Urticarial vasculitis is an eruption characterized by inflamed itchy or painful red papules or plaques that resemble urticaria but last longer than 24 hours and heal with residual pigmentation or purpura. Histopathologically, urticarial vasculitis presents as leukocytoclastic vasculitis with perivascular infiltrate and fibrin deposits. The treatment options are oral antihistamines, oral corticosteroids, dapsone, colchicine and hydroxychloroquine. We report four cases with normocomplementemic urticarial vasculitis who were treated with omalizumab and a brief review of the literature on the use of omalizumab in normocomplementemic urticarial vasculitis.
Introduction: Chronic urticaria (CU) is a common disease that primarily affects females. Although commonly encountered, there is little data on how pregnancy interact with CU. We analysed treatment use by CU patients before, during and after pregnancy as well as outcomes of pregnancy. Material and Methods: PREG-CU was a prospective and retrospective, international, multicentre, study of the Urticaria Centers of Reference and Excellence (UCARE) network. Data were collected via a 47-item-questionnaire completed by CU patients who became pregnant during their disease course. Results: Questionnaires from 288 CU patients from 13 countries were analysed. During pregnancy, most patients (60%) used urticaria medication (standard dose second generation H1-antihistamines [SD-sgAH] (35.1%), first generation H1-antihistamines (7.6%), high-dose sgAH (5.6%), and omalizumab (5.6%). The preterm birth rate was 10.2%, and preterm rates of patients who did and did not receive treatment during pregnancy were similar (11.6% vs 8.7%). Emergency referrals for CU and twin birth were risk factors for preterm birth. The caesarean delivery rate was 51.3%. More than 90% of new-borns. were healthy at birth. There was no link between any patient or disease characteristics or treatments and medical problems at birth. Eight of 10 (78.8%) women with CU breastfed their babies and most (54.3%) used urticaria medication while breastfeeding. Conclusion: Rates of preterm births and medical problems of new-borns in patients with CU were similar to population norms and not linked to treatment used during pregnancy. Emergency referrals increase risk of preterm birth and emphasizes the importance of urticaria control during pregnancy.
Recurrent Angioedema (RAE) is characterized by sudden swelling of mucosal surfaces or deep dermis and is either mast cell-(MMAE) or bradykinin-mediated (BMAE). How patients with BMAE and MMAE differ in terms of disease activity and impact remains largely unknown. Here, we determined validity, reliability, and sensitivity to change of Turkish versions of angioedema activity score (AAS) and quality of life questionnaire (AE-QoL) and used both instruments to investigate and compare patients with BMAE and MMAE.Turkish versions of AAS28 and AE-QoL were applied to 94 patients with RAE (18-72 years). Patients' global self-assessment of QoL (PGA-QoL), disease activity (PGA-DA-VRS, PatGA-DA-VAS), and 12-Item-Short Form Survey were used at week 4 (visit 2), and week 8 (visit 3). Demographic characteristics, clinical features, and AAS28 and AE-QoL values were compared between 31 patients with BMAE and 63 patients with MMAE.Turkish AAS28 and AE-QoL showed excellent internal consistency, high reproducibility and known-groups validity. Compared to patients with MMAE, BMAE patients were younger (34.6 ± 10.7 vs. 40.7 ± 13.3 years), had longer disease duration (236 ± 178 vs. 51 ± 78 months), high prevalence of family history (63% vs 14%), longer duration of attacks (65 ± 20 vs. 40 ± 25 h), and they were more commonly affected by upper airway angioedema (70% vs 23%). Disease activity (AAS28) was lower (29.3 ± 24.6 vs 55.2 ± 52.9), but AE-QoL was higher (44.2 ± 16.1 vs 34.5 ± 22.5) in BMAE patients as compared to MMAE patients.Patients with BMAE and MMAE have distinct disease characteristics. Recurrent bradykinin-mediated angioedema impacts quality of life more than mast cell-mediated angioedema. The discriminating characteristics of patients with BMAE and MMAE may help to improve the diagnosis and management of patients with RAE.
