scholarly journals Successful induction treatment of bullous pemphigoid using reslizumab: a case report

2021 ◽  
Vol 17 (1) ◽  
Author(s):  
Hyo-In Rhyou ◽  
Song-Hee Han ◽  
Young-Hee Nam

Abstract Background Bullous pemphigoid (BP) is a potentially life-threatening autoimmune blistering disease which is characterized by autoantibodies against hemidesmosomal proteins of the skin and mucous membranes. In recent years, the role of eosinophil and immunoglobulin E autoantibodies have been further elucidated in BP, and have been considered potential therapeutic targets. Case presentations A 67-year-old male presented with erythematous bullous eruption. The skin eruption was located on whole body, and suggested BP. Peripheral blood eosinophil count and total immunoglobulin E markedly elevated in initial laboratory findings. Topical and systemic steroid (methylprednisolone 2 mg/kg/day) treatment was started, and his skin symptoms worsened repeatedly, whenever systemic steroid were reduced. On admission day 29, reslizumab (anti-interleukin-5) 3.5 mg/kg was administered intravenously to the patients. The bullous skin lesion began to improve rapidly, and methylprednisolone (8 mg/day) was reduced without any worsening of symptoms during two doses of reslizumab. Conclusions We report a case of successful treatment response to reslizumab administration in a patient with BP. Further studies are needed to confirm the role of anti-interleukin-5 as a treatment for BP in the future.

Allergy ◽  
2020 ◽  
Author(s):  
Guogang Xie ◽  
Fengming Ding ◽  
Lei Han ◽  
Dongning Yin ◽  
Hongzhou Lu ◽  
...  

2020 ◽  
Vol 21 (3) ◽  
pp. 913 ◽  
Author(s):  
Hayato Nomura ◽  
Mutsumi Suganuma ◽  
Takuya Takeichi ◽  
Michihiro Kono ◽  
Yuki Isokane ◽  
...  

The serine proteases kallikrein-related peptidase (KLK) 5 and KLK7 cleave cell adhesion molecules in the epidermis. Aberrant epidermal serine protease activity is thought to play an important role in the pathogenesis of atopic dermatitis (AD). We collected the stratum corneum (SC) from healthy individuals (n = 46) and AD patients (n = 63) by tape stripping and then measuring the trypsin- and chymotrypsin-like serine protease activity. We also analyzed the p.D386N and p.E420K of SPINK5 variants and loss-of-function mutations of FLG in the AD patients. The serine protease activity in the SC was increased not only in AD lesions but also in non-lesions of AD patients. We found, generally, that there was a positive correlation between the serine protease activity in the SC and the total serum immunoglobulin E (IgE) levels, serum thymus and activation-regulated chemokine (TARC) levels, and peripheral blood eosinophil counts. Moreover, the p.D386N or p.E420K in SPINK5 and FLG mutations were not significantly associated with the SC’s serine protease activity. Epidermal serine protease activity was increased even in non-lesions of AD patients. Such activity was found to correlate with a number of biomarkers of AD. Further investigations of serine proteases might provide new treatments and prophylaxis for AD.


2021 ◽  
Vol 12 ◽  
Author(s):  
Xiaoyan Zhang ◽  
Dongjiang Sui ◽  
Dong Wang ◽  
Lina Zhang ◽  
Ruiyan Wang

The programmed cell death protein 1 inhibitor pembrolizumab, an immune checkpoint inhibitor, has subsequently been approved for the treatment of a wide variety of malignant tumors. Compared with conventional chemotherapy, immunotherapy is associated with a unique set of immune reactions, known collectively as immune-related adverse events. Although often mild, dermatologic toxicity can occasionally be high grade and potentially life-threatening. Here we describe a rare case of bullous pemphigoid (BP) associated with pembrolizumab. A 79-year-old male patient presented with scattered erythema, papules, blisters, and pruritus after pembrolizumab treatment. Then, the rash gradually aggravated and spread to the whole body. The extensive edematous erythema, blisters, bullae, and blood blisters were loose and easy to rupture, forming an erosive surface and with pruritus and obvious pain. The hemidesmosomal protein BP180 (type XVII collagen) was detectable in the serum, and the histological examination diagnosis was bullous pemphigoid. After 10 days of glucocorticoid (methylprednisolone, iv, 80 mg/day) treatment, new blister formation ceased. We need to increase the awareness on and facilitate the earlier identification of the cutaneous adverse effects of BP with immunotherapy so that treat can begin early in order to limit the duration and severity of toxicity.


2019 ◽  
Vol 8 (3) ◽  
pp. 205846011983469 ◽  
Author(s):  
Elie Barakat ◽  
Nathalie Guischer ◽  
Frédéric Houssiau ◽  
Frederic E. Lecouvet

The magnetic resonance imaging (MRI) appearance of an “established” bone marrow infarct is well-known, consisting of an area of preserved bone marrow signal surrounded by a serpiginous line. We report the uncommon observation of the very early phases of appearance of a bone marrow infarct, showing its progressive de novo appearance on MR images paralleling clinical symptoms and high-dose systemic steroid administration in a young female patient, presenting with acute knee pain. The initial knee MR examination performed one week after pain onset showed no abnormality. One week later, a second examination showed subtle ill-defined dotted signal abnormalities of the bone marrow of uncertain significance, of high signal on PDFS sequences. A third MR study obtained again one week later showed more evident findings with confluence of the high signal “dots” into a serpiginous line with a geographical appearance of the lesion, corresponding to the typical MRI presentation of bone marrow infarcts. Follow-up MRI at seven weeks showed definitive stability of this bone marrow infarct. A whole-body MRI performed for whole skeleton screening revealed multiple bone marrow infarcts typical for systemic avascular necrosis. This case represents a novel observation of the “birth” of a bone marrow infarct, from early intriguing changes to its typical ring-shaped appearance on MR images. It also reminds of the key role of MRI for early diagnosis of bone marrow infarcts and illustrates the emerging role of whole-body MRI for the detection of multifocal, asymptomatic skeletal involvement by ischemic lesions in systemic osteonecrosis.


