Bone cement implantation syndrome during hip replacement in a patient with pemphigus and Parkinson’s disease: A case report

BACKGROUND Bone cement implantation syndrome(BCIS)is characterized by hypotension,arrhythmia,diffuse pulmonary microvascular embolism,shock,cardiac arrest,any combination of these factors,or even death following bone cement implantation.CASE SUMMARY An 80-year-old patient with pemphigus and Parkinson’s disease underwent total hip replacement under spinal subarachnoid block and developed acute pulmonary embolism after bone cement implantation.The patient received mask mechanical ventilation with a continuous intravenous infusion of adrenaline(2μg/mL)at a rate of 30 mL/h.Subsequently,the symptoms of BCIS were markedly alleviated,and the infusion rate of adrenaline was gradually reduced until the infusion was completely stopped 45 min later.The patient was then transferred to the Department of Orthopedics,and anticoagulation therapy began at 12 h postoperatively.No other complications were observed.CONCLUSION This is a rare case of BCIS in a high-risk patient with pemphigus and Parkinson’s disease.

Intra-abdominal inflammatory pseudotumor-like follicular dendritic cell sarcoma associated with paraneoplastic pemphigus: A case report and review of the literature

BACKGROUD Follicular dendritic cell(FDC)sarcomas are rare neoplasms that occur predominantly in the lymph nodes.They can also occur extranodally.Extranodal FDC sarcomas most commonly present as solitary masses.Inflammatory pseudotumor(IPT)-like FDC sarcomas,a subcategory of FDC sarcomas,are rarer than other sarcoma subtypes.They are composed of spindle or ovoid neoplastic cells and exhibit an admixture of plasma cells and prominent lymphoplasmacytic infiltration.Paraneoplastic pemphigus(PNP),also known as paraneoplastic autoimmune multiorgan syndrome,is a rare autoimmune bullous disease that is associated with underlying neoplasms.PNP has a high mortality,and its early diagnosis is usually difficult.CASE SUMMARY We describe a 27-year-old woman who presented with stomatitis,conjunctivitis,and skin blisters and erosions as her first symptoms of PNP with an intraabdominal IPT-like FDC sarcoma.The patient underwent surgical tumor resection and received tapering oral corticosteroid treatment.She showed no recurrence at the 1-year follow-up.CONCLUSION IPT-like FDC sarcomas are rare underlying neoplasms that have an uncommon association with PNP.PNP-associated FDC sarcomas predominantly occur in intra-abdominal sites and suggest a poor prognosis.Surgical resection is an essential and effective treatment for PNP and primary and recurrent FDC sarcomas.

Detection of ATP2C1 Gene Mutation in Familial Benign Chronic Pemphigus

Summary： The ATP2C1 gene mutation in one ease of familial benign chronic pemphigus was investigated.One patient was diagnosed as familial benign chronic pemphigus by pathology, ultrastructral examination and clinical features. Genomic DNA was extracted from blood samples. Mutation of ATP2CI gene was detected by polymerase chain reaction （PCR） and DNA sequencing. The results showed that deletion mutation was detected in ATP2C1 gene in this patient, which was 2374delTTTG. No mutation was found in the family members and normal individuals. It was coneluded that the 2374delTTTG mutation in ATP2C1 gene was the specific mutation for the clinical phenotype for this patient and was a de novo mutation.

