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Objectives: Chilblain lupus erythematosus (LE) is a rare chronic cutaneous lupus erythematosus (CCLE) characterized by the appearance of violaceous plaques in acral regions most exposed to cold. The isolated form affects middle-aged women, while the familial form manifests in early childhood and is associated with mutations in the TREX1 gene. Result(s): A 13-year-old adolescent, with no relevant family history, was referred in March 2021 for suspected chilblain-like lesions associated with COVID-19 infection. The patient presented with multiple violaceous papules on hands and feet. The lesions were slightly painful. Small hyperkeratotic papules were also observed on finger pads. Physical examination also revealed some aphthae affecting the lips. No other systemic symptoms were reported. A skin biopsy and blood tests were performed due to presumed chilblain LE with probable systemic involvement. Histology revealed basal vacuolar damage and intense perivascular and periadnexal lymphocytic inflammatory dermal infiltrate. Remarkably, mucin was noted among the collagen bundles. Leukopenia and positive ANA antibodies (titre 1:320) were detected. Complement levels were normal. SARS-CoV2 infection was ruled out. Skin lesions disappeared within 1 month under topical corticosteroids. Hydroxychloroquine was afterwards started by Rheumatology without recurrence of skin symptoms until last follow-up. Discussion(s): We present an uncommon case of an adolescent with systemic LE presenting as chilblain LE. Chilblain LE can be accompanied by other discoid CCLE. It can progress to systemic LE in up to 20% of patients, especially when concomitant CCLE is present. This rare presentation of CCLE should be differentiated from typical chilblain and other resembling lesions, such as SARS-CoV2-associated chilblain and acral purpuric lesions (COVID toes). The Mayo Clinic diagnostic criteria can be helpful, particularly in this last SARS-CoV2 outbreak scenario, when the reporting of similar skin lesions has been significant.
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The COVID-19 pandemic has affected the lives of people around the world since March 2020. These sudden changes have caused an increase in stress, anxiety, and depression, especially in students who are making adjustments in their academic world. Therefore, this research aims to determine the relationship between the level of stress experienced during the COVID-19 pandemic and the incidence of Recurrent Aphthous Stomatitis (RAS). This research involves an analytic survey with a cross-sectional approach. The populations used were students who are actively learning at the Faculty of Dentistry, University of Sumatra Utara. Sample selection method is by purposive sampling method, where the sample size is determined based on specific characteristics. Inclusion criteria: students with ID numbers in 2017 and 2018 who are willing to sign an informed consent. Determination of a minimum sample size of 130 students. Measurement of research variables was conducted using CPDI questionnaire, and RASDX questionnaire. Data collection is performed online by using Google Forms while data analysis is performed by Chi-square test. In the RASDX test, 27.7% of the students were RAS-positive, 72.3% RAS-negative. There were 32.3% of students who had normal stress levels, 57.7% of students had mild-moderate distress, and 10% of students had severe distress. The major stressors used in this research were;the fear of contracting the virus (36.9%). Chi-square analysis showed p of 0.004, it was concluded that there was a significant relationship between stress during the COVID-19 pandemic and the incidence of RAS in college students. © 2023,Journal of International Dental and Medical Research. All Rights Reserved.
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Introduction: Varicella zoster virus is a highly infectious alpha-herpesvirus, pathogenic only to humans. The primary infection of varicella zoster virus causes chickenpox, which is contagious and primarily infects children and adolescents in India. Following the primary infection, the virus remains dormant in sensory root ganglia. Activation of the dormant virus in later stages of life causes herpes zoster infection which may vary from subclinical infection to typical zoster, scattered vesicles, zoster sine herpete or disseminated zoster, which depends on the individual's immune status. Case report: In this case series, we present two patients with herpes zoster involving the mandibular branch of the trigeminal nerve. Cytology revealed characteristic features of the infection including nuclear moulding, multinucleated giant cells and ballooning degeneration. Conclusion(s): More recently, patients presenting with herpes zoster have been reported to have sub-clinical Covid-19 infection, suggesting a possibility that herpes zoster might be an indicator for latent Covid-19. Timely detection and treatment of this infection can reduce the risk of post herpetic neuralgia.Copyright © 2022 Sciendo. All rights reserved.
