Increased blood group 2 innate lymphoid cells are involved in blood eosinophilia and itching in Kimura disease

Background: Kimura disease (KD) is a rare, chronic inflammatory disorder characterized by blood eosinophilia, general itching, and subcutaneous head and neck mass lesions; however, the etiology of this disease is unknown. We hypothesized that group 2 innate lymphoid cells (ILC2s) in peripheral blood may play an essential role in the pathogenesis of KD. Methods: The prevalence of blood ILC2s and their ability to produce interleukin (IL) -4, IL-5, IL-13, and IL-31 in patients with KD were compared with those in control subjects and in patients with house dust mite (HDM) -induced allergic rhinitis (AR). Changes in blood ILC2 prevalence, blood eosinophilia, and clinical symptoms after surgery and steroid therapy were evaluated. Results: Blood ILC2 prevalence in patients with KD were eight times and six times higher than those in control subjects and in patients with AR, respectively. There was a strong positive correlation between ILC2 prevalence and blood eosinophilia. Patients with KD showed increased serum IL-13 and decreased IL-31 levels. KD patient-derived blood ILC2s produced large amounts of IL-5 and IL-13 in response to prostaglandin (PG) D and leukotriene (LT) D , compared to ILC2s derived from control subjects and patients with AR. Surgery and systemic steroid therapy ameliorated general itching with a concomitant decrease in blood ILC2s and blood eosinophilia. Upon disease recurrence, blood ILC2 prevalence and blood eosinophilia increased concurrently with general itching. Conclusion: Increased blood ILC2s may be involved in blood eosinophilia and general itching through the production of IL-5 and IL-13 in patients with KD.

Outcomes of Patients With Acute Vogt–koyanagi–harada Disease Treated With Intravenous Corticosteroid Pulse Followed by the Slow Tapering of Oral Corticosteroid Therapy

Purpose We investigated the treatment outcomes of patients with acute Vogt–Koyanagi–Harada (VKH) disease and assessed the differences between patients with no inflammation worsening and those with persistent or worsening inflammation. Potential factors responsible for eyes with low visual outcomes were also investigated.Methods We retrospectively reviewed the clinical records of patients with acute VKH disease who first visited us between 2009 and 2018 and were followed up for >300 days. Clinical characteristics, treatments, and posttreatment conditions were assessed. Patients were classified into no inflammation worsening (acute–resolved [AR]) and inflammation worsening (chronic–recurrent [CR]) groups based on conditions after 6 months from disease onset.Results This study assessed 62 eyes from 31 patients (mean age: 52.8 years). One patient was treated with topical treatment alone and showed poor visual outcomes. In total, 30 patients were treated with methylprednisolone pulse followed by the slow tapering of oral prednisolone; 73% of them developed AR and 27% CR. Although the total prednisolone dose was higher in patients with CR disease, no significant difference was noted in the final best-corrected visual acuity (BCVA). Among the patients receiving systemic steroid, five eyes had a final BCVA of ≤0.5 due to anisometropic amblyopia, diabetic maculopathy, pre-existing macular hole, epiretinal membrane, and ellipsoid zone loss. Conclusions Patients with acute VKH disease treated with corticosteroid pulse followed by the slow tapering of prednisolone appear to demonstrate good visual outcomes, including patients with CR; most eyes with low visual outcomes have pre-existing conditions that explain low vision.

