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INTRODUCTION Behcet's disease (BD) is a systemic vasculitis of unknown cause. The spectrum of the disease ranges from mucocutaneous manifestations to other organ diseases with relevant morbidity. Associations between disease severity and male sex, earlier age at onset, and the presence of erythema nodosum have been described. OBJECTIVES To evaluate clinical factors associated with manifestations of severe disease in a single-center cohort. METHODS A longitudinal, prospective, unicentric cohort study with patients followed in a specialized outpatient clinic between 1981 and 2020. Severe BD was defined as a Krause total clinical severity score >4 points. RESULTS We included 243 patients, of whom 31% were male, with an average follow-up time of 14.6 years. Regarding organ manifestations, all patients had mucous manifestations (N=243, 100%), 133 (55%) skin, 104 (43%) joint, 71 (29%) ocular, 48 (20%) vascular, 47 (19%) neurological, 22 (9%) gastrointestinal and 1 (0.4%) cardiac involvement by BD. One hundred fifty-six (64%) patients were classified as having severe BD. Severe BD was more frequent in men (OR=2.004, p=0.024), increasing with age (OR=1.021 per year, p=0.037), in the presence of skin manifestations (OR=4.711, p

Toxic epidermal necrolysis (TEN) and Stevens-Johnson syndrome (SJS) are potentially fatal mucocutaneous diseases characterized by extensive necrosis and exfoliation of the epidermis. TEN and SJS are most often caused by various kinds of drugs. Other risk factors for SJS/TEN include pneumonia infection, HIV infection, genetic factors, underlying immune diseases, and tumors. SJS and TEN were first identified in 1922, but at present, a widely recognized view is that SJS and TEN represent phases in the continuous progress of the same disease. SJS/TEN has a very high mortality, but is rare, and cases of SJS/TEN combined with systemic lupus erythematosus (SLE) are even less common. Occasionally, acute cutaneous manifestations of SLE and SJS/TEN can be phenotypically similar, both causing extensive epidermal necrosis. In this paper, we present a recent case of a 32-year-old female SLE patient with a druginduced (the health product, astaxanthin) TEN/SJS. To provide context to this case, we have reviewed relevant case studies published in English, accessed via PubMed databases. The search covers all published case studies from 1988 to 2019. We collected a total of 30 cases in the literature, and analyzed their characteristics from the aspects of gender, suspicious medication history, and treatment in order to expand clinicians' approach to diagnosis and treatment.

Epidermolysis bullosa (EB) encompasses a heterogeneous group of inherited skin fragility disorders, with mutations in genes encoding the basement membrane zone (BMZ) proteins that normally ensure dermal-epidermal integrity. Of the four main EB types, recessive dystrophic EB (RDEB), especially the severe variant, represents one of the most debilitating clinical entities, with recurrent mucocutaneous blistering and ulceration leading to chronic wounds, infections, inflammation, scarring and ultimately cutaneous squamous cell carcinoma, which leads to premature death. Improved understanding of the molecular genetics of EB over the past three decades and advances in biotechnology have led to rapid progress in developing gene and cell-based regenerative therapies for EB. In particular, RDEB is at the vanguard of advances in human clinical trials of advanced therapeutics. Furthermore, the past decade has witnessed the emergence of a real collective, global effort involving academia and industry, supported by international EB patient organizations such as the Dystrophic Epidermolysis Bullosa Research Association (DEBRA), among others, to develop clinically relevant and marketable targeted therapeutics for EB. Thus, there is an increasing need for the practising dermatologist to become familiar with the concept of gene therapy, fundamental differences between various approaches, and their human applications. This review explains the principles of different approaches of gene therapy, summarizes its journey, and discusses its current and future impact in RDEB.

A significant barrier to the usage of isotretinoin has been concerns regarding its adverse effect profile. The dose-dependent mucocutaneous side effects of isotretinoin are well recognized and easily managed, particularly if a lower dose is used. A possible association with depression has gained widespread media attention and is a source of concern for many patients and their carers, but data from prospective studies and recent meta-analyses have been reassuring. Furthermore, there has been much confusion amongst both patients and physicians regarding a possible association with inflammatory bowel disease, as well the ocular and rheumatological adverse effects of isotretinoin. We provide an update on the evidence surrounding the adverse effects of isotretinoin and discuss practical strategies to prevent and manage these adverse effects. We also discuss appropriate laboratory monitoring for patients taking isotretinoin.

Abstract: Leishmaniasis is a parasitic infectious neglected tropical disease transmitted to humans by the parasites of Leishmania species. Mainly, three types of leishmaniases are usually observed: visceral (VL), cutaneous (CL), and mucocutaneous leishmaniasis. In many western countries, almost 700,000 to 1 million people suffer from leishmaniasis, and it is estimated that around 26000 to 65000 deaths occur from leishmaniasis. Few drugs are available for its treatment; however, none of them are ideal for leishmaniasis due to long treatment, discomfort mode of administration, risk of high-level toxicity, high resistance, etc. Hence, so many patients are unable to take complete treatment due to the high drug resistance. The present review will focus on antileishmanial activity of reported derivatives of betacarboline, chalcone, azole, quinoline, quinazoline, benzimidazole, benzadiazapine, thiaazoles, semicarbazone, and hydontoin analogues. We believe that this present study will be helpful for researchers to design new antileishmanial agents.

Behçet disease and its related disorder, Sweet disease, are multifactorial disorders whose susceptibility loci have been identified in the genes of various immunological factors aside from human leukocyte antigens. The neurological involvement of these diseases, including encephalitis, myelitis, and meningitis, referred to as neuro-Behçet disease (NBD) and neuro-Sweet disease (NSD) respectively, is sometimes difficult to diagnose, especially when the characteristic mucocutaneous symptoms do not precede neurological symptoms or when characteristics of both diseases are present in a single patient. NBD and NSD constitute a spectrum of diseases that are differentiated according to the combination of risk factors, including the genetic background. Encephalitis, myelitis, and meningitis similar to NBD or NSD can be diagnosed as spectrum disorders, even if the characteristic mucocutaneous symptoms fail to be detected. Understanding these conditions as a disease spectrum may help elucidate the disease pathogenesis and assist in the development of therapeutic agents.

HIV-associated Kaposi's sarcoma is an angioproliferative neoplasia caused by infection with human herpesvirus 8. It typically presents with mucocutaneous involvement. Pulmonary Kaposi's sarcoma is rare, and an uncommon form of initial presentation of the disease. The authors present the clinical case of an HIV-positive patient with Kaposi's sarcoma who had exclusively pulmonary involvement at diagnosis. This case is highlighted for its rarity, by the diagnostic challenge it presented, and for the important role of bronchoscopy as a diagnostic tool for this pathology. Bronchoscopy allows direct visualization of the lesions and the ability to perform a bronchoalveolar lavage and directed biopsies.

