamide was supplemented in 2/5 cases. Adequate control of the disease was noted in 3/5 cases while one case had a recalcitrant form of the disease and developed a dermalised ocular surface in both eyes. The last patient was lost to follow up during the course of therapy. Bullous pemphigoid can present with an aggressive form of cicatrizing conjunctivitis similar to other variants of autoimmune blistering disorders and must be considered as a differential in cases presenting with ocular cicatricial disease. Long-term intensive immunosuppression is required for the management of these cases to preserve the visual function and the integrity of the globe.

HISTOPATHOLOGICAL STUDY OF VESICULOBULLOUS LESIONS OF SKIN WITH SPECIAL REFERENCE TO DIRECT IMMUNOFLUORESCENCE

Skin represents a window to the internal well-being of the disease. Vesiculobullous diseases represents a heterogenous group of dermatoses with protean manifestations. One of the main reason for continued identication of new bullous diseases is because the diagnosis is based on immunological & molecular basis in addition to clinical ndings & immunomorphology. This study aims to correlate Direct Immunouorescence with clinical and Histopathological ndings and to analyze the utility of Immunouorescence in the diagnosis of vesiculobullous lesions of skin. A total of 50 cases were evaluated with vesiculobullous lesions with clinical history, signs, histopathological and immunouorescence studies. In this study, Bullous Pemphigoid and Pemphigus vulgaris constituted 82 % of cases. Oral mucosal involvement was present in 89.5 % cases of Pemphigus Vulgaris & 22.7% cases of Bullous pemphigoid. Subepidermal blister/ bullae is commonest in the study, next being supra basal blister/ bullae. 71.4 % were positive for Direct IF in which 2/3 cases staining pattern was throughout the entire epidermis & in 1/3 cases staining pattern was restricted to lower 1/3 of epidermis. 50% of Dermatitis Herpetiformis showed positive staining by IgA in papillary dermis showing granular BM zone pattern. In Bullous Pemphigoid subjected for DIF, 70% cases were positive with linear basement membrane zone deposition of antibodies.

A case of bullous pemphigoid associated with plasma cell leukemia

A case of bullous pemphigoid associated with plasma cell leukemia

Case of bullous pemphigoid in patient with psoriasis, after new coronavirus infection COVID‑19

In this work we discuss a case of Bullosus pemfigoid (B.P.) developed after pneumonia caused by new coronavirus disease COVID‑19 in a patient with psoriasis. Provided description is supported with the data of histological and serologic analysis, the lgG of epithelial basement membrane was detected to antigen ВР230-CF and to antigen ВР180-NC16A.

A case of bullous pemphigoid in the oral cavity after treatment with nivolumab

A case of bullous pemphigoid in the oral cavity after treatment with nivolumab

A Cross-Sectional Study to Correlate Disease Severity in Bullous Pemphigoid Patients with Serum Levels of Autoantibodies Against BP180 and BP230.

Context:Enzyme-linked immunosorbent assay (ELISA) for BP 180 and 230 antibodies is commonly done in patients with bullous pemphigoid. We could not find much data regarding the usefulness of this test to predict the disease severity in Indian population.Aims:We studied the correlation of IgG anti BP180 and anti BP230 antibody titer with disease severity and clinical features in bullous pemphigoid.Settings and Design:This cross-sectional study was conducted at a tertiary care center in western India.Materials and Methods:Forty-two clinically diagnosed treatment-naive cases of bullous pemphigoid were enrolled and investigated with skin punch biopsy, IgG anti BP180, and anti BP230 ELISA, direct immunofluorescence, and indirect immunofluorescence tests. Disease severity was assessed by calculating modified Autoimmune Bullous Skin Disorder Intensity Score (ABSIS) score. Thirty patients with a final diagnosis of bullous pemphigoid were included in the statistical analysis. Pearson's correlation coefficient (r) was used to study correlation.Results:The mean ABSIS skin score was 32.81 when both tests were negative, 42.13 when only BP230 was positive, 76.28 when only BP180 was positive, and 78.16 when both were positive. Pearson's correlation coefficient (r) for BP180 and ABSIS skin score was 0.6 (P value: 0.0005), and for BP230 was -0.055 (P value: 0.600).Conclusions:BP antibody titers correlate partially with disease severity. Anti-BP180 antibody is associated with more severe disease. Anti-BP230 antibody titer does not correlate with disease severity.