Azzopardi Phenomenon in a Non-neoplastic Phthisical Eye: A Case Report

Objective: The Azzopardi phenomenon, known as the deoxyribonucleic acid deposition on various structures due to cellular necrosis, has never been reported in non-neoplastic eyes. Methods: We report a case of a 48-year-old man who had congenital nystagmus with poor vision in both eyes, presented with decreased vision and photophobia in his left eye. An exudative retinal detachment was found, which did not respond to systemic steroid treatment. Glaucoma due to occlusio pupillae was later developed. Laser iridotomy and anti-glaucoma medications decreased intraocular pressure to an acceptable level. Vision in the left eye gradually deteriorated during the 10-year clinical course. Evisceration was finally performed due to persistent dull aching ocular pain along with signs of ocular hypotony Results: Histopathological examination showed phthisis bulbi and focal nodular retinal gliosis. The Azzopardi phenomenon was found at the retinal vessel walls, within the retinal layers and along the internal limiting membrane. There was neither evidence of intraocular tumors nor foreign bodies. Conclusion: This case demonstrated that the Azzopardi phenomenon could be present in a non-neoplastic eye with a longstanding disease that proceeds to phthisis bulbi.

Assessment of Clinical Asthma Score and Asthma Severity Score in Preschool Children with Recurrent Wheezing

Objective: There is no definitive consensus on asthma exacerbation scoring for preschool children with recurrent wheezing. The Clinical Asthma Score (CAS) and Asthma Severity Score (ASS) are two scoring systems that can be used in this population. The aim of this study was to evaluate the relationship between CAS and ASS, acute treatment, and exacerbation outcomes in preschool children with wheezing. Materials and Methods: The study included 70 patients aged 2-5 years who presented to the pediatric emergency department due to an acute wheezing episode. CAS and ASS were evaluated at exacerbation presentation and after initial salbutamol therapy. Results: Presenting scores were significantly higher among patients who had three or more episodes within the last year (p=0.01 for CAS, p=0.019 for ASS). Presenting scores were significantly higher in patients treated with systemic steroid therapy during the episode compared to those who were not (p=0.006 for CAS; p=0.003 for ASS). CAS and ASS predicted the use of acute steroid therapy with a sensitivity of 73.7% and 52.6%, and predicted hospitalization with a sensitivity of 95% and 82.5%, respectively. Conclusion: Our data suggest that these scoring systems can be used to judge the need for systemic steroid therapy and that high scores are associated with greater likelihood of hospital admission. Keywords: Asthma score, children, hospitalization, wheezing, exacerbation

Antituberculosis Drug-induced Linear IgA Dermatosis

Introduction: Linear IgA dermatosis is a rare autoimmune vesiculobullous disease characterized by homogeneous linear IgA deposits in basement membrane of epidermis, and it can be idiopathic or drug-induced. The pathogenesis of drug-induced linear IgA dermatosis is not fully known yet, but it is associated with specific T cells. The clinical manifestations of the disease include vesiculobullous eruption, erythematous plaques, or string of pearls. Most cases still need additional therapy to avoid the expansion of the disease. Case Presentation: In this study, we present a 17-year-old male patient with erythema plaques, vesicles, and bullae with erosion in facial, oral, neck, trunk, genital, and extremities, pruritus, and burning sensation. The patient was undergoing pulmonary tuberculosis (TB) treatment for one week. Physical examination was done, and total BSA 10% and negative Asboe-Hansen sign were seen. The treatment consisted of delaying administration of TB drugs, desoximetasone cream 0.25%, cetirizine 10 mg, and aspiration of bullae. Conclusions: Drug-induced linear IgA dermatosis can occur at any age due to the administration of rifampicin and other antibiotics, angiotensin-I converting enzyme (ACE) inhibitors, or nonsteroidal anti-inflammatory drugs (NSAIDs). The drug can stimulate specific T cells that release Th2 cytokines to produce IgA antibodies against the basement membrane of epidermis. Drugs may cause an autoimmune response by cross-reaction with the target epitope, altering the conformation of epitopes, or exposing previously sequestered antigens to the immune system. The causative drug was stopped, and methyl prednisolone 0.5 - 1 mg/kg/day was given as initial therapy. In this study, we reported a rare case of a 17-year-old male with anti-TB drug-induced linear IgA dermatosis. Diagnosis was done based on clinical manifestation, histopathology, and immunofluorescence. The causative drug was stopped, the patient was given topical and systemic steroid therapy and drug desensitization. Remission was noted after six weeks of therapy, and oral steroid was slowly tapered and stopped on day 42. After stopping oral steroids, no lesions were reported. A 6-month follow-up revealed no signs of recurrence.