Lymphoproliferative disorders (LPDs) are a group of conditions characterized by excessive production of lymphocytes manifested in various patterns including lymphadenopathy, tumor-like lesions, and lymphomas. LPD may be stimulated by Epstein-Barr virus (EBV) infection that most commonly appears in the setting of immunocompromised status such as long-term use of immunosuppressive medications and in individuals with primary immunodeficiency disorders. EBV mucocutaneous ulcer is a benign LPD reaction that mostly regresses spontaneously but sometimes requires medical or surgical intervention. This article presents a case of oral EBV mucocutaneous ulceration that affected an individual with a history of complex primary immunodeficiency disorders consisted of common variable immunodeficiency disease associated with T-cell dysfunction. This case is unique because the oral lesions led to the diagnosis of concurrent widespread EBV-negative follicular lymphomas, seemingly unrelated to her EBV-positive LPD oral disease. Yet, both occurred in the setting of severe immunosuppression from the primary immunodeficiency disorders.

The present report describes a case of Epstein-Barr virus (EBV)-positive mucocutaneous ulcer (EBVMCU) with an ulcerative lesion on the right tongue. A 69-year-old Japanese woman with awareness of pain in the right lingual margin visited our hospital. The patient had rheumatoid arthritis and had been treated with methotrexate (MTX) for 9 years. Vitiligo was found throughout the oral cavity, and ulceration was found on the right tongue. Under a diagnosis of oral candidiasis and suspected right tongue tumor, treatment for candidiasis and biopsy of the right tongue ulcer were performed. Histopathological results were positive for CD20 and CD30 and negative for CD3 and CD5. EBV-encoded small RNA in situ hybridization confirmed positive results in large, atypical cells, suggesting involvement of EBV. EBVMCU was diagnosed based on the history of MTX use associated with rheumatoid arthritis, blood biochemical findings, and histopathological findings. The ulcer on the right tongue margin resolved spontaneously after treatment for oral candidiasis prior to discontinuation of MTX. No recurrence was seen for 18 months.

Mycoplasma pneumoniae is a well-known cause of community-acquired pneumonia, mostly associated with dermatological manifestations especially with mucosal involvement and targetoid cutaneous lesions. For many years, it was considered among the spectrum of erythema multiforme. Recently, some authors have recommended the creation of a new syndrome called “mycoplasma-induced rash and mucositis.” This new syndrome has distinct epidemiological, clinical and histological features making it different from drug-induced Stevens-Johnson syndrome, toxic epidermal necrosis and erythema multiforme. Herein, we report two patients with acute Mycoplasma pneumoniae respiratory tract infection presenting severe mucocutaneous lesions in accordance with this new syndrome.

Epstein–Barr virus-positive mucocutaneous ulcer (EBVMCU) is a new disease, described by the World Health Organization in 2017. It has been recognized as a specific type of immunodeficiency-associated lymphoproliferative disorder. Since patients with EBVMCU present with only cutaneous or mucosal ulcers, it is difficult to clinically distinguish them from carcinoma. A 72-year-old man, who took methotrexate (MTX) (12 mg/week) for rheumatoid arthritis, was referred to our hospital because endoscopy revealed an ulcerated mass in the left pyriform sinus, suggesting hypopharyngeal squamous cell carcinoma. Contrast-enhanced computed tomography and magnetic resonance imaging revealed an ill-defined mass in the left pyriform sinus without lymphadenopathy in the head and neck region. A biopsy of the ulcerative lesion in the hypopharynx was performed, and lymphoproliferative disease was suspected, based on the histopathological findings. Two weeks after MTX withdrawal, the lesions in the hypopharynx disappeared. The patient was diagnosed with EBVMCU, based on the clinical and histopathological findings. This is the first case report of EBVMCU of the hypopharynx. EBVMCU should be considered as a differential diagnosis in immunocompromised patients with hypopharyngeal mucosal ulcers without lymph node or organ involvement.

To know the clinical presentation and outcome of children with pediatric inflammatory multisystem syndrome temporally associated with SARS-CoV- 2 (PIMS-TS) at a pediatric tertiary care center in Chennai. Clinical and biochemical parameters of 65 children with PIMS-TS treated between July and October 2020 were studied. All children had their COVID RT-PCR and IgG COVID antibodies tests done. Mean age of the study group was 5.65 ± 3.68 y. Fever with red eyes, rash, vomiting, abdominal pain, and shock were common presenting features. Sixty percent of the study group had Kawasaki/incomplete Kawasaki features. Sixty-seven percent of the study group had coronary dilatation, 41% presented with shock, and 25% had left ventricular dysfunction. Coronary aneurysms were documented in 58% of the study group (z score more than 2.5). Respiratory presentation with pneumonia was seen in 10%. Four children presented with acute abdomen. Acute kidney injury, acute liver failure, hemolysis, pancytopenia, macrophage activation syndrome, encephalopathy, and multiorgan dysfunction syndrome (MODS) were other features. Forty-three percent required noninvasive oxygen support and 15.4% required mechanical ventilation. Intravenous immunoglobulin (73.8%) and methylprednisolone (49.8%) were used for therapy. Mortality in the study was 6%, which was due to MODS. Acute febrile illness with mucocutaneous and gastrointestinal manifestations should have PIMS-TS as a possible differential diagnosis and needs evaluation with inflammatory markers and SARS-CoV-2 antibodies.

Objectives This study aimed to determine the clinical efficacy of apremilast for oral ulcers (OUs), extra-oral manifestations, and overall disease activity in patients with Behçet’s disease (BD). Methods A systematic literature search was performed in PubMed, Embase, Cochrane Library, and Web of Science Core Collection. Studies assessing the treatment effects of apremilast in BD were included. The odds ratios (ORs) of being symptom-free for individual manifestations and mean difference (MD) of Behçet’s Disease Current Activity Form (BDCAF) scores were calculated with 95% confidence intervals (CIs) at 12 and 24 weeks using a random-model meta-analysis. Results Of 259 screened articles, eight were included. After 12 weeks of apremilast treatment the OR of symptom-free was as followings: OUs, 45.76 (95% CI, 13.23–158.31); genital ulcers, 4.56 (95% CI, 2.47–8.44); erythema nodosum, 3.59 (95% CI, 1.11–11.61); pseudofolliculitis, 2.81 (95% CI, 1.29–6.15); and arthritis, 3.55 (95% CI, 1.71–7.40). Furthermore, BDCAF scores at 12 weeks were significantly reduced (MD=−1.38; −1.78 to −0.99). However, the proportion of oral-ulcer-free patients increased at 24 weeks (OR = 14.88; 4.81 to 46.07). Conclusions The currently accumulated data indicate an improvement in mucocutaneous and articular symptoms by short-term apremilast treatment in patients with BD.

