Bullous pemphigoid associated with anti-programmed cell death protein 1 and anti-programmed cell death ligand 1 therapy: A case series of 13 patients.

We present a case series of 13 patients, the first Australian single-centre study of bullous pemphigoid (BP) associated with immune checkpoint inhibitors (ICI): cytotoxic T-lymphocyte antigen (CTLA4) and programmed cell death receptor (PD1) inhibitors. All our patients achieved adequate control of BP with a combination of treatments including oral prednisolone, intravenous immunoglobulin, rituximab and omalizumab. The majority of patients ceased or interrupted immunotherapy treatment upon diagnosis of BP and greater tumour progression was seen in the cohort who ceased immunotherapy.

Association between chronic kidney disease and risk of bullous pemphigoid: a nationwide population-based cohort study.

BACKGROUND: Studies have shown that bullous pemphigoid (BP) occurs in patients with chronic kidney disease (CKD). However, the risk of developing BP in patients with CKD remains inconclusive. OBJECTIVE: To investigate whether CKD increases the risk of BP. METHODS: Participants were recruited from the National Health Insurance Database of Taiwan between 2007 and 2018. Overall, 637,664 newly diagnosed patients with CKD and 637,664 age-, sex-, and comorbidity-matched non-CKD participants were selected. A competing risk model was used to evaluate the risk of development of BP. RESULTS: After adjusting for age, sex, and comorbid diseases in the multivariate model, CKD was a significant risk factor for BP (adjusted hazard ratio [aHR]: 1.29; 95% confidence interval [CI]: 1.17-1.42; p < 0.001). CKD patients were classified into the dialytic or non-dialytic groups and compared to non-CKD participants, and this revealed that patients with dialysis-dependent CKD had the highest risk of BP (aHR 1.75; 95% CI 1.51-2.03), followed by patients with non-dialysis-dependent CKD (aHR 1.20; 95% CI 1.08-1.32). LIMITATIONS: We lacked detailed laboratory data on the severity of CKD. CONCLUSIONS: Compared with individuals without CKD, those with CKD had a 1.3-fold increased risk of BP. Patients with dialysis-dependent CKD had an even higher BP risk (1.8-fold).

Anti-laminin 332 antibodies in graft-versus-host disease-associated bullous pemphigoid after allogeneic peripheral blood stem cell transplantation.

We report a 48-year-old woman with bullous pemphigoid (BP) with antibodies against the B3 subunit of laminin 332 after the development of graft-versus-host disease (GVHD). She was diagnosed with recurrent acute lymphoblastic leukemia at 40 years of age and underwent two rounds of allogeneic peripheral blood stem cell transplantations (PBST). Two and a half years after the second PBST, multiple tense blisters appeared on her face, hands, and lower legs. The diagnosis of BP was based on hematoxylin eosin and immunofluorescence staining and immunoblotting analyses. A combination regimen of topical corticosteroids (clobetasol propionate) and tetracycline/niacinamide was administered. Complete clinical resolution was achieved after four weeks of therapy without the use of immunosuppressive drugs. To maintain the graft-versus-tumor effect, topical corticosteroids and immunomodulatory drugs are preferred for BP after hematopoietic stem cell transplantation considering the risk of recurrence of hematologic malignancies. To date, there have been no reports of successful treatment of GVHD-associated BP without immunosuppressive drugs. Chronic GVHD is characterized by the production of autoantibodies. Furthermore, this autoimmune subepidermal blistering disease, BP, may be a manifestation of chronic GVHD. However, the precise mechanism of autoantibody production in chronic GVHD is not yet fully elucidated.

Necrotizing Fasciitis-Severe Complication of Bullous Pemphigoid: A Systematic Review, Risk Factors, and Treatment Challenges.

Background and objectives: Bullous pemphigoid (BP), the most common subepidermal autoimmune skin blistering disease (AIBD) has an estimated annual incidence of 2.4 to 42.8 new cases per million in different populations, designating it an orphan disease. Characterized by disruption of the skin barrier combined with therapy-induced immunosuppression, BP could pose a risk for skin and soft tissue infections (SSTI). Necrotizing fasciitis (NF) is a rare necrotizing skin and soft tissue infection, with a prevalence of 0.40 cases per 100,000 to 15.5 cases per 100,000 population, often associated with immunosuppression. Low incidences of NF and BP classify them both as rare diseases, possibly contributing to the false inability of making a significant correlation between the two. Here, we present a systematic review of the existing literature related to the ways these two diseases correlate. Materials and methods: This systematic review was conducted according to the PRISMA guidelines. The literature review was conducted using PubMed (MEDLINE), Google Scholar, and SCOPUS databases. The primary outcome was prevalence of NF in BP patients, while the secondary outcome was prevalence and mortality of SSTI in BP patients. Due to the scarcity of data, case reports were also included. Results: A total of 13 studies were included, six case reports of BP complicated by NF with six retrospective studies and one randomized multicenter trial of SSTIs in BP patients. Conclusions: Loss of skin integrity, immunosuppressive therapy, and comorbidities commonly related to BP patients are risk factors for necrotizing fasciitis. Evidence of their significant correlation is emerging, and further studies are deemed necessary for the development of BP-specific diagnostic and treatment protocols.