Sensorineural Hearing Loss of Suspected Autoimmune Etiology: Two Cases of Cogan’s Syndrome

Cogan’s syndrome is a rare inflammatory disease characterized by non-syphilitic keratitis and vestibulo-auditory symptoms including hearing loss, tinnitus, and vertigo. Although its precise pathogenesis is not known, Cogan’s syndrome is generally considered an autoimmune disease. This hypothesis is supported by the frequently successful remission of hearing loss after steroid administration and the association with other autoimmune disorders such as rheumatoid arthritis. Medical treatment of Cogan’s syndrome depends on disease severity and on how extensive the disease is. The involvement of inner ear pathology requires systemic corticosteroid therapy. In cases of treatment failure or the need for a corticosteroid-sparing effect, other immunosuppressive drugs can be used. We experienced two patients with typical Cogan’s syndrome, presenting bilateral progressive sensorineural hearing loss and dizziness with ocular involvement, which we have successfully treated with systemic steroid administration and immunosuppressive therapy.

A gyógyszeres kezelésre nem javuló hirtelen halláscsökkenés lépcsőzetes sebészi terápiája

Összefoglaló. A hirtelen halláscsökkenés patofiziológiája még nagyrészt tisztázatlan, így oki terápia nem lehetséges. Az elsődleges kezelést a helyileg vagy szisztémásan adott kortikoszteroid jelenti, egységes protokoll azonban nem áll rendelkezésre. Nagy vagy súlyos fokú hirtelen halláscsökkenés esetén kóroki tényezőként felmerül a perilymphafistula lehetősége még azoknál a betegeknél is, akiknél nem szerepel trauma az anamnézisben. A kórkép műtéti kezelése a dobüreg feltárását követően a belső fül ablakainak obliterálása. Amennyiben ez a megoldás nem eredményez megfelelő hallásjavulást, hagyományos vagy implantálható hallókészülékek alkalmazása javasolt. A közleményben részletezett esetünkben teljes siketséggel járó, jobb oldali hirtelen halláscsökkenés alakult ki, melynek hátterében egyértelmű okot azonosítani nem sikerült. Az eredménytelen kombinált, intratympanalis és szisztémás szteroidkezelést követően exploratív tympanotomiát végeztünk, melynek során a belső fül ablakait obliteráltuk. Hallásjavulást ezt követően sem sikerült kimutatni, így cochlearis implantáció elvégzése mellett döntöttünk. Az implantációt a kerek ablakon keresztül végeztük, mely alapján kijelenthetjük, hogy az előzetes kerekablak-obliteráció nem zárja ki a későbbi cochlearis implantációt. Orv Hetil. 2021; 162(51): 2055–2060. Summary. The pathophysiology of sudden sensorineural hearing loss is mainly unknown, therefore no causative treatment exists. Systemic and local administration of corticosteroids serves as first line therapy although protocols vary. In cases of severe or profound hearing loss with no improvement for medical therapy, perilymphatic fistulae can be assumed even without any history of trauma. Therefore, inner ear window obliteration as a primary surgical option in the early stage can be considered. For patients without complete recovery, conventional hearing aids or implantable hearing devices can be offered. In our case report, we present a patient with right sided idiopathic sudden deafness. After failure of conservative combined intratympanic and systemic steroid therapy, explorative tympanotomy and obliteration of the inner ear windows were performed. As no hearing improvement was witnessed, successful cochlear implantation via round window insertion was performed. Our case justifies that obliterating the round window membrane does not rule out further successful cochlear implantation. Orv Hetil. 2021; 162(51): 2055–2060.