Bullous pemphigoid of pregnancy: clinical picture, diagnostics, treatment

Pregnancy is a particular period in womans life. It accompanies with plenty of adaptation changes include many on skin. Pathological morphofunctional changes form a group of pregnancy-specific dermatoses.
 The clinical case of bullous pemphigoid of pregnancy is presented in the article. The disease occurs during pregnancy or postpartum period. The incidence is estimated to be approximately 1 in 60,000 pregnancies. Clinically, PG is characterized by intense pruritus and polymorphic skin eruptions. Skin lesions develop including erythematous patches and plaques, followed by urticarial rash and blisters. Diagnosis of the disease is based on the features of the clinical picture, complex data of pathomorphological and immunological research methods, including direct or indirect immunofluorescence, enzyme-linked immunosorbent assay (ELISA), immunohistochemical determination of a fragment of the complement component C4d and determination of serum antibodies to BP180.
 The most effective treatment is systemic glucocorticosteroids in combination with topical steroids and/or antihistamines. Timely diagnosing and prescribing appropriate medication are highly important in preventing intrauterine fetal suffering.

Extension of separation along the follicular epithelium and follicular basement membrane in pemphigus and bullous pemphigoid.

The outer root sheath of hair follicle and the interfollicular epidermis are continuous and basically similar in many aspects. The expression of various basement membrane (BM) components in the upper portion of the hair follicle is similar to that of the interfollicular BM. The extension of acantholysis into the adnexal structures was highlighted in the pemphigus group of diseases. On the other hand, extension of separation down the hair follicle BM has not been studied in subepidermal autoimmune bullous dermatoses. The aim of the present work was to study the extension of separation along follicular epithelium and follicular BM in pemphigus and bullous pemphigoid. This retrospective study was conducted on pemphigus and bullous pemphigoid cases. For each case, 4-μm-thick sections stained with hematoxylin and eosin were examined for the presence of hair follicles and the extension of separation down follicular epithelium and follicular BM. Positive extension of acantholysis down the follicular epithelium was seen in 23/27 (85.2%) of pemphigus vulgaris (PV) and 7/9 (77.8%) of pemphigus foliaceus (PF) cases with hair follicles. Follicular extension was limited to the infundibulum in PF but down the entire follicular length in PV. As for bullous pemphigoid (BP) cases, 20/26 cases (76.9%) that had hair follicles showed positive extension of the separation down the follicular BM. The cellular infiltrate detected was identical in its nature and density to that of subepidermal blister in all cases. Extension of the separation along follicular epithelium and follicular basement membrane was observed in PV, PF, and BP without significant difference.

A case of bullous pemphigoid after a long‐term administration of anti‐PD‐1 antibodies in a patient with non‐small‐cell lung cancer

We described a case of BP that developed 2.5 years after the administration of nivolumab for metastatic non‐small cell lung cancer. Our report highlights the fact that dermatologists should be aware of anti PD‐1‐related BP, which can appear very late during treatment.

Clinical, biological and histological characteristics of bullous pemphigoid associated with anti‐PD‐1/PD‐L1 therapy: A national retrospective study

Immune checkpoint inhibitors (ICI), most commonly anti-PD-1 (programmed cell death 1) and anti-PD-L1 (programmed cell death ligand 1) therapies, have revolutionized the treatment of many cancers. ICI may activate the immune system response even in healthy tissues, which can cause immune-related adverse events (irAEs).(1) Several cases of bullous pemphigoid (BP) triggered by anti-PD-1/PD-L1 therapy have been reported.

Cutaneous toxicities occurring during palbociclib (CDK4/6 inhibitor) and endocrine therapy in patients with advanced breast cancer: a single-centre experience.