Low and high IgE is linked to improvement and worsening of chronic urticaria during pregnancy, respectively Kocatürk E, Thomsen SF, Al-Ahmad M, Gimenez-Arnau A, Conlon N, Savk E, Criado RF, Danilycheva I, Fomina D, Khoshkhui M, Gelincik A, Degirmentepe EN, Demir S, Ensina LF, Kasperska-Zajac A, Rudenko M, Bauer A, Medina I, Maurer M.1 Urticaria Center of Reference and Excellence (UCARE), Dept. of Dermatology, Koç University School of Medicine, Istanbul, Turkey ekocaturk@ku.edu.tr2 Urticaria Center of Reference and Excellence (UCARE), Center of Reference and Excellence (UCARE), Department of Dermatology, Bispebjerg Hospital, Copenhagen, Denmark simonfrancisthomsen@gmail.com3 Urticaria Center of Reference and Excellence (UCARE), Microbiology Department, Faculty of Medicine, Kuwait University, Safat, Kuwait monaalahmad@yahoo.com4 Urticaria Center of Reference and Excellence (UCARE), Department of Dermatology, Hospital del Mar, IMIM, Universitat Autònoma, Barcelona, Spain anamariagimenezarnau@gmail.com5 Urticaria Center of Reference and Excellence (UCARE), Dermatology, and Immunology, St James’s Hospital, Dublin, Ireland conlonn1@tcd.ie6 Aydın Adnan Menderes University, Aydın, Turkey esavk@adu.edu.tr7 Urticaria Center of Reference and Excellence (UCARE), Faculdade de Medicina do ABC (FMABC), Santo André, Brazil roberta.criado@fmabc.br8 Urticaria Center of Reference and Excellence (UCARE), NRC Institute of Immunology FMBA of Russia, Moscow, Russia ivdanilycheva@mail.ru9 Urticaria Center of Reference and Excellence (UCARE), First Moscow State Medical University, Moscow Center of Allergy and Immunology , Clinical Hospital 52 , Ministry of Moscow Healthcare, Moscow, Russia daria.s.fomina@gmail.com10 Urticaria Center of Reference and Excellence (UCARE), Allergy Research Center, Mashhad University of Medical Sciences, Mashhad, Iran Khoshkhuim@mums.ac.ir11 Urticaria Center of Reference and Excellence (UCARE), Istanbul Faculty of Medicine Istanbul University, Istanbul, Turkey gelincikasli@hotmail.com12 Urticaria Center of Reference and Excellence (UCARE), Okmeydani Training and Research Hospital, Istanbul, Turkey ecenuryksel@gmail.com13 Urticaria Center of Reference and Excellence (UCARE), Istanbul Faculty of Medicine Istanbul University, Istanbul, Turkey ERTANSEMRA@yahoo.com14 Urticaria Center of Reference and Excellence (UCARE), Federal University of São Paulo, Sao Paulo, Brazil 100alergia@gmail.com15 Urticaria Center of Reference and Excellence (UCARE), European Center for Diagnosis and Treatment of Urticaria (GA2LEN UCARE Network) Medical University of Silesia in Katowice, Poland alakasperska@gmail.com16 Urticaria Center of Reference and Excellence (UCARE), The London Allergy & Immunology Centre, London, United Kingdom consultation@ukallergy.com17 Urticaria Center of Reference and Excellence (UCARE), Department of Dermatology, University Allergy Center, University Hospital Carl Gustav Carus, Technical University Dresden, Germany. Andrea.Bauer@uniklinikum-dresden.de18 Urticaria Center of Reference and Excellence (UCARE), the Centro Médico Vitae, Buenos Aires, Argentina irisvmedina@gmail.com19 Urticaria Center of Reference and Excellence (UCARE), Dermatological Allergology, Allergie-Centrum-Charité, Dept. of Dermatology and Allergy, Charité – Universitätsmedizin Berlin, Germany marcus.maurer@charite.deDear Editor,PREG-CU, the recent study on pregnancy and chronic urticaria (CU) by the Urticaria Centers of Reference and Excellence (UCAREs), showed that CU improves in half (51.1%) of patients during pregnancy, whereas 28.9% and 20% of patients, respectively, experienced worsening and no change. Low disease activity, no angioedema, and no treatment before pregnancy were risk factors for worsening during pregnancy (1).We hypothesized that patients with chronic spontaneous urticaria (CSU) that worsens during pregnancy are more likely to have type I autoimmune CSU, also called autoallergic CSU. We also hypothesized that patients who improve during pregnancy are more likely to have type IIb autoimmune CSU (2). This hypothesis is supported by the immunological changes observed during pregnancy, i.e., decreased Th1 and Th17 immunity and a switch to a Th2-type cytokine profile (3).To test this hypothesis, we retrieved total IgE levels of CSU patients who gave consent to be included in the PREG-CU study (1). Elevated IgE levels have been reported to be linked