Idiopathic Chiasmal Optic Neuritis in a Pediatric Patient

Purpose: We report a case of idiopathic chiasmal optic neuritis in a pediatric patient.Case summary: A 13-year-old boy with no history of systemic disease was referred to our ophthalmology clinic because of visual disturbance in both eyes of 5 days in duration. The best-corrected visual acuity was 0.08 in the right eye and finger counting at 30 cm in the left eye; mild blurring of the disc margins (both eyes) was evident on fundus examination, as were temporal hemianopsia in the right eye and diffuse field loss in the left eye. Brain magnetic resonance imaging revealed focal nodular enhancement in the optic chiasm. Blood and cerebral fluid analysis yielded no evidence of infection or autoimmune disease. Therefore, we diagnosed isolated idiopathic chiasmal optic neuritis and commenced a systemic steroid. After 5 days, the visual acuity began to improve, and the field defect was almost eliminated (except for a small central scotoma) at 1 month. He has remained stable to the time of writing (4 months after treatment) and his visual acuity has normalized.Conclusions: Isolated idiopathic chiasmal optic neuritis developed in a pediatric patient and the visual function improved after steroid treatment. Although there was no evidence of systemic demyelinating disease, regular observation is scheduled given the possibility of late-onset disease.

Evaluation of drug use heath related quality of life and pharmacoeconomics in autoimmune skin disorders: focus on blistering skin disorders-a prospective observational study

Background: Autoimmune skin disorders (ASDs) are complex diseases triggered by autoantibodies action against epidermal antigens or the dermo epidermal junction. Although rare, they present high morbidity, affecting the quality of life (QoL) of patients and financial status of patient.Methods: This prospective, observational study was carried out in department of dermatology for 2-3 months after ethical approval. Drug usage pattern, heath related QoL (HRQOL) by using DLQI (Dermatology life quality index) and cost were evaluated in patients with ASDs. Statistical analysis was done using Microsoft excel office 2019 and rechecked with SPSS (version 23.0). P<0.001 was considered as statistically significant.Results: Out of 73 patients enrolled, 32 were male and 41 were female with the mean age was 48.27±14.93 years; 55% patients had autoimmune blistering skin disorders (AIBDs) and 45% having other ASDs (OADs). Pemphigus vulgaris (PV) (35%) being the most common among all ASDs. Systemic steroid (60.27%), topical steroid (79.45%), levocetirizine (63%) were most commonly prescribed drugs. Mean DLQI score at baseline and after treatment was 11.64±2.49 and 6.8±2.75 respectively. It was highly significant statistically (p<0.0001). Total cost of illness per month was 813.64±481.21 INR. Maximum percentage variation in cost was seen with prednisolone (1706.28%).Conclusions: ASDs have a female bias and inflict severe impairment to the QoL of patients. Appropriate drug therapy with corticosteroids and other adjuvant drug lead to positive impact on QoL. There was very wide price variation of different brands of the same generic most commonly prednisolone and levocetirizine.

Successful induction treatment of bullous pemphigoid using reslizumab: a case report

Background Bullous pemphigoid (BP) is a potentially life-threatening autoimmune blistering disease which is characterized by autoantibodies against hemidesmosomal proteins of the skin and mucous membranes. In recent years, the role of eosinophil and immunoglobulin E autoantibodies have been further elucidated in BP, and have been considered potential therapeutic targets. Case presentations A 67-year-old male presented with erythematous bullous eruption. The skin eruption was located on whole body, and suggested BP. Peripheral blood eosinophil count and total immunoglobulin E markedly elevated in initial laboratory findings. Topical and systemic steroid (methylprednisolone 2 mg/kg/day) treatment was started, and his skin symptoms worsened repeatedly, whenever systemic steroid were reduced. On admission day 29, reslizumab (anti-interleukin-5) 3.5 mg/kg was administered intravenously to the patients. The bullous skin lesion began to improve rapidly, and methylprednisolone (8 mg/day) was reduced without any worsening of symptoms during two doses of reslizumab. Conclusions We report a case of successful treatment response to reslizumab administration in a patient with BP. Further studies are needed to confirm the role of anti-interleukin-5 as a treatment for BP in the future.