Hereditary haemorrhagic telangiectasia is a rare, genetic disorder that can present at any age. It is characterised by epistaxis, mucocutaneous telangiectasia and visceral arteriovenous malformations, which can affect multiple organs. Early diagnosis and management reduces the morbidity and mortality associated with the disease.There is a well-established hereditary haemorrhagic telangiectasia clinic in London, and excellent links across Europe via the European Reference Network. However, local coordinated care for patients with hereditary haemorrhagic telangiectasia across the UK can be variable and often absent for children and young people. Some patients travel long distances to receive care in London, while others are referred to local clinicians or lost to follow up entirely.This article presents the experience to date from two regional UK centres (Liverpool and Dundee) where care for patients with hereditary haemorrhagic telangiectasia is being coordinated and streamlined. While there is still a lot to learn, this article highlights some of the successes and challenges identified so far, with suggestions for how these could be addressed.Collaborative regional networks such as these can facilitate the sharing of best practice and ensure that all patients with hereditary haemorrhagic telangiectasia are able to access safe, high-quality care.

Background: Mucocutaneous adverse events are common during anticancer treatment, with variable consequences for the patient and their therapeutic regimen. Objective: To evaluate the most common adverse events, as well as the drugs associated with their appearance and the consequences for cancer treatment. Methods: A retrospective study was carried out through the analysis of patients treated at the Clinical Dermatology Unit of a public oncologic hospital. Results: A total of 138 patients with 200 adverse events were evaluated. The most commonly identified adverse events were nail and periungual changes (20%), papulopustular eruptions (13%), acneiform eruptions (12%), hand-foot syndrome (6.5%), hand-foot skin reaction (6%), and xerosis (6%). The most frequently associated antineoplastic treatment groups were classical chemotherapy (46.2%), target therapy (32.3%), and other non-antineoplastic drugs used in neoplasia protocols (16.5%). Of the total number of patients, 17.4% had their treatment suspended or changed due to a dermatological adverse event. Study limitations: Retrospective study and analysis of patients who were referred for specialized dermatological examination only, not allowing the assessment of the actual incidence of adverse events. Conclusion: A wide variety of dermatological manifestations are secondary to antineoplastic treatment with several different drugs resulting, not rarely, in the interruption or modification of therapeutic regimens.

Introduction Behcet's disease (BD) is a systemic inflammatory disease with various presentations. The data on the course of BD in Egyptian patients are limited. Objectives The objective of the study was to describe the evolution and association of the different phenotypes of BD. Material and methods This chronological cohort study included adult Egyptian patients suffering from BD. Demographic data and the chronological order of the disease's manifestations were collected. Results The study included 233 patients. Their mean age at the onset of the disease was 26.3 ± 6.9 years. The mean duration from onset of the disease to meeting the criteria was 11.2 ± 30.3 months. The mean duration of the disease was 96.8 ± 72.2 months. On onset of the disease, the most common phenotypes were mucocutaneous (84.5%), musculoskeletal (15.9%), ocular (14.6%) and peripheral venous disease (PVD) (7.3%); on the other hand, pulmonary, peripheral arterial and great vessel phenotypes evolved several years after onset of the disease. The mean time from meeting the criteria to the evolution of a new phenotype was 53.8 ± 58.7 months. Associations between the different phenotypes were observed: PVD and superficial thrombophlebitis, peripheral arterial disease and PVD; another association was also observed between aortic involvement and cerebrovascular disease. Conclusion BD could continue to evolve several years after onset of the disease, making the previous belief about BD yield questionable. BD tends to respect the anatomy of the affected system. Some phenotypes tend to coexist, suggesting a shared aethiopathogeny and that the disease is of a systemic nature.

Kaposi sarcoma (KS) is one of the most common neoplasms diagnosed in HIV- seropositive subjects. KS is an angioproliferative disorder of endothelial origin and is a locally aggressive multicentric mucocutaneous malignant neoplasm. Aggressive HIV-KS is associated with increased HHV8 viral load, disseminated lesions, and intraoral exophytic lesions, and may also present with facial lymphoedema which may be life-threatening or mild. A 26 -year-old, male, presented with severe oral pain and facial swelling. He had multiple oral lesions and an incisional biopsy confirmed Kaposi sarcoma. A multiple site incisional biopsy and immunohistochemistry for HHV8 was done and based on the clinical, histopathological, and immunohistochemical results a diagnosis of HIV associated oral and cutaneous KS with severe lymphoedema was confirmed. This aim of this case presentation is to describe a case of immunodeficiency virus-related lymphadenitis, oral and cutaneous KS associated with the complication severe lymphoedema in a HIV infected patient who was not on HAART therapy for three years after being diagnosed with HIV.

The place of pemphigus vulgaris (PV) among autoimmune bullous diseases (AIBD) is well established. It is an acquired chronic, autoimmune, vesiculobullous disease in which IgG antibodies target desmosomal proteins to produce intraepithelial mucocutaneous blistering. The diagnosis is often challenging for the clinicians. It requires a combination of three major features: clinical, histopathological, and immunological. Clinically, oral lesions are the first manifestations of the disease in 50-90% of the patients with widespread blisters affecting the oral mucosa. On the skin, lesions are characterized by flaccid blisters that rapidly progress into erosions and crust formation. Umbilical lesions as a clinical manifestation of PV are peculiar and have rarely been reported, and they are not yet completely elucidated. Umbilical region involvement in patients with pemphigus was assessed in a limited study totalling just 10 patients. This localisation may be a valuable hint easing the diagnosis at the clinical level for patients with oral mucosal blisters. Dentists must be familiar with the clinical manifestations of PV to make an early diagnosis and start an early treatment which determines the prognosis of the disease. To the best of the authors’ knowledge, the coexistence of these lesions with the oral lesions as a first sign of PV in the absence of skin involvement was reported in only one case of pemphigus vegetans (PVe). In this paper, we describe an observation of a female patient that was diagnosed with PV that begun with simultaneous oral and umbilical locations which coexisted for a period of 4 months before the appearance of other cutaneous lesions. We highlight the role of dentists, by being familiar with the clinical manifestations of PV, to make an early diagnosis to start an early treatment which determines the prognosis of the disease and to follow closely the evolution of lesions to change treatment if required. We also discuss the clinical, histological, and immunological features of the disease that enabled the differential diagnosis as well as the appropriate therapeutic management.

Objectives To report an atypical presentation of a pathogenic STK11 gene variant in siblings not fulfilling the clinical diagnostic criteria for Peutz-Jeghers Syndrome (PJS). Case presentation Two siblings presented with prepubertal gynaecomastia and bilateral macro-orchidism, without mucocutaneous pigmentation or gastrointestinal symptoms. There was no family history of PJS. Sibling 1 had unilateral gynaecomastia. Sibling 2 had bilateral gynaecomastia, advanced bone age and bilateral testicular microlithiasis, not indicative of a large-cell calcifying Sertoli cell tumour. Genetics revealed a paternally inherited heterozygous pathogenic STK11 variant (910C>T) in both siblings. The diagnosis was confirmed following the identification of multiple intestinal polyps in their father. Conclusions Prepubertal gynaecomastia and prepubertal macro-orchidism (testicular enlargement without virilisation), always warrant endocrinological investigation, with PJS being an important differential diagnosis. Children may not fulfil the clinical criteria for a diagnosis of PJS at presentation. Genetic testing and gastroenterological investigation of parents may aid diagnosis.