Pseudopemphigoid as caused by topical drugs and pemphigus disease

Pseudopemphigoid can cause a chronic cicatricial conjunctivitis that is clinically identical to the manifestations seen in mucous membrane pemphigoid, a disorder with a common clinical phenotype and multiple autoimmune links. For the purpose of this review, we will describe pseudopemphigoid as caused by topical drugs, the most common etiology with ocular manifestations, and as caused by the pemphigus disease, a more rare etiology. Specifically, we will discuss the ophthalmological features of drug-induced cicatricial conjunctivitis, pemphigus vulgaris, and paraneoplastic pemphigus. Other etiologies of pseudopemphigoid exist that will not be described in this review including autoimmune or inflammatory conditions such as lichen planus, sarcoidosis, granulomatosis with polyangiitis(Wegener's granulomatosis), erythema multiforme(minor, major, and Stevens-Johnson syndrome), bullous pemphigoid, skin-dominated linear IgA bullous dermatosis, and skin-dominated epidermolysis bullosa acquisita. Prompt diagnosis of the underlying etiology in pseudopemphigoid is paramount to the patient's outcome as certain diseases are associated with a more severe clinical course, increased ocular involvement, and differential response to treatment. A completehistory and ocular examination may find early cicatricial changes in the conjunctiva that are important to note and evaluate to avoid progression to more severe disease manifestations. When such cicatricial changes are noted, proper diagnostic techniques are needed to help elucidate a diagnosis. Lastly, collaboration between ophthalmologists and subspecialists such as dermatologists, pathologists, immunologists, and others involved in the care of the patient is needed to ensure optimal management of disease.

Assessing the Effects of a Health Belief Model-Based Educational Program on Knowledge Attitudes and Practice (KAP) among Patients with <i>Pemphigus vulgaris</i>

Introduction: This study aimed to evaluate the effects of an educational program based on Health Belief Model on knowledge, attitudes and practice (KAP) in patients with Pemphigus vulgaris (PV) referred to Razi Hospital in Iran. Materials and Methods: This quasi-experimental study was conducted on 88 patients in 2013-2014. Subjects were divided into two intervention and control groups using block randomization. In addition to their usual care for both groups, the intervention group sat through a six-month self-care educational program in a specialized outpatient clinic. A self-designed questionnaire was used to gather information about demographic characteristics, PV related variables, and KAP-related questions. Data were analyzed using SPSS Software. p value of less than 0.05 was set as statistically significant. Results: mean scores of KAP increased significantly after intervention compared to control groups when adjusted for baseline differences of these scores and house ownership and employment status differences in two groups. Conclusion: study results show the effectiveness of an HBM based educational program on KAP in PV patients that can lead to adoption of self care behaviors and help them gain self efficacy in controlling their disease and assisting their treatment process, counting as a tertiary preventive measure.

ASSOCIATION BETWEEN PEMPHIGUS VULGARIS AND HUMAN LEUKOCYTE ANTIGEN IN HAN NATION OF NORTHEAST CHINA

Objective To investigate the relationship between pemphigus vulgaris （PV） and human leukocyte antigen （HLA） in Han nation of northeast China. Mothods Standard microcytotoxicity test and polymerase chain reaction-sequence specific primers method were used to detect the HLA class Ⅰ antigens and HLA-DRBI and DQBI alleles in 27 patients with PV and results were compared with control group. Gene and phenotype frequencies of HLA-A3, A26（10）, B60（40）, and B13 （27.99%, 48%; 16.11%, 30%; 23.02%, 41%; 16.11%, 30%, respectively） increased significantly in PV group compared with control （1.01%, 2%; 0.5%, 1%; 4.61%, 9%; 5,13%, 10%, respectively）. After P value correction, the difference of A3, A26 （10）, and B60 （40） between the two groups was still significant. The gene frequencies of HLA-DRB 1^＊ 140x （1401, 1404, 1405, 1407, 1408）, DRBI^＊I20x, and DQBI＊0503 alleles in PV group （42.26%, 25.46%, and 23.02%） were significantly higher than control group （5.09%, 7.74%, and 1.89%）. After P value correction, the difference was still significant between the two groups. Collusion PV significantly relates with HLA in PV patients of Han nation of northeast China.

Effects of chimeric molecule of recombinant Dsg3EC_(1-2) with toxin PE40 on T and B lymphocytes in Pemphigus Vulgaris

Objective:To observe the effects of the recombinant chimeric toxin Dsg3EC 1-2PE40 on T and B lymphocytes isolated from Pemphigus Vulgaris (PV) patients to further study its biological therapeutic function for PV. Methods:Recombinant chimeric toxin Dsg3EC 1-2PE40 was first identified, expressed and purified, and then its effects on T and B lymphocytes of PV patients in vitro were detected and quantified by ELISPOT assay and MTT assay.Results:The purity of the expressed protein Dsg3EC 1-2PE40 was up to 80%. In ELISPOT assay, with Dsg3EC 1-2PE40, the overall number of B cells that produce anti-Dsg3 antibodies among PV patients was only about 60% of the comparable number with Dsg3EC 1-2. The proliferation of T cells of PV patients was inhibited markedly by Dsg3EC 1-2PE40. There was significant difference between the different groups with Dsg3EC 1-2PE40 and Dsg3EC 1-2.Conclusion:The recombinant chimeric toxin Dsg3EC 1-2PE40 decrease the number of B cells that produce anti-Dsg3 antibodies in PV patients and can inhibit or kill T cells of PV patients in vitro.