2021 ◽  
Author(s):  
Ge Wu ◽  
Teng Zhang ◽  
Xiaohua Douglas Zhang ◽  
Baoqing Sun

Abstract BackgroundImmunoglobulin E (IgE) plays an important role in asthma, but a few cases exhibited extremely high levels of serum total IgE. This study aimed to investigate the profiles of complications, severity, and sensitizations in asthma patients with serum total IgE level >1000 kUA/L.MethodsWe retrospectively analyzed 170 asthma patients with serum total IgE levels >1000 kUA/L from the in-patient database of First Affiliated Hospital of Guangzhou Medical University from January 2014 to June 2019. Available information including age, gender, body mass index (BMI), diagnosis, results of blood routine, pulmonary function, fractional exhaled nitric oxide (FeNO), induced sputum (if any), IgE (both total and specific) and medication records were analyzed. ResultsAbout 15% patients had at least one complication, and 78.82% patients were positive for at least one allergen. The top two complications were airway infections (44.71%) and rhinosinusitis (41.18%), followed by hypertension and/or cardiovascular diseases (20.59%) and COPD (12.94%). And in patients without sensitization, rhinosinusitis accounted for the highest proportion over all complications (45.83%). Serum total IgE levels did not differ among patients with different complications. Overall, mites had the highest positive rate (59.4%). In minors, the positive rates of mites (81.25% vs. 54.35%, P <0.01) and food (68.75% vs. 39.86%, P <0.01) were significantly higher than those in adults. Serum total IgE levels were positively correlated to house dust mite specific IgE levels (r=0.23, P <0.05), peripheral blood eosinophil counts (r=0.21, P <0.01) and number of confirmed sIgE positivity (r=0.19, P <0.01) and optimal scaling analysis showed that asthma severity was associated with A. fumigatus specific IgE levels. ConclusionsIn asthma patients with markedly increased serum total IgE levels (>1000 kUA/L), the most common two complications were airway infections and rhinosinusitis despite of sensitization. A. fumigatus specific IgE levels were closely associated with total IgE levels and asthma severity.


Dermatology ◽  
2021 ◽  
pp. 1-8
Author(s):  
Khalaf Kridin ◽  
Ralf J. Ludwig ◽  
Dana Tzur Bitan ◽  
Arnon D. Cohen

Background: Bullous pemphigoid (BP) and asthma both share a pathogenic role of eosinophils and immunoglobulin E (IgE) and favorable response for corticosteroids and omalizumab. However, the association between these conditions is yet to be investigated. We sought to estimate the risk of having BP among patients previously diagnosed with asthma and to characterize patients with coexistent BP and asthma. Methods: Utilizing the dataset of Clalit Health Services, a population-based case-control study was conducted comparing BP patients (n = 3,924) with age-, sex-, and ethnicity-matched control subjects (n = 19,280) regarding the presence of asthma. Logistic regression models were utilized for univariate and multivariate analyses. Results: The prevalence of preceding asthma was higher in patients with BP than in control subjects (11.1 vs. 7.9%, respectively; p < 0.001). A history of asthma was associated with a 50% increase in the risk of BP (OR 1.45; 95% CI 1.30–1.62). The association was not altered greatly after adjusting for demographics (adjusted OR 1.43; 95% CI 1.28–1.61) as well as for demographics and comorbidities (adjusted OR 1.40; 95% CI 1.25–1.57). The average (SD) latency between the diagnosis of asthma and the development of BP was 12.5 (14.7) years. When compared with other patients with BP, those with a dual diagnosis of BP and asthma were older, had higher BMI, and were more frequently managed by corticosteroids and immunosuppressive and immunomodulatory adjuvants. Conclusions: Asthma confers a predisposition to the development of BP. Awareness of this association may be of help for physicians managing patients with BP and asthma. Further research is required to elucidate the mechanism underlying this observation.


2003 ◽  
Vol 23 (03) ◽  
pp. 109-112
Author(s):  
A. Hirt ◽  
C. Zwicky ◽  
W.A. Wuillemin ◽  
K. Leibundgut

SummaryA boy (age: 71/12 years) with acute lymphoblastic leukaemia developed thrombosis of the sinus sagitalis superior with secondary haemorrhagic infarction while on induction treatment with vincristine, prednisone, and asparaginase. Based on this report, the potential pathogenic mechanisms are discussed with respect to congenital prothrombotic defects as well as to the role of antileukaemic treatment. Current hypotheses on mechanisms for thromboembolism in children and proposed prophylactic strategies are briefly summarized.


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