Objective Randomized controlled trials (RCTs) in SLE (lupus) typically adopt composite responder definitions as primary efficacy endpoints; however, outcomes within individual organ domains are also important to understand. The aim of this scoping review was to evaluate how organ-specific disease activity and therapeutic responses have been measured and reported in lupus RCTs. Methods We searched MEDLINE, EMBASE, Cochrane registry and clinicaltrials.gov. Eligible studies were RCTs investigating efficacy of an immune-directed drug therapy in active SLE, published January 2000–March 2021, excluding studies limited to lupus nephritis. Data were extracted independently in duplicate into a template and summarized descriptively. Results Thirty-four RCTs were included, of which 32 (94%) reported activity and/or responses in at least one organ domain. Study populations had a high, although variable, frequency of baseline musculoskeletal and mucocutaneous activity and low, but also variable, representation of other domains. Definitions of organ-specific responses were inconsistent, even within individual instruments. Response in most organ domains were evaluated using BILAG and SLEDAI components but meaningful comparison between treatment arms was limited by small subgroups analysed in a post hoc fashion. Specific mucocutaneous and arthritis instruments were also used, including within pre-specified organ-specific endpoints, which discriminated between treatment arms in some studies. Conclusion Mucocutaneous and musculoskeletal manifestations predominate in SLE RCTs. Organ-specific outcome measures are commonly reported, but definitions of involvement and response are inconsistent. Research into the development of new outcome measures for key organ domains, and validation and comparison of response definitions using existing instruments, is needed.

Introduction Autoimmune gastritis (AIG) is associated with nutritional deficiencies, autoimmune diseases, and gastric malignancies. The aims of the study were to test the hypothesis that mucocutaneous (MC) manifestations occur more often in patients with vs without AIG and to delineate patterns of MC manifestations in AIG. Methods A single-center, prospective 2:1 case-control study was conducted. Cases were patients with the diagnosis of AIG based on consistent serologic and histologic findings. Controls had a normal gastric biopsy. MC manifestations were independently evaluated by 3 experienced dermatologists. We conducted a multivariable logistic regression model adjusted for age, sex, Helicobacter pylori, tobacco use, and alcohol consumption to estimate the association between AIG (vs no AIG) and MC manifestations (adjusted odds ratio; 95% confidence interval). Results We prospectively enrolled 60 cases and 30 controls (mean age 53.5 ± 15.8 vs 53.4 ± 14.5 years; 75% vs 73.3% women). The pooled prevalence of MC immune-mediated diseases was higher in patients with vs without AIG (66.7% vs 23.3%; adjusted odds ratio 12.01 [95% confidence interval: 3.51-41.13]). In patients with AIG, seropositive vs seronegative anti-intrinsic factor antibodies more often had concomitant immunological diseases with MC manifestations (100% vs 58.5%; P = 0.016). The most common MC immune-mediated diseases in AIG were Sjogren syndrome (n = 5, 8.3%), alopecia areata (n = 5, 8.3%), and vitiligo (n = 4, 6.7%). Nutritional deficiency-related MC findings, mainly xerosis, lingual, and nail disorders, were also more common in AIG. Discussion This is the first comparative study specifically designed to evaluate MC manifestations in AIG. We demonstrated that AIG is more frequently associated with both immune- and nutritional deficiency-related MC manifestations, which might have both diagnostic and therapeutic clinical implications.

Background: Multisystem inflammatory syndrome in children (MIS-C) is a new hyperinflammatory variant that evolved during the coronavirus disease 2019 (COVID-19) pandemic. Although the precise pathophysiology of MIS-C is uncertain, it is thought to be due to immune dysregulation occurring after recovery from acute infection. Purpose: Our study aimed to analyze the clinical spectrum, laboratory parameters, imaging characteristics, treatment strategies, and short-term outcomes of children with a diagnosis of MIS-C. Methods: This retrospective and prospective observational study included children less than 16 years of age who were admitted to the pediatric unit of a tertiary care teaching hospital in south India between August 2020 to January 2021 with a diagnosis of MIS-C according to World Health Organization (WHO) criteria. Results: Twenty-one children were included in the analysis; all had fever with variable combinations of other symptoms. The mean age was 6.9 years; 71.4% were male. Gastrointestinal (80.9%) and cardiovascular (80.9%) systems were the most commonly affected. The majority of children had elevated inflammatory markers, and 16 (76.2%) had echocardiographic abnormalities mimicking Kawasaki disease. Eleven (52.4%) children required intensive care admission, three (14.3%) required supplemental oxygen, and four (19%) required inotropes. Nine (42.9%)were treated with intravenous immunoglobulin alone, six (28.6%) with steroids alone, and three(14.3%) with steroids and immunoglobulin. The median hospital stay was 6 days; there were no fatalities. Overweight/obesity, elevated ferritin, and mucocutaneous involvement were significantly associated with a prolonged hospital stay (≥7 days). Sixteen (76.2%) children were followed up till now and all of them had no clinical concerns. Conclusion: MIS-C is an emerging disease with variable presentation. A high index of suspicion is necessary for its early identification and appropriate management. Further research is essential for developing optimal treatment strategies.

Erythema multiforme (EM) is an acute mucocutaneous syndrome mediated by hypersensitivity reactions. Symptoms are often mild and self-limiting but have a high recurrency rate. The disease is more prevalent in adolescents and adults, with incidence in childhood often precipitated by infection of herpes simplex virus type 1. However, EM induced by infection of Mycoplasma pneumonia is a rare occurrence. This paper reports a case of pneumonia induced EM in a 5-year-old child with a typical clinical feature supported by laboratory and chest X-ray features that were confirmed with histopathology. The patient showed significant clinical improvements after antibiotic therapy.

OBJECTIVE We aimed to investigate the impact of applying the 2019 European Alliance of Associations for Rheumatology (EULAR)/American College of Rheumatology (ACR) classification criteria for systemic lupus erythematosus (SLE) in a previously described cohort of women with undifferentiated connective tissue disease (UCTD). METHODS This study included 133 women with UCTD. At the time of inclusion into the study, none of the patients met any classification criteria for other defined systemic connective tissue disease. RESULTS When applying the 2019 EULAR/ACR classification criteria to the cohort, 22 patients (17%) fulfilled the classification criteria for SLE. Patients classified as having SLE had significantly higher frequencies of mucocutaneous manifestations (23% versus 5%; P = 0.007), arthritis (59% versus 17%; P < 0.001), isolated urine abnormalities (18% versus 1%; P < 0.001), and highly specific antibodies (50% versus 15%; P < 0.001) compared to the other patients with UCTD. At follow-up, these patients were statistically significantly more likely to also meet the 1997 ACR revised SLE criteria and the Systemic Lupus International Collaborating Clinics (SLICC) criteria (18.2% versus 1.8%; P < 0.001) compared to the other UCTD patients. Patients who were diagnosed as having SLE according to the ACR 1997 update of the SLE revised criteria and the SLICC criteria during the follow-up scored higher on outcome measures when classified as having SLE according to the new 2019 EULAR/ACR classification criteria when compared to the other patients with UCTD (mean ± SD score 8.3 ± 3.7 versus 4.5 ± 4; P < 0.05). CONCLUSION When applying the 2019 EULAR/ACR criteria for SLE in a cohort of patients with UCTD, we observed that in up to 17% of cases the original classification could be challenged. New implementation will help to identify earlier patients at higher risk of developing more severe CTD manifestations.