Pemphigus vulgaris macroscopically and cytologically resembling oral squamous cell carcinoma

We describe the clinical, macroscopic, cytological, histopathological, immunohistochemical, serodiagnostic and aspects of pemphigus vulgaris (PV) in the oral gingiva that clinically mimicked oral squamous cell carcinoma (OSCC) in a 57-year-old Japanese man. He developed slight haphalgesia of the buccal gingiva around teeth numbers 18 and 19 2 years ago. A dentist diagnosed intractable ulcer, but the patient ignored the condition for about 2 years until a sharp pain in the gingiva worsened. He consulted an otolaryngologist, who referred the patient to our hospital under a cytological diagnosis of OSCC. An oral examination revealed several extensive painful erosions/ ulcers from the buccal and lingual gingiva around teeth numbers 18 to 21 to the distal alveolar mucosa of no. 18 and the buccal and lingual gingiva around tooth number 31. A presumptive diagnosis of PV with dysplastic changes was determined from cytological smears. The cytological Nikolsky test was positive. The diagnosis of PV was confirmed from clinical and histopathological findings of a biopsy specimen obtained from the perilesional site. Although the definitive diagnosis of PV required only 2 weeks after this patient presented at our hospital, 2 years had elapsed since the onset of oral lesions.

Two Cases of Hallopeau Type of Pemphigus Vegetans in Two Women in Burkina Faso

Pemphigus of Hallopeau (PH) is a mild and pustular form of pemphigus vegetans, termed initially as “pyodermite végétante” described by Hallopeau. We report cases in two black women. Two women aged 32 and 36 respectively (case 1 and case 2) were seen for a pustulosis associated with blisters and erosions. Their history and their review have found the mouth ulcerations. The clinical examination showed pustules on the cephalic end, the trunk, the limbs and the large folds. Lesions had a polycyclic aspect and spread in a centrifugal way, developing towards budding, squamous and scabby erosions quickly surrounded by new pustules. Regression left a pigmented macula. Histological examination of the skin lesion showed images similar to vulgaris pemphigus (PV), together with hyperacanthosis and papillomatosis. Aspects observed in immunofluorescence were identical to those of PV (case1). General corticoid therapy led to a rapid disappearance of lesions in Case 1 but in Case 2 the Lever protocol combining corticoid and methotrexate have been used. PH is rarely described in our regions. Clinical diagnosis is not obvious for the uninitiated. Beyond the clinical presentation, PH has the same paraclinical diagnosis and the same treatment as PV.

Preliminary Application of High-Frequency Ultrasound in the Differentiation of Pemphigus and Bullous Pemphigoid:An Observational Study

Objective:Ultrasonography is a diagnostic imaging technique used to visualize subcutaneous body structures for identification of possible pathologies.In this study,we aimed to explore the clinical significance of high-frequency ultrasound in differentiating the location of blisters between pemphigus and bullous pemphigoid.Methods:Eighteen patients were recruited in Department of Dermatology,Zhongda Hospital from 2020 to 2021 and divided into a pemphigus group(n=8)and a bullous pemphigoid group(n=10)according to the diagnostic criteria for each.Ultrasonographic images were collected using a 50-MHz high-frequency ultrasound system.The indexes of ultrasonic evaluation were the layer(epidermis or dermis),size,shape,internal echo,and boundary of the blister.Categorical variables are expressed as n(%),and differences were compared using Fisher’s exact test.Results:The ultrasonographic images in the pemphigus group showed an intraepidermal semi-arc or irregular anechoic or hypoechoic areas.The inferior borders were situated above the characteristic thin linear hyperechoic bands of the epidermis.A linear hypoechoic band was present at the dermoepidermal junction in some cases.In the ultrasonographic images of the bullous pemphigoid group,the linear hyperechoic bands of the epidermis were continuous and intact.An oval fluid anechoic area(subepidermal blister)was present immediately below the bands.The location of the blisters in the ultrasonographic images was significantly different between the 2 groups(P<0.001).Conclusion:High-frequency ultrasound can be used as an auxiliary means to differentiate the location of blisters between pemphigus and bullous pemphigoid.