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Introduction: COVID-19 disease is caused by SARS COV-2 virus. Though it primarily affects the lower respiratory tract, reports have indicated that specific cutaneous manifestations are associated with COVID-19. Objective(s): To evaluate the persistent dermatologic long term sequelae of SARS-CoV-2 infection, among recovered COVID-19 infected patients. Method(s): Baseline data were retrospectively collected from patient's medical records from the department of dermatology over 1 year (January 2021-January 2022), at a designated tertiary care centre. The demographic data, severity of COVID disease, and pre-existing cutaneous and systemic co-morbidities were noted. Dermatologic, hair and nail manifestations were recorded. The results were statistically analyzed. Result(s): Record of total 972 patients were analyzed in our study, with 432 males and 340 females. Out of these, 88 cases (9.05%) had skin manifestations, of which 35 (39.77%) were male, and 53 (60.22%) were females. About 50% of cases experienced long term skin diseases after 6 months of the recovery. The majority, 47 (53.4%) of patients with skin manifestations, were in the age group of 30-50 years, followed by 31 (35.22 %) of patients in the 50 years age and above group. Urticaria and Pruritus were the most common manifestations 26 (29.5%), followed by telogen effluvium 24 (27.7%), herpes zoster 16(18.1%), pityriasis rosea, acneform eruptions, acral erythema, irritant contact dermatitis, palmar keratoderma, aphthous ulcer with lip crackling, eruptive pseudo angiomatosis, aquagenic keratoderma, and others. Conclusion(s): Prevalence of cutaneous, hair and nail manifestations among COVID-19 patients was 88 (9.05%) in our study. More extensive research is required to establish our knowledge on the relation between skin and COVID-19.Copyright © 2023, Dr Yashwant Research Labs Pvt Ltd. All rights reserved.
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Background: Coronavirus disease 2019 (COVID-19) is caused by severe acute respiratory syndrome coronavirus 2 (SARS CoV-2) infection in human respiratory cells. The first spread of this disease first occurred in Wuhan, China, in December 2019, and on March 11, 2020, the World Health Organization declared the spread of the infection as a global pandemic. People who are infected experience mild to moderate clinical symptoms which are characterized by an increase in T cell, CD8, and IgM/IgG levels. The occurrence of severe clinical symptoms reflects an excessive and uncontrolled increase in the secretion of pro-inflammatory cytokines, which impacts the systemic condition and periodontal tissues of affected patients. Objective(s): The aim of this study is to describe the impact of SARS CoV-2 infection on periodontal tissues. Method(s): In this study, we searched the NCBI, PubMed, Google Scholar, and Research Gate databases. The keywords used were "coronavirus disease" o"COVID-19" or "SARS-CoV-2" with "oral manifestations" and "periodontal," and we screened the articles using the PRISMA flow diagram. Result(s): Patients with COVID-19 present with a variety of systemic symptoms, as well as manifestations in the periodontal tissues, especially in the gingival area. Conclusion(s): SARS CoV-2 infection can impact periodontal tissues in the form of necrotizing ulcerative gingivitis, aphthous stomatitis, gingivahyperpigmentation, edema and erythema, lichen planus, and aphthous-like ulcers.Copyright © 2023 Karyadi et al.
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Background: It is very important to increase awareness and understanding of oral manifestations of post COVID-19 disease among dentists which can happen by continuing education and training for dentists to recognize and manage oral manifestations of post COVID-19 disease. Aims & objectives: The aim of this is to aware dentists regarding oral manifestation of covid 19 disease. The objectives of study were to assess the level of awareness, understanding and factors influencing awareness among dentists regarding oral manifestations of post COVID-19 disease. Evaluate the of oral manifestations of post COVID-19 among dentists in central India. Methodology: A cross-sectional, questionnaire-based study was carried out among COVID-19 recovered patients. A sample of 100 subjects, diagnosed as mild and moderate cases of COVID-19 disease were selected based on inclusion and exclusion criteria. A well-structured questionnaire composed of total 21 Closed ended questions was send to sunjects. Result(s): The study comprised an almost equal number of male (54%) and female (48%) participants and among them, 47% belong to the health professional group. A total of 56% of subjects were aged above 35 years and 47% below 35 years. Xerostomia, frequent aphthous ulcers, swallowing difficulty, and burning mouth were the most frequently encountered symptoms in study subjects during the disease and post recovery. Conclusion(s): In the present study, dentists were found to have good knowledge toward novel corona virus disease with optimum preparedness level for dental practice modification. Dental fraternity is further advised to follow the standard guidelines overcoming this pandemic.Copyright © 2022 Authors. All rights reserved.