Treatment with Palbociclib, a cyclin-dependent kinase 4/6 inhibitor, has demonstrated significantly improved progression-free survival in patients with hormone receptor-positive, HER2-negative, advanced breast cancer, when used in combination with letrozole or fulvestrant endocrine therapies. However, limited information exists on its cutaneous adverse effects (AE). Hence, we conducted a retrospective cohort study to investigate the prevalence and management of cutaneous AE during palbociclib and endocrine therapy. We included 324 adult patients with advanced breast cancer who received palbociclib between March 2016 and August 2020 within a tertiary comprehensive cancer centre. Patient demographics, details of previous and concurrent treatments, as well as treatment-related cutaneous AE were recorded from electronic records. The incidence of treatment-related cutaneous AE was 14.2% (46 from a total of 324 patients). The most frequent cutaneous reactions included maculopapular rash (41%), asteatosis (37%), pruritus and urticaria (20%), and bullous dermatitis reactions (9%). We identified two patients with treatment-induced subacute cutaneous lupus erythematosus, one case of bullous pemphigoid, and a single erythema multiforme. Patients received an average of 9 cycles of treatment, completing an average of 6 cycles before developing cutaneous AE, which persisted for a median of 43days. Only 15% (n = 7) of affected patients required temporary suspension, and 4% (n = 2) required discontinuation. The majority were managed with potent topical steroids, with oral corticosteroids being required in 3 patients, and one patient required hydroxychloroquine. Our study describes both the spectrum of cutaneous AE of palbociclib and endocrine therapy, and approaches to management. Prompt management may limit the negative impact on patients, facilitating beneficial continuation of palbociclib and endocrine therapy.

Bullous pemphigoid receiving a novel long‐acting dipeptidyl‐peptidase‐4 (DPP‐4) inhibitor omarigliptin in a patient with type 2 diabetes: A case report

The present paper reports a case of bullous pemphigoid receiving a novel long-acting dipeptidyl-peptidase-4 inhibitor omarigliptin, by which serum IgG autoantibodies are directed against BP180-NC16a domain. Our present case had non-inflamed phenotype, and the relatively higher antibody titer was prolonged even after withdrawal of the culprit omarigliptin.

Using electronic health records to inform trial feasibility in a rare autoimmune blistering skin disease in England

BackgroundTrials of novel agents are required to improve the care of patients with rare diseases, but trial feasibility may be uncertain due to concerns over insufficient patient numbers. We aimed to determine the size of the pool of potential participants in England 2015–2017 for trials in the autoimmune blistering skin disease bullous pemphigoid.MethodsThe size of the pool of potential participants was estimated using routinely collected healthcare data from linked primary care (Clinical Practice Research Datalink; CPRD) and secondary care (Hospital Episode Statistics; HES) databases. Thirteen consultant dermatologists were surveyed to determine the likelihood that a patient would be eligible for a trial based on the presence of cautions or contra-indications to prednisolone use. These criteria were applied to determine how they influenced the potential pool of participants.ResultsExtrapolated to the population of England, we would expect approximately 10,800 (point estimate 10,747; 95% CI 7191 to 17,239) new cases of bullous pemphigoid to be identified in a three-year period. For a future trial involving oral prednisolone (standard care), the application of cautions to its use as exclusion criteria would result in approximately 365 potential participants unlikely to be recruited, a further 5332 could be recruited with caution, and 5104 in whom recruitment is still possible. 11–17% of potential participants may have pre-existing dementia and require an alternative consent process.ConclusionsRoutinely collected electronic health records can be used to inform the feasibility of clinical trials in rare diseases, such as whether recruitment is feasible nationally and how long recruitment might take to meet recruitment targets. Future trials of bullous pemphigoid in England may use the data presented to inform trial design, including eligibility criteria and consent processes for enrolling people with dementia.

L’apport de l’immunofl uorescence directe dans le diagnostic des dermatoses infl fl ammatoires cutanées

Our work concerned a retrospective study. This was done on the basis of the records of patients attending dermatology and having undergone an (DIF) over a period of two years (2015-2016). Thus, we have selected 30 cases in dermatology and for which a DIF was performed. The final diagnosis were distributed as follows: 02 cases of bullous pemphigoid, 04 cases of pemphigus (01 cases of deep pemphigus, 03 cases of superficial pemphigus) and 02 cases of lupus Erythematosus. According to the literature, the positivity rate of IFD in DBAI is very high. This doesn’t correspond to the results of our study which were: 66% in the pemphigus and 30% in the LES. Except for the bullous pemphigoïde whose rate correspond to the literature with 40%. The IFD has succeeded in demonstrating that it possesses a high diagnostic value in cutaneaous inflammatory dermatosis. It confirms a diagnosis and retains another.