Molecular cloning and protein expression of EC1-2 and EC3-4 epitopes of pemphigus vulgaris antigen

To clone and express EC1 2 and EC3 4 epitopes of PVA (pemphigus vulgaris antigen, desmoglein 3) in order to diagnose pemphigus and study the relationship between epitopes of PVA and anti PVA antibody Methods RNA was extracted from keratinocytes and the cDNA of epitopes EC1 2 and EC3 4 was synthesized by reverse transcription Amplified genes of EC1 2 and EC3 4 were inserted into the expression plasmid, PGEX 4T 1, and transformed into E coli BL21 by electric transduction Recombinant fusion proteins of EC1 2 and EC3 4 epitopes were expressed by IPTG induction These proteins were separated on SDS PAGE gels and electroblotted to nitrocellulose to detect the anti PVA antibody Results The sequences of cloned EC1 2 and EC3 4 genes were identical to the sequence registered in PC/GENE Expressed recombinant proteins reacted only to sera from patients with pemphigus vulgaris, not to sera from patients with bullous pemphigoid, systemic lupus erythematosus or normal persons Conclusions These recombinant proteins are very specific in antigenicity This may provide a new method for the diagnosis of pemphigus vulgaris (PV) or the differential diagnosis of other bullous cutaneous diseases via patient sera It is also helpful in understanding the relationship between adhesion molecules and the pathogenic mechanism of pemphigus vulgaris

Role of autoantibodies against the linker subdomains of envoplakin and periplakin in the pathogenesis of paraneoplastic pemphigus

Background The presence of autoantibodies against multiple epidermal proteins is an important feature in paraneoplastic pemphigus （PNP）. Circulating anti-desmoglein 3 autoantibody, the major pathogenic autoantibody in pemphigus vulgaris （PV）, has been proved pathogenic in PNP. Because of many clinical differences between PNP and PV, we speculate about the involvement of other autoantibodies in the pathogenesis of PNP. Envoplakin （EPL） and periplakin （PPL） are recognized by most PNP sera. Their linker subdomains are highly homologous and necessary for the association of intermediate filaments. Methods We characterized the autoantibodies against the linker subdomains of EPL and PPL in PNP patients＇ sera and their associated tumors by enzyme-linked immunosorbent assay （ELISA） and immunofluorence. We also applied the purified autoantibodies against EPL and PPL from PNP sera to cultured human epidermal keratinocytes （HEK）, to evaluate the changes of cell-cell adhesion. Results Autoantibodies against EPL and PPL were detected in most PNP patients by ELISA, and the decrease of these autoantibodies after removal of the tumors was roughly comparable to the improvement of clinical symptoms. Cultured tumor cells from PNP patients secreted these autoantibodies. Specific immunoglobulin receptors for EPL and PPL were found on B lymphocytes in tumors from PNP. Furthermore, purified anti-EPL and anti-PPL autoantibodies from PNP sera were capable of dissociating cultured human epidermal keratinocytes. Conclusion Autoantibodies against EPL and PPL may also be pathogenic in PNP.

Hallopeau type of pemphigus vegetans confined to the right foot： case report

In pemphigus vegetans, intertriginous areas are more commonly involved, and verrucous vegetations develop easily in these areas. In this article, we report a case of Hallopeau type of pemphigus vegetans confined to the right foot.