Acquired Hemophilia A associated with bullous pemphigoid: A rare combination.

Acquired Hemophilia A (AHA) is a rare autoimmune disorder, caused by the development of circulating autoantibodies against coagulation factor VIII (FVIII). AHA is associated with bullous pemphigoid in 2% of patients. We report a case of a 74-year-old man admitted with anemia and a tense subcutaneous and muscular hematoma in the right thigh. Blood analysis confirmed AHA. The patient had a recent diagnosis of bullous pemphigoid. Response to bypass agents and corticosteroids was good with bleeding control and normalization of FVIII and negative autoantibodies, respectively. In a 3-month follow-up period after tapering and stopping prednisolone, a relapse occurred, and immunosuppression was reinitiated. An early diagnosis and effective treatment in AHA are essential to reduce morbimortality. A careful tapering of immunosuppression is important to minimize FVIII inhibitor recurrence, as observed in this case.

A new eruption of bullous pemphigoid following mRNA COVID-19 vaccination.

The rapid development and implementation of COVID-19 vaccines throughout the global population has given rise to unique, rare, adverse skin reactions. This case report describes an elderly man with new-onset bullous pemphigoid following the second dose of the Pfizer-BioNTech (mRNA) COVID-19 vaccine.

Diagnosis and treatment of bullous pemphigoid that developed twice after total knee replacement arthroplasty: a case report.

BACKGROUND: Total knee replacement arthroplasty (TKA) is frequently performed in South Korea. Simple swelling-associated blistering around the periphery of the operative wound is a well-known adverse effect. However, in rare cases, the blisters are bullous pemphigoid (BP). CASE PRESENTATION: A 75-year-old male presented with knee pain that had not improved despite 5 years of medication. We performed TKA of the left knee, placing a Stryker posteriorly stabilized prosthesis. Three days later, blisters developed near the buttocks and thighs and, on day 10 after surgery, around the operative site. A skin biopsy revealed BP. Commencing on day 14 after surgery, prednisolone 10 mg was administered twice daily. The symptoms improved by 3 weeks after surgery and were healed at 4 months. After 1 year, we performed TKA of the right knee. On day 2 after surgery, as formerly, blisters developed on the buttocks and an immediate biopsy revealed BP. Commencing on day 3 after surgery, prednisolone 10 mg was administered twice daily. On day 10 after surgery, the blisters on the buttocks had improved and no blisters were observed at the surgical site. All symptoms had resolved by 2.5 months after surgery. CONCLUSIONS: After TKA surgery, generalized BP may develop, diagnosed via skin biopsy. A quick diagnosis is important because early treatment can prevent symptom progression and shorten treatment.

Bullous pemphigoid in patients receiving peritoneal dialysis: a case series and a literature survey.

Bullous pemphigoid (BP) is an autoimmune subepidermal blistering disease. Although several cases of BP in end-stage renal disease patients receiving peritoneal dialysis (PD) or hemodialysis have been reported, the incidence of BP in these patients remains unknown. We recently experienced three PD patients diagnosed with BP. The skin injury was likely to be a trigger of BP in all the three PD patients. Nifedipine and icodextrin exposures were possible factors directly or indirectly affecting the onset of BP, because they were common in the three cases. We also report that the incidence of BP in PD patients was 3/478.3 person-years in a single-center 10-year study. This case series with a literature survey describes that the skin and tissue injuries are potential triggers responsible for the onset of BP in dialysis patients and that the incidence of BP in these patients seems to be much higher than that in the general population.

Localized bullous pemphigoid after knee replacement surgery.

Bullous pemphigoid (BP) most commonly presents as widespread, itchy, tense blisters in older patients. Localized bullous pemphigoid is a less common form of BP that can be more difficult to diagnose because of its similarity to more common conditions such as allergic contact dermatitis or bullous cellulitis. Prompt recognition of localized BP is important so that appropriate treatment can be started. We present a 57-year-old woman who presented with pruritic tense bullae overlying the surgical scar from a knee replacement 6 months prior on her anterior right knee. This case illustrates the potential for localized BP to be triggered by surgical procedures.