Objectives: The aim of this study was to add to the descriptive data pertaining to the epidemiology, presentation, and clinical course of multisystem inflammatory syndrome (MIS) temporally associated with coronavirus disease 2019 in adults and adolescents from low- and middle-income countries. Methods: Patients presenting to the adult wards (14 years and older) of three academic hospitals in South Africa, who were diagnosed with MIS between August 1, 2020 and May 31, 2021, were reviewed retrospectively. The presentation, laboratory and radiographic findings, and clinical course are described. Results: Eleven cases of MIS were reported, four in adolescents (14–19 years) and seven in adults (≥19 years). Fever was universal. Gastrointestinal symptoms (90.9%), cardiorespiratory abnormalities (90.9%), and mucocutaneous findings (72.7%) were prominent. Echocardiography in 10/11 patients (90.9%) showed a median left ventricular ejection fraction of 26.3% (interquartile range 21.9–33.6%). All patients required high care admission and 72.7% required inotropic support. Glucocorticoids were initiated in all cases and 72.7% received intravenous immunoglobulin. Conclusions: This constitutes the largest multicentre review of adults and adolescents with MIS in Africa. MIS may be overlooked in resource-limited settings, and heightened suspicion is needed in patients with multi-organ dysfunction, especially where repeated investigations for other aetiologies are negative.

Behçet's disease (BD) significantly increases morbidity and mortality, especially in young men. While vascular involvement is the most frequent cause of mortality, ocular involvement, which can cause visual loss, is the most important cause of morbidity in BD. Immunosuppressive treatment is the mainstay for major organ involvement. However, despite optimal immunosuppressive treatment, relapses and disease-related damage develop in a subgroup of patients, especially among those with ocular or vascular involvement. With the recent understanding of the immuno-pathogenesis, biologic treatments targeting potential pathogenic cells, cytokines or pathways are better optimized in BD. Data from large series showed that tumor necrosis factor-α inhibitors and interferon-α are effective and safe treatment options for the treatment of refractory and major organ involvement, such as ocular, neurologic, vascular, and gastrointestinal. Anakinra and ustekinumab also seem to be promising agents for refractory mucocutaneous disease. IL-1 inhibitors and tocilizumab may be alternatives for the treatment of patients with refractory eye involvement. Still, randomized controlled trials of biologic agents, especially for the treatment of major organ involvement, are insufficient, and further prospective, long-term follow-up studies are needed to clarify the efficacy, safety, and optimal treatment duration of biologic agents in BD.

Autosomal recessive mutations in Inducible T Cell Costimulator Ligand (ICOSLG) result in a combined immunodeficiency syndrome of humans, saliently marked by recurrent respiratory tract infections and significant disease with DNA-based viruses at epithelial barriers, including human papillomavirus (HPV). These features are also seen in persons with loss of function of the complementary gene, ICOS. The infection phenotypes associated with these natural experiments disclose a critical role of the corresponding proteins, ICOSL and ICOS, in human immunity at mucocutaneous barriers. Here, we review the syndromes of ICOSL and ICOS deficiency and explore the mechanisms by which the ICOSL:ICOS axis mediates epithelial host defenses.

Introduction: The aim of this study was to assess the differences between childhood-onset and adult-onset systemic lupus erythematosus (cSLE and aSLE) for clinical manifestations and mortality using a meta-analytic approach. Methods: The PubMed, EMBASE, and the Cochrane library were searched for eligible studies published between January 1982 and March 2021. The odds ratio (OR) with 95% confidence interval was used to calculate the pooled effect estimates using the random-effects model. Results: Thirty-four studies involving 21,946 SLE patients were included. cSLE was associated with an increased risk of malar rash (OR: 1.64; p < 0.001), ulcers/mucocutaneous involvement (OR: 1.22; p = 0.039), general neurological involvement (OR: 1.52; p < 0.001), seizures (OR: 1.92; p < 0.001), general renal involvement (OR: 2.08; p < 0.001), proteinuria (OR: 1.35; p = 0.015), urinary cellular casts (OR: 1.67; p = 0.047), fever (OR: 2.31; p < 0.001), anemia (OR: 1.91; p < 0.001), thrombocytopenia (OR: 1.41; p < 0.001), leucopenia (OR: 1.57; p = 0.017), lymphadenopathy (OR: 2.40; p < 0.001), and cutaneous vasculitis (OR: 1.72; p = 0.001) as compared with aSLE. Moreover, cSLE versus aSLE was associated with a reduced risk of articular manifestations (OR: 0.63; p = 0.001), pulmonary involvement (OR: 0.54; p = 0.001), and pleuritis (OR: 0.61; p < 0.001). There were no significant differences between cSLE and aSLE for mortality risk (OR: 1.20; p = 0.203). Conclusion: We found that certain clinical manifestations of SLE are different in cSLE and aSLE. Moreover, the mortality risk of cSLE and aSLE was not significantly different.

The data on prevalence of extraintestinal manifestations (EIM) in ulcerative colitis (UC) are scanty and highly variable. Consecutive patients with UC were prospectively evaluated from November 2016 to August 2017. A detailed history was obtained and physical examination was done. Presence of EIM was confirmed by a consultant rheumatologist, ophthalmologist and dermatologist. Tests performed were hemogram, liver function test, abdominal ultrasound, slit lamp examination, X-ray and magnetic resonance imaging when deemed necessary. A total of 227 patients with UC were enrolled in this study. The prevalence of EIM was 7.92%. Mucocutaneous (4.84%) manifestations were the commonest, followed by musculoskeletal (1.32%) and ocular (0.88%). Hepatobiliary (0.44%) and vascular (0.44) manifestations were present in equal frequency. History of appendicectomy was associated with EIM. The prevalence of EIM in UC was low in our study. History of appendicectomy was a risk factor for EIM.

