Management of patients with pemphigus vulgaris during the COVID-19 pandemic: Experience of a second level dermatology center.
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ABSTRACT:
SUBMITTER: Diotallevi F
PROVIDER: S-EPMC7436698 | biostudies-literature |
REPOSITORIES: biostudies-literature
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Project description:Pemphigus vulgaris is an intraepidermal autoimmune mucocutaneous blistering disease whose etiopathogenesis includes various trigger factors, i.e., drugs and malignancies. We present a case of malignancy-exacerbated pemphigus vulgaris which required a careful diagnostic process in order to rule out paraneoplastic pemphigus, along with the challenges posed by the need of treating both cutaneous and oncologic diseases. Possible post-operative complications post-poned the start of first-line immunosuppressive treatment of pemphigus. Moreover, the infective risks had to be minimized during the peak of the COVID-19 pandemic in Italy. Intravenous immunoglobulins were chosen as "bridge" therapy before the tumor surgical excision, followed by rituximab in post-operative phase.
| S-EPMC8385714 | biostudies-literature
Project description:Pemphigus vulgaris is perhaps the most formidable disease encountered by dermatologists. In the days before steroid therapy the mortality rate was 95 per cent, death occuring usually within 14 months. The cause of death was septicaemia, starvation and toxic state. Corticosteroid, immunosuppressants and adjuvant therapy have reduced the mortality to 10-40 per cent with the cause of death being uncontrolled pemphigus, complications of corticosteroid and immunosuppressant therapy, septicaemia and thromboembolism. Elderly patients and patients with extensive lesions have higher mortality rate. Prognosis has further improved by intensive care, adequate fluid replacement, nutritional support, a co-herent antibacterial policy alongwith aggressive corticosteroid therapy and immunosuppressants. Plasmapheresis has been used in patients who fail to respond to conventional management. Extracorporeal photophoresis has been reported to be effective in patients with 'treatment resistance' pemphigus vulgaris.
| S-EPMC5533110 | biostudies-other
Project description:Pemphigus vulgaris (PV) is a life-threatening disease belonging to the pemphigus group of autoimmune intra-epidermal bullous diseases of the skin and mucosae. The therapeutic management of PV remains challenging and, in some cases, conventional therapy is not adequate to induce clinical remission. The cornerstone of PV treatment remains systemic corticosteroids. Although very effective, long-term corticosteroid administration is characterized by substantial adverse effects. Corticosteroid-sparing adjuvant therapies have been employed in the treatment of PV, aiming to reduce the necessary cumulative dose of corticosteroids. Specifically, immunosuppressive agents such as azathioprine and mycophenolate mofetil are widely used in PV. More recently, high-dose intravenous immunoglobulins, immunoadsorption, and rituximab have been established as additional successful therapeutic options. This review covers both conventional and emerging therapies in PV. In addition, it sheds light on potential future treatment strategies for this disease.
| S-EPMC5931200 | biostudies-other
Project description:Pemphigus is a rare group of autoimmune mucocutaneous blistering conditions for which the mainstay of treatment is immunosuppression. This is usually achieved with high dose corticosteroids as well as steroid sparing agents. Rituximab is now recommended as a first line treatment for moderate to severe pemphigus vulgaris, the commonest form of pemphigus, alongside corticosteroids. During the early stages of the COVID-19 pandemic the use of rituximab was reduced in our department due to its long term irreversible B-cell suppression. During the COVID-19 pandemic careful pharmacological selection was undertaken for our pemphigus patients to balance the risks of immunosuppression. To demonstrate this, we report three pemphigus patients who required treatment for COVID-19 and assessment throughout the pandemic. To date there has been limited published data regarding the clinical outcomes of pemphigus patients who have developed COVID-19 infections following rituximab infusions, especially in those patients who have received COVID-19 vaccinations. Following careful personalized consideration, all three pemphigus patients presented received rituximab infusions since the start of the COVID-19 pandemic. These patients had also received COVID-19 vaccinations prior to becoming infected with COVID-19. Each patient had a mild COVID-19 infection after receiving rituximab. We advocate for all pemphigus patients to have a full course of COVID-19 vaccinations. Antibody response to COVID-19 vaccinations should ideally be confirmed by measuring pemphigus patient's SARS-CoV-2 antibodies prior to receiving rituximab.
| S-EPMC10043414 | biostudies-literature
Project description:Severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) is a new coronavirus responsible for the pandemic named coronavirus disease 2019 (COVID-19). The disease causes SARS with a significant morbidity and mortality. We provide a review with a focus on COVID-19 in dermatology. We discuss triage of suspected infectious patients, protection of medical doctors and nurses. We discuss the available data on cutaneous symptoms, although disease-specific symptoms have yet not been observed. COVID-19 is a challenge for the treatment of dermatologic patients, either with severe inflammatory disorders or with skin cancer. The consequences for systemic treatment are obvious but it will be most important to collect the clinical data for a better decision process. Last but not least, education in dermatology for students will not be temporarily possible in the classical settings. COVID-19, although not a skin disease, by itself has an immense impact on dermatology.
