The neurological complications of Behçet's syndrome have not been characterized with clarity. We present the clinical features, imaging characteristics and CSF findings of a series of 50 patients seen at the National Hospital for Neurology and Neurosurgery over the past 10 years. In this series, vascular complications had a low prevalence, whereas involvement of the brainstem was common; spinal cord lesions, hemisphere lesions and meningoencephalitis also occurred. Optic neuropathy, vestibulocochlear and peripheral nerve involvement occurred, but were rare. The prognosis for recovery was in general good, and the majority of those followed-up over a median of 3 years (range 1-19 years) had only single attacks. One-third of patients underwent further attacks, and four underwent progressive deterioration leading to disability. Factors suggesting a poor prognosis are repeated attacks, incomplete recovery, progressive disease course and a high level of CSF leucocytosis during acute attack. These data should be of help in the further definition of the clinical characteristics of this rare neurological disorder and in the planning of treatment trials.
In our population, BD confers a 14-fold risk of developing venous thrombosis. The risk is sixfold higher in male BD patients, who fare worse than females with regard to thrombophlebitis, folliculitis and retinal vasculitis.
describe was stung by the P noctiluca species. Furthermore, to our knowledge, the RCM characteristics of jellyfish sting have not been described to date. In this patient, RCM proved useful to identify the presence of nematocysts in the epidermis, confirming the clinical diagnosis of jellyfish sting and the related persistence of symptoms. Remarkably, in the follow-up visit after topical therapy, the dermoscopic and RCM features appeared dramatically reduced or no longer visible, paralleling the resolution of symptoms and supporting the decision to discontinue treatment.Conclusions | In conclusion, although further studies are needed to validate these observations, in vivo skin imaging may represent an adjunctive and valuable tool to confirm clinical diagnosis and monitor treatment response over time in patients with jellyfish stings.
Conflict of Interest Disclosures: Dr Pomeranz reported being a paid member of the scientific advisory board of Procter & Gamble and receiving royalties from UpToDate. Dr Femia reported receiving royalties from UpToDate. No other disclosures were reported.
Background Patients with systemic lupus erythematosus (SLE) are at risk of developing COVID-19 due to underlying immune abnormalities and regular use of immunosuppressant medications. We aimed to evaluate the presence of SARS-CoV-2 IgG antibodies in patients with SLE with or without previous COVID-19-related symptoms or RT-PCR-confirmed SARS-CoV-2 infection. Methods For this analysis, we included patients with SLE from two cohorts based in New York City: the Web-based Assessment of Autoimmune, Immune-Mediated and Rheumatic Patients during the COVID-19 pandemic (WARCOV) study; and the NYU Lupus Cohort (a prospective registry of patients at NYU Langone Health and NYC Health + Hospitals/Bellevue). Patients in both cohorts were tested for SARS-CoV-2 IgG antibodies via commercially available immunoassays, processed through hospital or outpatient laboratories. Patients recruited from the NYU Lupus Cohort, referred from affiliated providers, or admitted to hospital with COVID-19 were tested for SARS-CoV-2 IgG antibodies as part of routine surveillance during follow-up clinical visits. Findings 329 patients with SLE were included in this analysis, 146 from the WARCOV study and 183 from the NYU Lupus Cohort, and were tested for SARS-CoV-2 antibodies between April 29, 2020, and Feb 9, 2021. 309 (94%) were women and 91 (28%) were of Hispanic ethnicity. 51 (16%) of 329 patients had a positive SARS-CoV-2 IgG antibody test. Seropositive patients were more likely than seronegative patients to be Hispanic (24 [47%] of 51 vs 67 [24%] of 278). Other demographic variables, SLE-specific factors, and immunosuppressant use were not associated with SARS-CoV-2 positivity. Of the 29 patients with COVID-19 previously confirmed by RT-PCR, 18 (62%) were on immunosuppressants; 24 (83%) of 29 patients tested positive for SARS-CoV-2 IgG antibodies. Of 17 patients who had symptoms of COVID-19 but negative concurrent RT-PCR testing, one (6%) developed an antibody response. Of 26 patients who had COVID-19-related symptoms but did not undergo RT-PCR testing, six (23%) developed an antibody response. Of 83 patients who had no symptoms of COVID-19 and no RT-PCR testing, four (5%) developed an antibody response. Among 36 patients who were initially SARS-CoV-2 IgG positive, the majority maintained reactivity serially (88% up to 10 weeks, 83% up to 20 weeks, and 80% up to 30 weeks). Seven (70%) of ten patients with confirmed COVID-19 had antibody positivity beyond 30 weeks from disease onset.Interpretation Most patients with SLE and confirmed COVID-19 were able to produce and maintain a serological response despite the use of a variety of immunosuppressants, providing reassurance about the efficacy and durability of humoral immunity and possible protection against re-infection with SARS-CoV-2.