Introduction: Systemic Lupus Erythematosus (SLE) is a disease of thousand faces whose manifestations vary with the interplay of genetic, ethnic, and host factors along with environmental exposures and geographical conditions. Since the first case of SLE reported in India in 1955, many case series have been published from various centers depicting Clinical and Immunological features in the Indian population. This study was undertaken to study the clinical and immunological profile in a tertiary care center in the sub-Himalayan region of northern India. Materials and Methods: This study is a cross-sectional retrospective study conducted at a tertiary care center in the hilly northern state of Himachal Pradesh over a period of 1 year; from 1st June 2017 to 31st May 2018. The records of all patients admitted in the study period with the department of medicine and diagnosed with SLICC 2012 classification criteria were studied to abstract the Clinical and Immunological profile of patients. Results: A total of sixty-nine patients were included in the study. 96% of the subjects were female patients, and 84 % of the patients were in the reproductive age group of 20 – 40 years. Mucocutaneous manifestations were most commonly seen in nearly 89 % of patients followed by musculoskeletal involvement in about 71 %. Hematological involvement was more frequent than reported in the literature. Lupus Nephritis was diagnosed in about 30 % of the patients. Fatigue and synovitis were the most common features, followed by oral ulcers and Acute cutaneous lupus. ANA and ds DNA positivity was 98.5 and 72.4 % respectively. Six cases of MCTD were d=found in the study group. Conclusion: In hospitalized SLE patients admitted in a tertiary care center of the northern hilly state, females of reproductive age group constituted the majority of patients. Mucocutaneous, Musculoskeletal, hematological, and renal involvement was most prevalent. Keywords: SLE, cross-sectional study, Mucocutaneous involvement, Renal Biopsy, Hilly area.

Epstein-Barr virus (EBV) -positive mucocutaneous ulcer (EBVMCU) was first described as a lymphoproliferative disorder in 2010. In recent years, EBVMCU has been reported in the field of oral surgery. On the other hand, medication-related osteonecrosis of the jaw (MRONJ) is an osteomyelitis that occurs in patients receiving antiresorptive agents including bisphosphonates (BP) and/or denosumab developing with bacterial infections such as dental diseases and mucositis. MRONJ caused by EBVMCU in the elderly has not been reported. Here, we report a rare case of MRONJ caused by EBVMCU in the elderly. The patient, an 82-year-old woman, had received BP for more than 2 years. An ulcerative lesion was found in the palatal mucosa; biopsy performed from the site confirmed the diagnosis of EBVMCU. At follow-up, the lesion disappeared spontaneously. At the 6-month follow-up, bone formation was observed at the site of the lesion, and the sequestrum was removed. At the 12-month follow-up healing of the EBVMCU region was seen indicating a good prognosis.

Peutz-Jeghers syndrome (PJS) is an autosomal dominant familial hamartomatous polyposis syndrome characterized by multiple gastrointestinal hamartomatous polyps and mucocutaneous melanin pigmentation. We report two cases, with no family history, presenting as emergency, one with perforation peritonitis due to spontaneous bowel disruption and another with bowel gangrene due to severe bowel congestion secondary to intussusception. Histological examination showed features of hamartomatous polyposis; other clinical features in these patients were suggestive of the diagnosis of Peutz-Jeghers syndrome. Given the multitude of carcinoma that these patients are susceptible to, aggressive screening protocols are recommended. Close monitoring and follow-up are essential in PJS patients for the prevention of potential complications, early detection of related malignancies due their increased risk for both intestinal and extraintestinal malignancies.

STK11 encodes for the protein liver kinase B1, a serine/threonine kinase which is involved in a number of physiological processes including regulation of cellular metabolism, cell polarity and the DNA damage response. It acts as a tumour suppressor via multiple mechanisms, most classically through AMP-activated protein kinase-mediated inhibition of the mammalian target of rapamycin signalling pathway. Germline loss-of-function mutations in STK11 give rise to Peutz-Jeghers syndrome, which is associated with hamartomatous polyps of the gastrointestinal tract, mucocutaneous pigmentation and a substantially increased lifetime risk of many cancers. In the sporadic setting, STK11 mutations are commonly seen in a subset of adenocarcinomas of the lung in addition to a number of other tumours occurring at various sites. Mutations in STK11 have been associated with worse prognoses across a range of malignancies and may be a predictor of poor response to immunotherapy in a subset of lung cancers, though further studies are needed before the presence of STK11 mutations can be implemented as a routine clinical biomarker.

Lichen planus is a benign, chronic, mucocutaneous disorder that affects the skin, mucosae, and nails. The occurrence of nail lichen planus has been reported to be about 10% to 15%.1,2 Nail lichen planus is more common in adults than in children, and it mostly affects the fingernails rather than the toenails.3 The many nail abnormalities found in nail lichen planus depend on its pathologic location: the nail matrix or the nail bed. Dorsal pterygium is an irreversible and classic finding in nail lichen planus. The disease may lead to permanent disfigurement, which has both functional and psychological consequences. Early diagnosis and prompt treatment are also important to prevent the development of pterygium. Nail lichen planus can sometimes be difficult to diagnose and differentiate from other nail disorders. This is especially the case in patients without other mucocutaneous findings. Systemic corticosteroids are the first-line treatment, but they have inconsistent outcomes. There are few published reports on the clinical characteristics and treatment outcomes of nail lichen planus in Asians. The aims of our study were to review the clinical characteristics of, treatment approaches to, and treatment outcomes of nail lichen planus.

Background Candida species is considered one of the normal inhabitant commensal microbiota of the human body. However, it can also act as an opportunistic pathogen especially in hospitals (nosocomial infection) and among immunocompromised patients. The accurate, precise, rapid and reliable identification of Candida to the species level is of great importance for control and management of candidiasis. Results One hundred and eighteen different samples were collected (59 urine samples, 39 oral swabs, 5 vaginal swabs and 15 skin swabs) from immunocompromised patients (diabetics—pregnant women—patients underwent organ transplantation—cancer patients—burned and wounded patients) for probable existence of Candida species. Eighty-six out of 118 (72.8%) samples were typed macroscopically and microscopically and found to be Candida species. Upon streaking 86 Candida isolates on CHROMagar plates separately, 48 isolates gave green colonies, 25 isolates gave rose colonies, 10 isolates gave white colonies, 2 isolates gave pale coloured colonies and 1 isolate gave blue colonies. Forty-eight out of 86 isolates showed positive Germ tube test. API 20C assay was performed on some isolates with different coloured colonies, the results were similar to those of CHROMagar. Upon performing PCR assay on 14 isolates using ITS1 and ITS4 primers, 8 out of 14 PCR product bands appeared between 510 and 535 bp and this was difficult to differentiate among them (C. albicans, C. tropicalis, C. krusei and C. parapsilosis). Five out of 14 PCR products were found at 871 bp (C. glabrata) and the last one is negative control with no band appeared. Further molecular studies should be recommended to fully differentiate among Candida species especially those with very close bands. Antifungal activity (expressed by inhibitory zone) of zinc oxide nanoparticles in comparison with some commercially available antifungals (nystatin and Voriconazole) was carried upon the obtained Candida isolates. Zinc oxide nanoparticles with 100 μg/disc showed 9, 26, 40, 42 mm inhibitory zones for C. tropicalis, C. glabrata, C. albicans and C. parapsilosis, respectively. However, zinc oxide only showed no antifungal activity against C. krusei. Conclusions A sheet of identification profile for Candida should include morphotyping, biotyping and genotyping to reach a rapid, reliable and accurate diagnosis. Candida species causes a myriad of infections causing non-invasive, mucocutaneous infections and severe systemic and deep-seated disease. Repress of Candida growth by ZnONPs provides an insight towards their therapeutic application for the prevention of Candida-associated infections. Further studies on the antifungal effect of nanoparticles combined with commercially available antifungal medicines maybe recommended.