Geriatric dermatosis; The concerns of aging skin.

Objectives: To study the cutaneous manifestations in elderly and their association with gender. Study Design: Cross Sectional study. Setting: Sir Syed Hospital Karachi. Period: Jan 2016 to Jan 2018. Material & Methods: Total 217 geriatric cases (>60 years; both genders) were included from outdoor dermatology clinic. The previous pregnancy, lactation, severe systemic disease and drug induced skin changes were excluded. The association of skin manifestation with gender was analyzed by Chi-square test with significant p<0.05. Results: Among 217 cases, there were 126(58%) females and 91(42%) males. Mean age was 69.3+4.99 years. Wrinkling was seen in 92%, skin laxity in 54.8%, xerosis (43%), dryness (65%), sagging (46%), pigmentation (37%), eczema (25%), sparse hair (21%), skin atrophy (19%), tinea (13%) and LSC (10%). While herpes zoster, psoriasis, onychomycosis, tinea corporis ,asteotic eczema in 7%. Folliculitis 6%, scabies and bullous pemphigoid seen in 5%, while lichen planus, actinic elastosis, BCC, Prurigo nodularis in 3%. Cellulitis, pediculosis, Seborrheic Dermatitis and contact dermatitis were seen in 2% followed by 1% cases of warts, candidiasis, photodermatosis and SCC seen in our study. Folliculitis (6%) was the most frequent bacterial infection followed by impetigo (0.9%). Herpes zoster (6.5%) was frequent among viral infections followed by warts (0.9%) and HSV-1 (0.9%). Tinea (13.4%) was most frequent fungal infection followed by onychomycosis (7%) and candidiasis (0.9%). Scabies was most common parasitic infection (4.6%) followed by pediculosis (2.3%). Eczema seen in 24.8%. The benign dermatosis, Actinic elastosis was frequent (3.2%), stucco keratosis 4(1.8%), Keratoacanthoma (0.9%). Amongst malignancies, BCC was common (3.2%), followed by SCC (1.4%). 5% Bullous Pemphigoid cases were found. The psychocutaneous disorder included LSC (10%), prurigo nodularis (2.8%) and neurotic excoriation (0.9%). Wrinkling, sagging, dryness of skin, actinic keratosis, actinic elastosis was found to be associated with female gender, however cellulitis, squamous cell carcinoma and asteatotic eczema with male gender. Conclusion: Geriatric population is facing multiple dermatological concerns. Contributory factors being the natural aging process, exposure to UV radiation, pathogens, dryness, humidity, temperature and chemical irritants. Female geriatrics are prone to wrinkling, sagging, dryness of skin and male geriatrics to basal cell carcinoma. There is need to address the skin problems in elderly for better quality of life in geriatrics.

Bullous Pemphigoid in Otolaryngology: Case Report

Bullous pemphigoid is a disease of the sub epithelial layer of the skin of autoimmune etology that ofen appears in elderly individuals and is characterized by the presence of cutaneous bullous lesions and erosions of the mucous membranes. The inital presentaton of a case of bullous pemphigoid in otorhinolaryngology is described. A 70-year-old man resorts to an otolaryngology consultaton for an history of odynophagia and pharyngeal foreign body sensaton with four months of evoluton. Concomitantly, he complained of severe itching related to the appearance of skin lesions. Physical examinaton revealed ulcerated lesions of the palate, tongue base and pharynx. Naso fbroscopy was performed, and mucous granulaton was observed in the right nasal cavity and hypo pharynx as well as larynx edema. The rest of the body had erythematous skin lesions in the region of the thighs and armpits as well as ?uid-flled, tense blisters with regular-sized borders compatble with the bullous pemphigoid. The patho physiological mechanisms responsible for pemphigoid lesions are not fully understood, but it's thought to be related to antbody-mediated basement membrane epithelial lesion. Usually, the manifestatons in the mucous membranes improve with systemic treatment insttuted to treat the skin lesions. The clinical course of these lesions is variable, generally following a relapsing chronic patern controlled by cortcotherapy. Long-term remission may occur afer months or years.

Validation study of bullous pemphigoid and pemphigus vulgaris recording in routinely collected electronic primary healthcare records in England

ObjectivesThe validity of bullous pemphigoid and pemphigus vulgaris recording in routinely collected healthcare data in the UK is unknown. We assessed the positive predictive value (PPV) for bullous pemphigoid and...