Radiation-induced bullous pemphigoid after surgery for soft tissue sarcoma: About a rare case.

INTRODUCTION: Radiation therapy may cause a range of side effects of the skin within the irradiated area. Not of all the reactive effects of the skin induced by radiation therapy have to be related to some forms of radiodermatitis, and when non-standard clinical presentations overcome, it may be necessary to undertake appropriate diagnostic tools to not be in trap of wrong diagnosis. CLINICAL CASE DESCRIPTION: A 76 years-old man undertook resection surgery after a neoadjuvant radiation therapy for a soft tissue sarcoma of his groin region. After surgery, he developed an acute skin reaction comparable with a severe form of radiodermatitis. Despite cares, his clinical status got worse. Only skin biopsies guided us to the right diagnosis: it was a form of a bullous pemphigoid induced by radiation therapy. The consequent appropriate treatment was finally resolute. CONCLUSION: These forms have to be recognized in time, to undertake skin biopsies as soon as an evocative clinical presentation appears. The appropriate treatment, which consists in local or systemic corticotherapy, is resolute in most cases.

[Bullous pemphigoid induced by cancer immunotherapy]. / Pemphigoïde bulleuse induite par l'immunothérapie antitumorale.

Bullous pemphigoid is the most frequent autoimmune bullous dermatosis. Cases induced by immune checkpoint inhibitors have been described. This antitumor immunotherapy changed the treatment of several malignant tumors, especially melanoma and non-small cell lung-cancer. Nevertheless, immune adverse events are common. We present a case of bullous pemphigoid induced by pembrolizumab to illustrate the link between this disease and the treatment with immune checkpoint inhibitors.

La pemphigoïde bulleuse (PB) est la dermatose bulleuse auto-immune la plus fréquente. De nombreux cas induits par les inhibiteurs des points de contrôle immunitaire (IPCI) ont été rapportés. L'immunothérapie antitumorale a révolutionné la prise en charge de certaines tumeurs malignes, notamment du mélanome et du carcinome pulmonaire non à petites cellules. Toutefois, les effets indésirables immunologiques sont fréquents. Nous présentons un cas de PB induite par le pembrolizumab pour illustrer le lien entre cette maladie bulleuse et le traitement par IPCI.

Cancer is not a risk factor for bullous pemphigoid: 10-year population-based cohort study.

BACKGROUND: Bullous pemphigoid (BP) is the most common autoimmune bullous disease. Whether there is an increased risk for subsequent BP among patients with cancer is still unclear. OBJECTIVES: To evaluate the risk for subsequent BP in patients with cancer. METHODS: This nationwide population-based cohort study was based on data obtained from the Taiwan National Health Insurance Database between 2000 and 2011. A total of 36 838 patients with cancer and 147 352 age-, sex- and index-date-matched controls were recruited. The hazard ratio (HR) of subsequent BP in the patients with cancer was analysed using a Fine-Gray competing risk regression model with mortality as the competing event. RESULTS: The incidence of BP per 100 000 person-years was 17·2 in the patients with cancer and 19·8 in the controls; therefore, the crude incidence rate ratio was 0·87 [95% confidence interval (CI) 0·53-1·36]. The HR of subsequent BP in the patients with cancer was 0·47 (95% CI 0·23-0·94) using the Fine-Gray competing risk regression model. Age (HR 1·05, 95% CI 1·03-1·07), diabetes mellitus (HR 1·69, 95% CI 1·10-2·59) and cerebrovascular disease (HR 2·14, 95% CI 1·36-3·34) were independent risk factors for BP. CONCLUSIONS: The incidence of BP in patients with cancer was not higher than in the control group. Cancer is not a risk factor for BP.

Infantile bullous pemphigoid successfully treated with combined pulse corticosteroids and high-dose IVIG.

Bullous pemphigoid is the most common autoimmune blistering disorder in the elderly. It affects people aged 70 years or older. Clinically it is characterized by intensely pruritic eruption consisting of widespread tense blisters on an erythematous background. It is associated with cellular and humoral responses against hemidesmosomal components of the skin and mucous membranes. In contrast, infantile bullous pemphigoid is exceedingly rare disease and presents with some unique features like favorable prognosis, possible association with vaccination, and primary involvement of acral surfaces. Herein, we present a case of 4,5-month-old infant with neonatal pemphigoid, successfully treated with a combination of intravenous immunoglobulins and pulse methylprednisolone.