Toripalimab and pembrolizumab belong to anti-programmed death receptor-1 monoclonal antibodies for the treatment of various cancers. Anti-programmed death receptor-1 therapy can cause mucocutaneous adverse events. Here, we report the first case, to our knowledge, of oral mucous membrane pemphigoid and lichenoid reaction triggered by toripalimab and aggravated by switching to pembrolizumab. Mucous membrane pemphigoid was a definite diagnosis, whereas lichenoid reaction was a clinical diagnosis without pathologic evidence. Although discontinuation of the culprit drugs achieved clinical resolution in most reported cases, multiple studies demonstrated statistically significant associations between the development of dermatologic adverse events and superior clinical outcomes. Thus, more studies are needed to find satisfactory measures in terms of both cancer control and avoidance of severe adverse events.

Reactive infectious mucocutaneous eruption (RIME) was proposed as new terminology to encompass postinfectious mucocutaneous eruptions. The term includes all postinfectious mucocutaneous eruptions such as the widely reported Mycoplasma pneumoniae‐induced rash and mucositis (MIRM). Very few reports in the literature regarding recurrent RIME are found. We present two adolescent cases of recurrent RIME that involve SARS‐CoV‐2 and influenza A where the latter is a newly reported infectious trigger; in both patients, the initial episode was likely triggered by Mycoplasma pneumoniae (MP) infection.

Introduction Patients qualified for the Polish government programme of treating severe pemphigus diseases with rituximab (RTX) available in 2018-2019 had to meet numerous criteria, including no active infectious disease. Aim The clinical usefulness of tuberculosis screening with the QuantiFERON-TB Gold Plus (QFT-Plus) in native pemphigus patients selected for RTX treatment was statistically evaluated. Material and methods Eighteen pemphigus patients were examined with QFT-Plus prior to the intended RTX therapy. Ninety hospital employees examined with QFT-Plus due to contact with a cleaning worker who was diagnosed with active pulmonary tuberculosis were the control group. Results Six of 18 pemphigus patients had a positive QFT-Plus test result, one indefinite result and one initially indefinite and then negative. In the control group, 26 of 90 employees had a positive test result and none had an indefinite result. Statistical analysis by Fisher's exact test showed no statistically significant difference in QFT-Plus positive results between the groups (p = 0.5577). Only in 1 patient with recurrent mucocutaneous pemphigus vulgaris previously treated with traditional immunosuppression, lung changes were detected by computed tomography. No employee had any changes in the chest radiograph. Conclusions Prior immunosuppression and autoimmunity might be the cause of indefinite test results, but they do not seem to increase positive results. In the native population, the QFT-Plus screening reveals a significant population exposure to M. tuberculosis infection independent of pemphigus autoimmunity, and such screening can be a starting point for identifying patients requiring anti-tuberculosis drug prophylaxis before combined RTX-glucocorticosteroid treatment.

Steven Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) are acute life threatening mucocutaneous drug reactions. Several therapies have been used in the treatment of SJS/TEN but none of them have been established as the gold standard treatment, until now. Studies showed that cyclosporine (CsA) can be used off-label in TEN/SJS and has shown promising therapeutic effectiveness in such diseases. Here we report a 38 year-old woman who presented to Ar Rustaq Hospital, Rustaq, Oman in 2019 with SJS/TEN overlap and was treated successfully with CsA along with supportive management. This case report also includes a literature review on use of CsA in the treatment of SJS/TEN. Keywords: Steven Johnson Syndrome; Toxic epidermal necrolysis; Epidermal necrolysis; cyclosporine; Drug eruption; case report; Oman

BACKGROUND: Active surveillance of methicillin-resistant Staphylococcus aureus (MRSA) carriers is associated with the lower incidence of bacteremia and lower mortality rates throughout literature; yet, this important step still remains problematic for developing countries, particularly Indonesia. AIM: The study aimed to demonstrate MRSA colonization rate in Haji Adam Malik Hospital, Medan, Indonesia. MATERIALS AND METHODS: The study enrolled 200 mucocutaneous isolates obtained from hospitalized patients during a 1-year period of study (2018). VITEK-2 system in addition to standard bacterial identification, such as gram staining, latex agglutination test, and hemolysis pattern, was performed to select S. aureus colonies in two different laboratories, Microbiology laboratory of Haji Adam Malik General Hospital and Multidisciplinary Laboratory, Faculty of Medicine, Universitas Sumatera Utara, for polymerase chain reaction (PCR) examination. RESULTS: Based on the VITEK-2 system preliminary identification, there were 80 S. aureus colonies which then underwent PCR examination. Through standard PCR assay, there were 32 bacterial isolates contained the mecA gene and it can be determined MRSA colonization rate of the hospital was 16% with consistent results of standard bacterial identification. CONCLUSIONS: Active surveillance of MRSA carriers is mandatory and urged it as a regular program in a hospital setting to decrease MRSA transmission rate.

A 50-year-old male Sudanese patient presented with a three-week history of jaundice, high-grade fever, and mucocutaneous eruption. For last months he was on compound therapy for leprosy, which had been confirmed recently. The patient’s face was prominent, along with the erythematous dusky morbilliform rash covering all the body. On examination, we detected hepatosplenomegaly and generalized lymphadenopathy. Laboratory investigations revealed hepatorenal impairment, and hematological analysis revealed leukocytosis mainly due to eosinophilia. The clinical and laboratory findings interpretation ranked DRESS or Drug-Induced Hypersensitivity Syndrome (DIHS) on top of possible causes before Dapsone Hypersensitivity Syndrome (DHS) and lepra reactions. We promptly discontinued MDT, admitted him to the dermatological ward. Two skin biopsies were sent to two different histopathologists, MF was suggested by one and Sezary syndrome by the other one. Besides the general conservative measures and vital functions monitoring, he received systemic and topical steroids. However, unfortunately, within the next three weeks, his condition deteriorated, and passed away from multi-systems failure.