A Case Report on Paraneoplastic Pemphigus Associated Colonic Carcinoma

Paraneoplastic pemphigus mucocutaneous syndrome, has （PNP）, an acantholytic a universal association with malignancy and a poor prognosis, The clinical picture is characterized by painful mucosal erosions, ulcerations, and polymorphous skin lesions that progress to blistering eruptions on the trunk and extremities. In the reviews of all PNP, hematologic malignancies such as non-Hodgkin＇s lymphoma, chronic lymphocytic leukemia, and Castleman＇s disease account for the most part; nonhematologic malignancies are very seldom especially adenocarciuoma of colon, These will be illustrated in the following paper on the basis of a case report of a patient with colonic adenocarcinoma who presented with PNP.

Chinese Expert Proposal on the Diagnosis and Management of Pemphigus Vulgaris (2020)

Pemphigus vulgaris(PV)is a life-threatening autoimmune bullous disease that causes blisters and erosions on the skin and mucous membranes to standardize the diagnosis and treatment of PV,Chinese experts in this field were invited to make recommendations which are presented in this article.Pemphigus vulgaris can be divided into cutaneous mucous type,cutaneous type and mucous mucous dominant type according to the different clinical manifestations.The pathological manifestations of PV are acantholysis above the basal layer.The diagnosis of PV and the assessment of the severity of the disease are made in accordance with the clinical manifestations,histopathological features,immunofluorescence assay results,and detection of pathogenic serum antibodies.The first-line treatment of PV comprises systemic glucocorticoids.Early combination with immunosuppressive agents or rituximab is recommended for moderate and severe PV.Intravenous immunoglobulin administration is recommended for patients being treated with rituximab.Plasma exchange and stem cell transplantation can be performed if necessary.During the course of therapy,the disease activity should be closely monitored,and actions should be taken to prevent adverse reactions.

Autologous peripheral hematopoietic stem-cell transplantation in a patient with refractory pemphigus

The aim of this study is to explore the effective-ness of autologous peripheral hematopoietic stem-cell transplantation in the treatment of refractory pemphigus.A 35-year-old male patient presented with a 4-year history of recurrent bullae on his trunk and extremities.The dia-gnosis of pemphigus was made on the basis of the clinical,histologic and immunofluorescence findings.The patient had shown resistance to conventional therapy with gluco-corticoid and immunosuppressive agents.Two months before admission,he complained of hip joint pain.X-ray and CT scan revealed aseptic necrosis of the femoral head.Stem-cell mobilization was achieved by treatment with cyclophosphamide,granulocyte colony-stimulating factor(G-CSF)and rituximab.Peripheral blood stem cells were collected via leukapheresis and cryopreserved for later use.Immunoablation was accomplished by using cyclophospha-mide(200 mg/kg;divided into 50 mg/kg on days-5,-4,-3,and-2),antithymocyte globulin(ATG;10 mg/kg;divided into 2.5 mg/kg on days-6,-5,-4,and-3),and rituximab(1200 mg/d;divided into 600 mg/d on days 0 and 7).Autologous peripheral hematopoietic stem cell transplanta-tion was followed by reconstitution of the immune system which was monitored by flow cytometry.The glucocorti-coid was withdrawn immediately after transplantation.The pemphigus titer turned negative 6 weeks after transplanta-tion and remained negative.The patient was in complete drug-free remission with no evidence of residual clinical or serological activity of pemphigus during 1 year of follow-up.The patient’s response suggests that autologous peri-pheral hematopoietic stem cell transplantation may be a potential“cure”for refractory pemphigus.However,fur-ther studies are needed to evaluate the risk-benefit ratio of this approach in patients with pemphigus showing resist-ance to conventional therapy.

Dapsone-induced infectious mononucleosis-like syndrome in a patient with pemphigus vulgaris

Dapsone syndrome is characterized by high fever, skin rash, methemoglobinemia, liver toxicity, and generalized lymphadenopathy.The incidence of me dapsone syndrome is increasing with the extensive application of dapsone. However it is rarely reported in pemphigus. We present a case of ＂infectious mononucleosis-like＂ dapsone syndrome in a patient with pemphigus vulgaris. The syndrome appeared when he was being treated methylprednisolone, which was commonly used as a treatment for dapsone syndrome.