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Background: Vulvar aphthous ulcers are defined by acute onset of painful genital lesions that are associated with viral illness. They classically present in non-sexually active girls and are diagnosed once other more common cause of genital ulcerations, including STIs or autoimmune bullous diseases, have been excluded. Previous reports of vulvar aphthous ulcers associated with COVID-19 have described treatment of the symptoms with either local or systemic corticosteroids. This case series illustrates the ability to manage these patients conservatively resulting in complete and spontaneous resolution of the ulcers. Case: Four females, ages ranging from 10 to 21, presented to the emergency department for vulvar pain. Each patient was diagnosed with vulvar aphthous ulcers based on physical examination. All four patients were diagnosed with COVID-19 with rapid antigen testing. With directed counseling, each patient and their family were instructed on a regimen of acetaminophen, sitz baths, and voiding in the bath. Topical analgesics such as lidocaine gel were not administered and steroids were not prescribed. All four patients were able to manage their symptoms at home and did not require admission to the hospital. All patients experienced spontaneous and complete restoration of anatomy in 1-2 weeks. Comments: The novel coronavirus pandemic caused by SARS-CoV02 has resulted in considerable morbidity and mortality. While immunocompromised hosts are more susceptible to complications of COVID-19, patients with intact immune systems may also experience distressing viral related symptoms. The pathogenesis of these ulcers has been hypothesized to be a result of non-specific inflammatory response to a viral systemic illness resulting in blistering of the mucosal genital surfaces It has been proposed that the ulcers are secondary to a cytokine storm that occurs in SARS-CoV-2 infections. Elevated cytokines, including TNF-alpha, result in neutrophil chemotaxis to mucosal tissue and subsequent ulceration of the tissue. Previous case studies discussing vulvar aphthous ulcers associated with COVID-19 all required hospitalization for pain control and/or urinary retention and treatment with steroids. Hospitalization can be a traumatic experience for both child and adolescent patients as well as their family. The added isolation and precautions required for treating COVID positive patients can have a further psychological impact. Through directive counseling, all patients in our case series were able to avoid hospitalization and supportive care of the genital lesions in the outpatient setting was sufficient.Copyright © 2023
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STUDY OBJECTIVE: To review and characterize reports of vulval aphthous ulcers (VAUs) following COVID-19 vaccination in VigiBase, the World Health Organization global database of reported potential adverse effects of medicinal products, to demonstrate the importance and power of case reports for rare suspected adverse reactions and to investigate whether they suggest a potential for COVID-19 vaccination to be a trigger METHODS: Cases reporting the Medical Dictionary for Regulatory Activities' (MedDRA) preferred term "Vulvovaginal ulceration" and related preferred terms in adolescent patients aged 12-17 years in association with any COVID-19 vaccine were extracted from VigiBase. The cases were clinically reviewed, and causality was assessed by applying the Bradford Hill criteria to the obtained case series. RESULTS: As of June 30th, 2022, there were 444 reports for the selected Medical Dictionary for Regulatory Activities preferred terms following COVID-19 vaccination in VigiBase. Ninety-four de-duplicated reports concerned adolescent female patients. Thirty-seven cases were clinically consistent with the diagnosis of VAU. On causality assessment, the analyzed case series fulfilled 6 of the 9 Austen Bradford Hill criteria supporting a potential causal relationship. CONCLUSION: VAUs can be perceived as a traumatic experience, especially in adolescent patients. There is, furthermore, a risk that the ulcers will be misdiagnosed, resulting in avoidable investigation and treatment burdens for patients. We communicate our findings to support the small number of published case reports and raise awareness of VAUs occurring in a temporal association with COVID-19 vaccination. Furthermore, our analysis supports observations about the value of case reports for the recognition and assessment of rare adverse events.