| S-EPMC7235502 | biostudies-literature
Project description:Pemphigus vulgaris is an autoimmune skin disorder with development of blisters in the skin and mucosa, and it can be a life-threatening disease if not treated. Corticosteroids have been a cornerstone for treating PV, but because of side effects the treatment is combined with other conventional immune modulating drugs and rituximab. The Danish treatment protocol for pemphigus vulgaris is similar to the other Scandinavian countries, and therefore this study is of importance for clinicians in the Scandinavian countries as well as other European countries. We retrospectively identified all patients with Pemphigus vulgaris in our tertiary center over a 7-year period in order to register patient characteristics, treatment, adverse events, comorbidities and the effect of prednisolone dose on remission. In this study 19 patients met the inclusion criteria and remission was seen after a mean of 19.9 weeks, and relapse was seen in 50% after the mean time of 15 weeks. Time to relapse in our study is relatively short compared to studies in which rituximab is used as a first-line drug in treating pemphigus vulgaris.
| S-EPMC6896223 | biostudies-literature
Project description:The majority of pemphigus vulgaris (PV) patients suffer from a live-threatening loss of intercellular adhesion between keratinocytes (acantholysis). The disease is caused by auto-antibodies that bind to desmosomal cadherins desmoglein (Dsg) 3 or Dsg3 and Dsg1 in mucous membranes and skin. A currently unresolved controversy in PV is whether apoptosis is involved in the pathogenic process. The objective of this study was to perform preclinical studies to investigate apoptotic pathway activation in PV pathogenesis with the goal to assess its potential for clinical therapy. For this purpose, we investigated mouse and human skin keratinocyte cultures treated with PV antibodies (the experimental Dsg3 monospecific antibody AK23 or PV patients IgG), PV mouse models (passive transfer of AK23 or PVIgG into adult and neonatal mice) as well as PV patients' biopsies (n=6). A combination of TUNEL assay, analyses of membrane integrity, early apoptotic markers such as cleaved poly-ADP-ribose polymerase (PARP) and the collapse of actin cytoskeleton failed to provide evidence for apoptosis in PV pathogenesis. However, the in vitro and in vivo PV models, allowing to monitor progression of lesion formation, revealed an early, transient and low-level caspase-3 activation. Pharmacological inhibition confirmed the functional implication of caspase-3 in major events in PV such as shedding of Dsg3, keratin retraction, proliferation including c-Myc induction, p38MAPK activation and acantholysis. Together, these data identify low-level caspase-3 activation downstream of disrupted Dsg3 trans- or cis-adhesion as a major event in PV pathogenesis that is non-synonymous with apoptosis and represents, unlike apoptotic components, a promising target for clinical therapy. At a broader level, these results posit that an impairment of adhesive functions in concert with low-level, non-lethal caspase-3 activation can evoke profound cellular changes which may be of relevance for other diseases including cancer.
| S-EPMC4352034 | biostudies-literature
Project description: Not available
| S-EPMC8107516 | biostudies-literature
Project description:ObjectiveThe COVID-19 pandemic has forced healthcare providers worldwide to bring in changes in the way cancer patients are cared for. Many cancer departments globally have brought in changes to their daily practice. This article is about our experience of evolving "COVID 19 PROTOCOL" devised in our department and taking a shape to suit a health care system with limited budget.Materials and methodsAll the patient census & details of department of surgical oncology, Royapettah cancer hospital, from month of March 2020 to July 2020, who were subjected to COVID protocol were compared to patient census of similar duration in immediate past five months of October 2019 to February 2020. The data from out-patient department, ward in-patient census and healthcare personnel data was analyzed.ResultsThere was a drop to 63.5% in OP census and 61.6% in IP census. There was a drop to 64.5% in number of major cases operated during initial phases of COVID pandemic. Health care workers were also infected with the COVID but cross infectivity can be checked if proper steps to adhere to an institutional protocol based on general measures of cleanliness are taken.ConclusionAdherence to an institutional protocol based on compliance to general measures of masking, hand washing and social distancing plays a major role in minimizing disease spread. The Royapettah COVID protocol, though in process of evolution, can be recommended for any health care center with limited resources.
| S-EPMC7834458 | biostudies-literature
Project description:Pemphigus vulgaris (PV) is a mucocutaneous blistering disease characterized by IgG autoantibodies against the stratified squamous epithelium. Current understanding of PV pathophysiology does not explain the mechanism of acantholysis in patients lacking desmoglein antibodies, which justifies a search for novel targets of pemphigus autoimmunity. We tested 264 pemphigus and 138 normal control sera on the multiplexed protein array platform containing 701 human genes encompassing many known keratinocyte cell-surface molecules and members of protein families targeted by organ-non-specific PV antibodies. The top 10 antigens recognized by the majority of test patients' sera were proteins encoded by the DSC1, DSC3, ATP2C1, PKP3, CHRM3, COL21A1, ANXA8L1, CD88 and CHRNE genes. The most common combinations of target antigens included at least one of the adhesion molecules DSC1, DSC3 or PKP3 and/or the acetylcholine receptor CHRM3 or CHRNE with or without the MHC class II antigen DRA. To identify the PV antibodies most specific to the disease process, we sorted the data based on the ratio of patient to control frequencies of antigen recognition. The frequency of antigen recognition by patients that exceeded that of control by 10 and more times were the molecules encoded by the CD33, GP1BA, CHRND, SLC36A4, CD1B, CD32, CDH8, CDH9, PMP22 and HLA-E genes as well as mitochondrial proteins encoded by the NDUFS1, CYB5B, SOD2, PDHA1 and FH genes. The highest specificity to PV showed combinations of autoantibodies to the calcium pump encoded by ATP2C1 with C5a receptor plus DSC1 or DSC3 or HLA-DRA. The results identified new targets of pemphigus autoimmunity. Novel autoantibody signatures may help explain individual variations in disease severity and treatment response, and serve as sensitive and specific biomarkers for new diagnostic assays in PV patients.
| S-EPMC3591405 | biostudies-literature