Expression Changes of Serum IL-1α,CCL2,and CXCL2 in Patients With Pemphigus

Objective:This study was performed to explore the possible changes of the serum levels of the cytokines including interleukin 1α(IL-1α),chemokine monocyte chemotactic protein 1(also known as chemokine[C-C motif]ligand 2[CCL2]),and C-X-C motif chemokine ligand 2(CXCL2)in patients with pemphigus.Methods:The expression levels of IL-1α,CCL2,and CXCL2 in the serum of 57 patients with pemphigus PV(including 42 patients in progressive stage and 15 patients in remission stage)and 31 healthy controls were examined by enzyme-linked immunosorbent assay.The indepent-samples t-test was used to compare the two groups.Oneway analysis of variance was used for multiple-group comparisons,and the post-hoc least significant difference test was used to detect differences among multiple groups.Results:The serum expression levels of CCL2 and IL-1a were all significantly higher in the patients in progressive stage than in the controls([2.69±0.23]ng/mL vs.[2.55±0.28]ng/mL,P=0.043;[0.62±0.27]ng/mL vs.[0.48±0.23]ng/mL,P=0.038,respectively).In addition,the serum expression level of CXCL2 was significantly higher in patients in progressive stage than in in the remission stage([61.70±46.38]ng/mL vs.[24.97±18.46]ng/mL,P=0.037).Sex,disease classification,disease severity,treatment,and mucosal involvement had no significant influence on the expression of IL-1α,CCL2,or CXCL2 in the serum of patients groups and controls(all P>0.05).Conclusion:IL-1α,CCL2,and CXCL2 are heavily involved in the occurrence and development of pemphigus and may be related to the activity of the disease.

Epidemiology of Pemphigus: A Single Center Experience in Morocco

Objective:Pemphigus is a life threatening autoimmune bullous disease which involves the skin and mucous membranes of the stratified squamous epithelium.The global distribution of Pemphigus varies according to genetic,ethnic,socioeconomic,and cultural backgrounds.The purpose of our study is to evaluate the epidemiological features of pemphigus a single center in Morocco and compare our results with those reported elsewhere.Methods:A retrospective analysis was conducted of 302 pemphigus patients seen between 1990 and 2020 in the Dermatology Department of Ibn Sina Hospital(Rabat,Morocco).We further collected all the Moroccan scientific researches published by now to compare.Results:The average annual incidence was 0.32/100,000 inhabitants.The incidence doubled to 0.72 in 2020.The most common variant was pemphigus vulgaris(125 cases)followed by pemphigus erythematosus(99 cases),pemphigus foliaceous(40 cases),and vegetans(27 cases).The female to male ratio was 0.75,the average age at onset was 53 years old and the mean duration of the disease before diagnosis was 13.36months.Conclusion:This study joins the main characteristics of pemphigus in the Maghreb and around the world(pemphigus vulgaris most frequent subtype).In 2020,an epidemiological peak occurred during the coronavirus disease 2019 pandemic;probably related to stress and delayed time consultation for fear of contracting the severe acute respiratory syndrome coronavirus 2.

Cutaneous Myiasis in a Neglected Case of Pemphigus Vulgaris:A Case Report

Introduction:Myiasis is an infestation of the tissues and organs of living vertebrates and humans by fly larvae,usually those belonging to the Calliphoridae family.The larvae feed on the host’s necrotic or living tissue.Case presentation:On mucocutaneous examination,the patient had multiple sinus tracts covered with purulent necrotic slough,and cylindrical maggots were crawling out of and around the areas of slough.The patient was diagnosed with wound myiasis caused byCochliomyia hominivorax.Turpentine oil flushes and oral ivermectin were prescribed.Discussion:Cutaneous myiasis can be papular,pustular,furuncular,and nodular.It is more common in people who have poor hygiene and intimate contact with domestic animals,such as people living in rural areas and tropical/subtropical countries.The chief aim of management is to remove the maggots as soon as possible,kill them to prevent maturation,promote wound healing,and prevent development of secondary complications.Conclusion:In the current case,the patient’s poor nursing and personal care and low socioeconomic condition predisposed her to secondary wound infection and maggot infestation.Such cases require prompt care to prevent secondary infection and septicemia.