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This case report describes a 15-year-old female who presented with acute onset of painful genital ulcers. She denied being sexually active and tested positive for severe acute respiratory coronavirus 2 five days prior. Associated symptoms on days 1 to 3 included fatigue, nausea, headaches, and fever. Lipschutz ulcers (LUs), also known as aphthous ulcers, acute genital ulcers, and acquired genital ulcerations; are an uncommon, self-limiting, nonsexually transmitted condition characterized by the rapid onset of painful, necrotic ulcerations of the vulva or lower vagina. The pathogenesis of LUs is unclear, and the diagnosis is one of exclusion.
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BACKGROUND: Due to Coronavirus disease, several body systems are involved, and, in the most severe cases, there may be a multiorgan disfunction. The general symptoms can be associated with lesions of the oral cavity. Among these the most reported are ulcerative lesions, together with vesicles and blisters, petechiae, and erythema multiforme-like lesions, aphthous-like lesions, herpetiform lesions and they typically appear at the same time as general symptoms or within one week. The aim of this study was to describe oral lesions observed in 41 Armenian patients with SARS-CoV-2 infection. METHODS: During the period of April 2020-January 2021, 43 patients aged 25 to 51 years positive for COVID-19 test were referred for a full oral mouth examination and diagnosis at Nord KS dental clinic. RESULTS: Of the 18 patients who were already diagnosed with SARS-CoV-2 infection at the time of the visit, 66.6% presented ulcerative lesions;in this group also Candida Albicans infection and lichenoid lesion were diagnosticated. 29 patients were suspected to be positive for COVID-19 and 23 tested positive. In this group of patient periodontal altera-tions relatable to hyperemia, gingival cyanosis and vascular dilation were the most frequently encountered manifesta-tions.CONCLUSIONS: Our study shows how the type of oral lesions during COVID-19 can be related to a determinate phase of the clinical progression: in the first stage of disease oral lesions could be an early sign of infection and during the full-blown phase of this disease they could be clinical manifestation of vasculitic and thrombotic phenomena responsible of COVID-19 complications. The role played by the oral examination in patients with COVID-19 is important in order to raise the suspicion of infection early and to monitor and improve the state of oral health and painful symptoms, in the event of the appearance of more disabling lesions.
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The novel coronavirus disease 2019 is highly contagious disorder cause severe illness in form of fever, myalgia, fatigue, altered smell and taste sensation. The oral cavity manifestations has been discovered in Covid 19 patients. Studies shows that SARS-COV -2 affect respiratory track along with other organs those who have Angiotensin coverting enzyme 2 (ACE 2) receptors. ACE 2 Receptors are found in numerous amount in the oral cavity tissue which are more prone for SARS-COV-2 infections. There are various types of oral lesion associated with covid -19 patients such as ulcers in oral cavity, candidiasis, recurrent herpes simplex, geographical tongue, mucositis and petechiae. It affects oral cavity as well as salivary glands. This review article discusses the major neglected clinical entity such as oral cavity lesion in Covid -19 patients. Copyright © 2022 Wolters Kluwer Medknow Publications. All rights reserved.