Systemic lupus erythematosus (SLE) is a chronic autoimmune disease that affects multiple organs such as skin, joints, kidneys, heart, lungs, nervous system and blood. Pulmonary hypertension is a rare complication of SLE.1 Systemic lupus erythematosus associated with pulmonary hypertension was defined as an increase in the average pressure in the pulmonary artery at rest ? 25 mmHg with pulmonary capillary wedge pressure ?15 mm Hg and an increase in pulmonary vascular resistance.2 The prevalence of pulmonary hypertension in SLE approximately 0.5 to 17.5%. Predictors factors of the occurrence of pulmonary hypertension in LES is Raynaud's phenomenon, anti-U1RNP antibody, and antibody positive anticardiolipin.3 A woman aged 37 years came with a chief complaint of pain in the fingers and toes with black-colored wounds felt since 6 months before admission. From the anamnesis, physical examination, support to meet 5 of the classification criteria for systemic lupus erythematosus based on the 1997 ACR criteria which includes manifestations of arthritis, mucocutaneous, serositis, lupus antiokoagulan, and ANA IF positive. In these patients also found the typical signs and symptoms of Raynaud's phenomenon which leads to the symptoms of pain in the fingers of both hands when exposed to cold and pale to red when heated and has been confirmed from the results of arteriography. From the results of echocardiography reveal any pulmonary hypertension. This patient was treated with steroids, immunosuppressants and antiplatelet. The case was removed because of pulmonary hypertension is a complication LES rare and necessary sharpness in diagnosis. Patients with pulmonary hypertension of unknown or untreated can become a progressive right heart failure and lead to death.3

Introduction Immunotherapy with immune checkpoint inhibitors (ICIs) has transformed cancer treatment over the past decade, improving survival rates in numerous advanced cancers. Immune-related adverse events (irAEs) are common and can affect any organ system, with many of these toxicities being well-characterized with clear grading criteria and management approaches. There has been less emphasis on oral manifestations of irAEs. Objective This review provides an overview of oral manifestations of irAEs, including mucosal and salivary gland toxicities, and proposes a grading system and management guidelines. Conclusion irAEs are common treatment-related toxicities in patients treated with ICIs. Oral irAEs can range from asymptomatic white reticulations to life-threatening mucocutaneous reactions requiring aggressive management with corticosteroids and/or permanent discontinuation of ICIs. Oral healthcare providers should be prepared to identify and manage oral irAEs in collaboration with oncologists and other specialists.

Purpose of review To review diagnosis, clinical characteristics and treatment of multisystem inflammatory syndrome in children (MIS-C) associated with severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2). Recent findings MIS-C emerged in spring 2020 as a hyperinflammatory syndrome following SARS-CoV-2 exposure in children. Despite growing awareness of MIS-C, diagnosis remains challenging due to the range of phenotypes and severity. Fever accompanied by shock, cardiac dysfunction, gastrointestinal symptoms, or mucocutaneous signs suggestive of Kawasaki disease, especially in the presence of known or suspected coronavirus disease 2019 exposure, should trigger consideration of MIS-C. However, clinical presentations are highly varied and may overlap with other infectious diseases. Clinicians must maintain a high index of suspicion for MIS-C and be aware that patients may develop coronary artery aneurysms and myocarditis even with few or no Kawasaki disease symptoms. More precise diagnostic criteria and specific biomarkers are needed to aid diagnosis. Intravenous immunoglobulin (IVIG) is first-line therapy, and steroids should be considered as initial adjunctive treatment for patients with severe manifestations or other risk factors. Prompt treatment is essential, as patients may worsen acutely, though overall prognosis is reassuring. Summary MIS-C associated with SARS-CoV-2 has varied clinical manifestations. Clinicians must be aware of the common presentation and potential for decompensation and cardiac sequalae to guide appropriate evaluation and treatment.

Mohammed Abd El Monem Teama,1 Marwa Adham El-Mohamdy,2 Fatma Abdellah Abdullah Mahmoud,2 Fatma Mohammed Badr1 1Internal Medicine Department, Division of Rheumatology and Immunology, Faculty of Medicine, Ain Shams University, Cairo, Egypt; 2Clinical Pathology Department, Faculty of medicine, Ain Shams University, Cairo, EgyptCorrespondence: Mohammed Abd El Monem Teama 2 Staff Member Buildings of Ain Shams University, El Al Waili/El-Abaseya, Cairo, 11517, EgyptTel +20 1277580006Email mohteama2009@yahoo.comObjective: This study was conducted to estimate the frequency of anti-nuclear antibodies (ANAs), anti-dsDNA, and anti-extractable nuclear antigen (ENA) antibodies in juvenile systemic lupus erythematosus (JSLE) patients and their association with different clinical manifestations and disease activity.Patients and Methods: A cross-sectional study that includes 100 JSLE patients from Ain Shams University Hospital was conducted. All subjects underwent history taking, clinical examination, assessment of disease activity based on the SLE disease activity index (SLEDAI), laboratory investigations, and tests for autoantibodies, namely ANA, anti-dsDNA, and anti-ENA antibodies, including anti-Ro (SSA), anti-La (SSB), anti-Smith (Sm), and anti-U1-ribonucleoprotein (U1-RNP).Results: The most common clinical features were polyarthralgia (71%), haematological manifestations (65%), malar rash (54%), and nephritis (51%), respectively. All patients had positive ANA (100%), while anti-dsDNA frequency was 83%. The most common anti-ENA antibodies were anti-RNP (41%), anti-Sm (31%), anti-SSA (27%), and anti-SSB (20%), respectively. Anti-RNP had a clinical association with oral ulcer, Raynaud’ phenomena, haematological, neuropsychiatric and thromboembolic manifestations. Meanwhile, anti-Sm had a significant association with serositis, mucocutaneous, constitutional, and neuropsychiatric manifestations. Anti-SSA was associated with mucocutaneous, musculoskeletal, Raynaud’ phenomena, renal, haematological and cardiac manifestations, while anti-SSB was significantly associated with malar rash, serositis, thromboembolic, musculoskeletal, and neuropsychiatric manifestations. Concerning SLEADI score, anti-dsDNA antibody was significantly associated with moderate disease activity score (p=0.032) while anti-SSA significantly associated with high disease activity (p=0.045). Both anti-SSB and anti-Sm were significantly associated with both moderate and high disease activities, meanwhile anti-U1-RNP was associated with moderate disease activity (p=0.014).Conclusion: Anti-dsDNA and anti-ENAs antibodies were frequently found in JSLE patients (83%, 63%), respectively. They were significantly associated with variable clinical manifestations and could be used as predictors for assessment of disease activity.Keywords: juvenile systemic lupus erythematosus, clinical manifestations, anti-ENA, disease activity score

Cutaneous, mucosal, and nail examination is the key to unveiling a plethora of systemic diseases. Mucocutaneous lesions directly related to human immunodeficiency virus (HIV) infection usually present as initial manifestations of immune deficiency, of which few lesions act as predictors of an immunocompromised state. Here, we report two cases who presented with onycho-mucocutaneous symptoms which raised the suspicion of and invariably led to the diagnosis of an underlying immunosuppression secondary to HIV infection.

EBV-driven B cell neoplasms can rarely present as an extranodal mass in the gastrointestinal tract and can be missed, even by experienced pathologists, because of this uncommon presentation. A selection of these neoplasms, namely EBV-positive diffuse large B cell lymphoma, not otherwise specified (DLBCL NOS), EBV-positive mucocutaneous ulcer (EBV MCU), extracavitary primary effusion lymphoma (EPEL), and EBV-positive Burkitt lymphoma, will be discussed in the present review. Besides the common thread of EBV positivity, these lymphoproliferative disorders arise in unique clinical settings that are often associated with immunodeficiency, immunosuppression or immunosenescence and can present as solitary masses albeit rarely, within the gastrointestinal tract.