Bullous pemphigoid associated with the use of dipeptidil peptidase-4 inhibitors: analysis from studies based on pharmacovigilance databases

Background Bullous pemphigoid has been associated to dipeptidase-4 inhibitors. Objectives Addressing the potential Bullous pemphigoid-dipeptidase-4 inhibitors association based on pharmacovigilance data currently available in Spain in order to obtain a composite disproportionality estimator from all the data generated by the case-non case studies conducted to this date. Setting The Spanish Pharmacovigilance System for Human Use Drugs database. Method Case-non case study based on the Spanish Pharmacovigilance System for Human Use Drugs notifications submitted between 2007 and 2018 (n = 169,280), using the Medical Dictionary for Regulatory Activities term (Preferred Term) ‘pemphigoid’ for sitagliptin, vildagliptin, saxagliptin, linagliptin, and alogliptin (n = 1952). As negative control, we used acetaminophen, while furosemide was the positive control. A pooled reported odds ratio analysis in the French, Japanese, and Spanish national pharmacovigilance databases was performed. On The Spanish Pharmacovigilance System for Human Use Drugs, we conducted a bullous pemphigoid-metformin association analysis within the period 1982–2018. Main outcome measure Adverse reaction cases in pharmacovigilance databases and the disproportionality through the reporting odds ratio. Results Within The Spanish Pharmacovigilance System for Human Use Drugs, we found 45 cases of bullous pemphigoid in dipeptidase-4 inhibitors patients. Median age was 77 years (range 72–82). The median latency period was 7 months (range 0.23–86). The Bullous pemphigoid-dipeptidase-4 inhibitors association was established with a reporting odd ratio = 70.0 (95% confidence intervals 49.1–10.1). In the combined analysis of the three aforementioned pharmacovigilance databases, the pooled reporting odd ratio was 81.0 (95% confidence intervals 69.5–94.4). Conclusion The composite estimator for the three national pharmacovigilance databases yields clear evidence of a Bullous pemphigoid-dipeptidase-4 inhibitors association, which was statistically significant for both the pharmacological class as a whole and each of the dipeptidase-4 inhibitors agents under investigation. Metformin’s role in the incidence of bullous pemphigoid appeared casual rather than causal. No differences between Caucasian and Asian populations were noted.

Dipeptidyl peptidase 4 inhibitors linked bullous pemphigoid in patients with type 2 diabetes mellitus: A series of 13 cases

BackgroundDipeptidyl peptidase 4 (DPP4) inhibitors have increasingly been linked to bullous pemphigoid, but there is paucity of data from India where about 1.85 million patients have been estimated to use these drugs. MethodsIn 30,000 patients with T2DM seen by us in two tertiary care centres since 2015, we detected 13 cases of bullous pemphigoid linked to DPP4 inhibitors. We used WHO-UMC (World Health Organisation-Uppsala Monitoring Centre) causality assessment system for assessment. ResultsLesions of bullous pemphigoid appeared at varied intervals (within 1 weeks–2 years) after start of DPP4 inhibitors. Implicated drugs were Linagliptin (n, 8), Vildagliptin (n, 4) and Sitagliptin (n, 1). Mostly, lesions were seen after 60 years age, and over trunk and extremities. Skin biopsy was compatible with bullous pemphigoid in two patients. Lesions regressed within a month of stopping DPP4 inhibitors in 9 patients while delayed regression up to 6 months in 4 patients. Overall, skin lesions remitted in all patients and did not recur. ConclusionAny new bullous lesion appearing while patient is on DPP4 inhibitors should be considered as bullous pemphigoid and should necessitate prompt withdrawal of the drug.

Bullous pemphigoid: can it occur in pediatric age?

Bullous pemphigoid in children is extremely infrequent, with no prevalence or incidence data in medical literature. Most of them predominate in elderly persons, resulting fundamental to consider that in medicine everything could be possible, and this disease could occur in children, so we must pay special attention to its clinical manifestations, to take the appropriate exams and in this way perform an effective treatment at the precise moment. Following, we present a clinical case of bullous pemphigoid that occurred in a pediatric patient attended at our university hospital.