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Background: Pandemic of coronavirus disease 2019 (COVID-19), caused by severe acute respiratory syndrome-coronavirus 2 (SARS-CoV-2), has become a major global health issue since December 2019. Patients affected by systemic rheumatic disorders represent a high-risk group for severe COVID-19. During the COVID-19 pandemic, vaccination has become one of the cornerstones of the fght against this disease. The EULAR and the ACR recommend vaccination in all patients with rheumatological diseases. There is a paucity of data regarding the safety of COVID-19 vaccines in patients with rheumatic and musculoskeletal diseases such as BehÇet's disease (BD). Objectives: In this study, we evaluated the safety and tolerance of COVID-19 vaccines, post-vaccine BD exacerbation and discontinuation of BD therapy in BD patients by retrospectively examining our BD cohort, from the patients' perspective. Methods: We retrospectively evaluated 450 BD patients followed in our clinic using hospital records and formed a retrospective cohort of patients who met the International Study Group (ISG) criteria. COVID-19 vaccination status, vaccine type (inactivated or mRNA), post-vaccine side effects and exacerbations, drug compliance, change in treatment after exacerbation, and post-vaccine COVID-19 occurrence were evaluated by interviewing patients over the phone or face to face. Patient demographics, comorbid diseases, and active BD treatments were collected from our hospital records. Disease activity was measured using the BSAS and the BDCAF form. Results: Our cohort consisted of a total of 450 BD patients. Two hundred and eighty seven patients had at least 1 dose of the COVID-19 vaccine. Of the total number of COVID-19 vaccines (n= 639), 379 (59%) were Pfzer-BionTech vaccines and 257 (41%) were Sinovac vaccines. The side-effects after frst, second, third and fourth vaccine dose were 151 (52.6%), 135 (47%), 29 (10.1%) and 3 (1%), respectively. BehÇet fare after frst, second, third and fourth vaccine dose were 151 (52.6%), 135 (47%), 16 (22.9%) and 3 (33.3%), respectively. The most common side effects were arm pain, joint pains or arthritis, malaise, while the most common BD exacerbations were arthralgia or arthritis, oral aphthae, pap-ulopustular eruption Pfzer-BionTech and Sinovac vaccines were compared in terms of side effects, there was a signifcant difference after the 1st (p<0.001) and 2nd doses (p<0.001), but no signifcant difference was found at the 3rd dose (p= 0.353) (Table 1). When Pfzer-BionTech and Sinovac vaccines were compared in terms of BD exacerbations, no signifcant difference was found after the 1st (p= 0.417), 2nd (p= 0.465) and 3rd doses (p= 0.565). Only 4 patients (1.3%) developed exacerbation with organ involvement after COVID-19 vaccine. Anterior uveitis developed in 2 patients, panuveitis in 1 patient, panuveitis and deep vein thrombosis in one patient. Conclusion: In conclusion, our study shows that the COVID-19 vaccine is well tolerated in BD patients, and that post-vaccine BehÇet's exacerbation predominantly includes mucocutaneous and articuler fndings, and exacerbations with organ involvement are rare. According to the COVID-19 vaccines, although the side effects were more in the Pfzer-BioNTech group compared to the Sinovac group, there was no difference between BehÇet's fare compared to the COVID-19 vaccines.
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Background: Behçet's disease (BD) is an systemic infammatory vasculitis characterized by recurrent oral aphthae and genital ulcers. In the course of the disease, skin, eye, musculoskeletal, nervous system and gastrointestinal system involvements can be detected. Objectives: To evaluate the clinical, laboratory and radiological fndings of pedi-atric cases diagnosed with BD. Methods: Fifty patients (0-20 years old) who were followed up with the diagnosis of BD at the Pediatric Rheumatology outpatient clinic of Uludag University Faculty of Medicine between January 2011 and July 2021 were included in our study. The patients were diagnosed according to the diagnostic criteria of the International BD Study Group. Results: Twenty-four (48%) of the patients were male and 26 (52%) were female. The mean age at the diagnosis of patients was 9 ±4.55 years (10.75±4.55 years in boys, 12.35±3.65 years in girls). Twenty patients (36.3%) had a family history of Behçet's Disease. Oral aphthae were present in 96% (n=48) patients, while genital ulcers were in 32% (n=16) (Table 1). Of the nine patients with uveitis, 6 had panu-veitis, 2 had anterior uveitis and 1 had posterior uveitis. There was no difference in the distribution of symptoms according to the gender of the patients. HLA-B51 allel was found in 39 (78%) and ANA was positive in 14 patients (28%). Immunological tests showed that serum immunoglobulins were low in 11 (32.3%) of 34 patients. Low IgG levels were detected in 6 patients, low IgM levels were in 3, and low IgA levels were in 2. Thrombus was presented in three cases (thrombus in the right ventricle in one case, in the intracranial transverse sigmoid sinus and left jugular vein in two cases). The most commonly used drug for aphthae was colchicine (n=45, 82%). The use of biological agents according to patient manifestations is shown in Table 2. In the follow-up, clinical fndings improved in 35 patients (70%) Complete improvement was detected only with biological agents in 8 patients with uveitis. One patient was operated due to the development of complicated cataracts secondary to uveitis. Three patients were diagnosed with Covid-19, one of them was followed without treatment, while two of them were treated with favipiravir at home. Three patients with Covid-19 infection were using only colchicine treatment. Conclusion: Behçet's disease is rare in childhood. Although it is not common, life-threatening complications can be observed. To reduce morbidity and complications, physicians should be aware of manifestations and rare clinical pictures of the BD.