To the Editor: Lichen sclerosus (LS) is a chronic, inflammatory disorder that may lead to anogenital malignancy and scarring. 1,2 The pathophysiology is poorly understood; although autoantibodies targeting extracellular matrix 1 protein and the basement membrane zone have been identified, experts favor a T-cellemediated pathogenesis. 1,2 Small studies show an association with thyroid disorders, vitiligo, and psoriasis. [1][2][3] Increased risk of vulvar squamous cell carcinoma is also reported, with a lifetime incidence of 0.4% to 6% in women with LS. 4 We sought to determine the prevalence of these commonly cited comorbidities in the largest cohort of women with LS to date.The study population included patients in the IBM MarketScan Databases from 2015 to 2017 with 2 or more independent diagnoses of LS (International Classification of Diseases, 10th RevisioneClinical Modification L90.0). This database comprises more than 21.7 million commercially insured women in the United States. The study population was compared to a control group of women without LS. Stata statistical analysis software version 15.0 (StataCorp, College Station, TX) was used to examine the prevalences of Hashimoto disease/chronic lymphocytic thyroiditis/autoimmune thyroiditis, Graves disease/thyrotoxicosis, vitiligo, psoriasis, and vulvar carcinoma. Odds ratios and 95% confidence intervals were calculated. A P value of \.05 was considered significant.We identified 10,004 women with LS and 21,672,016 female control individuals without LS from 2015 to 2017 in the United States. The mean age was 50.8 years; 79% were between 45 and 65 years old. The rates of comorbid conditions are shown in Table I. In the LS population, the prevalences were as follows: autoimmune thyroid diseases, 6.11%; vitiligo, 1.95%; psoriasis, 5.12%; and vulvar carcinoma, 1.9%. All comorbidities were more likely to occur in patients with LS compared to control individuals (Table II). The most commonly associated autoimmune disease was hypothyroidism, with a prevalence of 4.26%. Notably, vulvar carcinoma was 26 times more likely and vitiligo was 12 times more likely to occur in patients with LS.We identified only 4 studies that examined the rates of similar comorbidities in LS. [1][2][3]5 One comparing 50 patients with LS to 50 control individuals cited an autoimmune disease prevalence This project was accomplished through a generous gift from The Louis and Rachel Rudin Foundation, Inc, which supports the research education of residents at New York University Langone Health's Ronald O. Perelman Department of Dermatology.
Background Vulvar lichen sclerosus (LS) is a chronic dermatosis for which the true prevalence is not well established. LS remains an underrecognized and undertreated disease, and treatment patterns and differences among various specialties that care for patients with LS are largely unknown. Objective This study sought to determine the prevalence of diagnosed vulvar LS, demographic characteristics, and diagnostic and treatment patterns in the United States. Methods A database comprising >21.7 million women with commercial insurance in the United States was analyzed, and the largest cohort of patients with LS in the United States to date was identified. Results We found that obstetricians/gynecologists are diagnosing and managing approximately half of women with LS, and regardless of specialty, the majority of providers are appropriately prescribing high-potency topical steroids. Conclusion Our results suggest that LS may truly be underrecognized and underdiagnosed, especially in younger women.
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