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The aim is to determine oral manifestations in patients with COVID-19 disease and in the postcovid period. Methods: A special survey (questionnaire) was made in 424 people who had COVID-19 confirmed by RT-PCR, ELISA for specific IgM and IgG antibodies and Chest CT scan (168 people). 123 people had complaints and clinical symptoms in the oral cavity 2-6 months after the illness and they came to the University dental clinic. Laboratory tests have been performed (clinical blood test, blood immunogram, virus and fungal identification). Results: Survey results showed that 16,0% participants had asymptomatic COVID-19, 23,6% - mild and 48,1% moderate disease. 12,3% with severe COVID-19 were treated in a hospital with oxygen support. In the first 2 weeks 44,3% indicated xerostomia, dysgeusia (21,7%), muscle pain during chewing (11,3%), pain during swallowing (30,2%), burning and painful tongue (1,9%), tongue swelling (30,2%), catharal stomatitis (16,0%), gingival bleeding (22,6%), painful ulcers (aphthae) (8,5%) and signs of candidiasis - white plaque in the tongue (12,3%). After illness (3-6 months), patients indicated dry mouth (12,3%), progressing of gingivitis (20,7%) and periodontitis (11,3%). In patients who applied to the clinic we identified such diagnoses: desquamative glossitis - 16 cases, glossodynia (11), herpes labialis and recurrent herpetic gingivostomatitis (27), hairy leukoplakia (1), recurrent aphthous stomatitis (22), aphthosis Sutton (4), necrotising ulcerative gingivitis (13), oral candidiasis (14), erythema multiforme (8), Stevens-Johnson syndrome (2), oral squamous cell papillomas on the gingiva (4) and the lower lip (1). According to laboratory studies, virus reactivation (HSV, VZV, EBV, CMV, Papilloma viruces) was noted in 52 patients (42,3%), immunodeficiency in 96 people (78,0%), immunoregulation disorders (allergic and autoimmune reactions) in 24 people (19,5%). Conclusions: Lack of oral hygiene, hyposalivation, vascular compromise, stress, immunodeficiency and reactivation of persistent viral and fungal infections in patients with COVID-19 disease are risk factors for progression of periodontal and oral mucosal diseases.
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Background: Aplastic anaemia is a disease that occurs when the body fails to produce enough blood cells. Oral lesions, such as canker sores, are often used as an early marker of this disease condition. In the COVID-19 pandemic era, a teledentistry method is expected to assist in the management of patients with various diseases including aplastic anaemia. Purpose: This case report discusses the management of patients with aphthous-like ulcers related to aplastic anaemia in a 34-year-old woman, based on history, clinical examination, and blood tests through teledentistry. Case: The patient complained of a bleeding wound on the inner left cheek and corner of the left lip that appeared one month ago. There was an ulcer lesion that was bleeding easily in the buccal mucosa sinistra and the angular sinistra, as well as macules lesion on her hand and foot. The complete blood count test exhibits a low platelet level. Case management: The diagnosis was an aphthous-like ulcer related to aplastic anaemia. Patient was treated with topical antiseptic and anti-inflammatory mouthwashes for the oral lesions, as well as collaboration with the medical team. The lesions improved after the patient received a blood transfusion and drug therapy from the internist while she was hospitalized, but the lesions reappeared in other locations in the oral cavity when the platelet level dropped. Conclusion: Management of oral manifestations in patients with aphthous-like ulcers related to aplastic anaemia in the COVID-19 pandemic era can be done through teledentistry. However, this requires sensitivity from the dentist, patient compliance in following instructions, and the involvement of a multidisciplinary approach, such as collaboration with internists to achieve recovery. Delay in detecting and treating aplastic anaemia will lead to death, as in this case. © 2022 The authors.
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We report a 15-year-old boy who developed aseptic meningitis 10 days after administration of the second dose of the COVID-19 vaccine BNT162b2. Although accompanying aphthous mouth ulcers resembling herpetic stomatitis initially led us to suspect an underlying viral infection, broad virological and microbiological screening did not identify any causative pathogen. Gonarthritis and skin lesions, which both developed within three days after admission, extended the clinical presentation eventually resembling an acute Behçet's disease episode. This is the first description of a juvenile patient with aseptic and pathogen-negative meningitis occurring in close temporal association with vaccination against COVID-19, along with a few previously reported adult patients with isolated meningitis and a further case with meningitis and an accompanying Behçet's disease-like multisystem inflammation episode as seen in our patient. With billions of individuals being vaccinated worldwide so far and only a few cases of aseptic pathogen-negative meningitis reported in close temporal relation, causality is unclear. However, aseptic meningitis should be kept in mind in the differential diagnosis of patients with persistent or delayed onset of headache and fever following COVID-19 vaccination.
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Introduction: We describe a case of non-specific colitis as the first manifestation of an autoinflammatory disease. Objectives: A., 12 months old, comes to our attention for severe normocytic anemia, without signs of hemodynamic failure. From 3 months of age, A. had a history of bloody diarrhea during exclusive maternal breast-feeding. This condition was diagnosed as allergic proctitis and maternal diet free of milk and derivatives was started. The weaning was conducted in a diet free from milk too. Despite a reduction in bloody diarrhea complete resolution of the symptom never occurred. At admission to our Center, the patient performed a colonoscopy, which revealed the presence of small, eroded translucent nodules and bleeding mucosa suggestive of nodular lymphoid hyperplasia throughout the colon. Histological examination showed active colitis without signs of colitis IBD or allergic colitis. After discharge, we began oral steroid therapy and gastroenterological follow-up. Two weeks later, during steroid withdrawal, A. came to Emergency Room for acute panniculitis of the limbs for which we prescribed an anti-inflammatory therapy. After 48 h the skin condition worsened and she was febrile and suffering. Therefore, A. was hospitalized for further investigations. Blood tests showed a rise in inflammation indexes. Given the inflammatory status, intravenous steroid therapy was started with rapid response. At each attempt to stop steroid treatment, the same symptoms recurred. We also noted the presence of adverse effects related to therapy (Cushing-like aspect and irritability). Methods: In differential diagnosis, the following hypotheses were evaluated and relative tests were performed. 1) Haematological disease: bone marrow aspirate negative for neoplastic conditions. 2) Infectious disease: swabs for viruses and bacteria, as well as serological tests all negative. No history of COVID19 or contact with COVID19 patients. 3)Immunological/inflammatory disease: family history of autoimmune conditions (father with recurrent oral aphthosis, vitiligo and thyroiditis) and unkown diseases with earlyonset (paternal grandfather with liver disease started when he was young, brother of paternal grandfather died at 16 years old due to unexplained causes). Therefore, first level autoimmune tests were done, resulted all normal. Then the possibility of early-onset chronic inflammatory bowel disease (VEO-IBD) were evaluated but genetics was negative. Finally, since autoinflammatory genesis was suspected, interferon signature and molecular investigations of the main known genes responsible for auto-inflammatory diseases were performed. These tests revealed mutation in TNFAIP3, leading to the diagnosis of haploinsufficiency A20 (HA20). In view of this diagnosis, therapy with a biological drug, IL1 receptor antagonist (Anakinra), was started. Results: HA20 is an autoinflammatory disease due to a loss of function of A20 protein, whose role is to down-regulate the proinflammatory pathway of NF-kb. It is autosomal dominant, with earlyonset and with very heterogeneous manifestations even within the same family. This condition is often called Behcet-like, with which it shares some features (oral ulcers, gastrointestinal and skin involvement). Regarded A., the disease appeared at 3 months of age with ulcerative colitis and later skin manifestations started. Probably father and paternal grandfather have the same condition but with milder manifestations. Conclusion: This case taught us that HA20 should be considered in patients with very early-onset inflammatory disease, characterized by colitis as the first manifestation and cutaneous involvement, often with positive family history.