to autoallergic CSU, whereas low IgE is a marker of type IIb autoimmune CSU (4).Total IgE blood levels were available for 115 of the 218 CSU patients not treated with omalizumab enrolled in PREG-CU. The median IgE level was 106 (range: 3-1664 IU/mL), more than half of patients (51.3%) had elevated IgE (≥100 IU/mL), and 17.4% had low IgE (<40 IU/mL). Most patients with mild disease (51%) or moderate disease (61%), but only one in four patients with severe disease (26%) had elevated IgE levels (≥100 IU/mL). IgE levels were lower in patients with severe disease (68 IU/mL) vs mild (112 IU/mL; p=0.009) or moderate disease (128 IU/mL; p=0.018), and low IgE levels (<40 IU/mL) were more frequent in patients with severe than mild disease (36.8 vs 11.6%; p=0.034).CSU patients who got worse during pregnancy had higher IgE levels (154 vs. 82.2 IU/mL; p=0.033) and numerically higher rates of elevated IgE (57.5 vs. 46%) compared to patients who got better during pregnancy. In contrast, patients who improved during pregnancy more often had low IgE levels than patients who deteriorated (22 vs. 12.5%), but this was not statistically significant. One in three of our patients (34.9%) had elevated anti-TPO, another marker of type IIb autoimmune CSU, but this was not linked to improvement during pregnancy.Worsening of CSU during pregnancy in patients with high IgE levels may be explained, in part, by the role that IgE and Th2 immunity play in the pathogenesis of their CSU. High IgE, in CSU, has been linked, in some studies, to autoallergy, characterized by the presence of IgE autoantibodies (5). Pregnancy skews immunity towards Th2 responses and patients with Th2-driven diseases, including allergies, often experience worsening of their disease during pregnancy (3). Improvement of CSU during pregnancy in patients with low IgE may point to a role of Th1 and Th17 cytokines in the pathogenesis of their disease. Low IgE is a type IIb autoimmune CSU marker, which is linked to Th1 and Th17 autoimmunity (6). Pregnancy decreases Th1/Th17 immunity, and patients with TH1/TH17-driven autoimmune diseases often experience improvement during pregnancy (3). Our finding that elevated IgG-anti-TPO, another marker of type IIb autoimmune CSU, is not linked to CSU improvement during pregnancy remains unexplained. Many CSU patients with IgG-anti-TPO also have IgE-anti-TPO and vice versa, which could point to both autoallergic and autoimmune drivers of their CSU. Better biomarkers are needed to identify which patients have autoallergic CSU, autoimmune CSU, both or none of these.Our findings support the notion that CSU is a heterogeneous disease, with at least two endotypes, i.e., autoallergic and autoimmune. Further studies are needed to better characterize the course of disease during and after pregnancy, in patients with autoallergic CSU and with autoimmune CSU. IgE levels may help to predict which CSU patients get worse and which improve when they get pregnant.
Abstract Background Chronic spontaneous urticaria (CSU) is a common disease both in the pediatric and in the adult population. However, there are differences between the two patient populations with respect to etiological factors, comorbidities, and treatment responses. Our aim was to determine differences between pediatric and adult CSU in terms of clinical characteristics, laboratory parameters, comorbidities, response to treatment, and indicators of response. Methods A retrospective analysis of CSU patients was performed. Data regarding differences between pediatric and adult CSU patients were analyzed. Indicators of treatment response were determined separately in both pediatric and adult patients. Results Of 751 CSU patients (162 pediatrics and 589 adults), female dominancy (48.8% vs. 69.6%) and rate of angioedema (19.1% vs. 59.8%) were lower, and disease duration (5 months vs. 12 months) was shorter in pediatric patients. Anti‐TPO positivity (24.7% vs. 9%), elevated CRP (46.5% vs. 11.1%), eosinopenia (38.5% vs. 18.1%), and skin prick test positivity (39.3% vs. 28.8%) were significantly more frequent in adult patients. Response to antihistamines was higher in the pediatric group, and only 7% used omalizumab versus 20.8% in the adults. The comparisons were also performed between <12‐year and ≥12‐year patients and yielded similar results. Conclusion Pediatric CSU shows distinct characteristics such as lower incidence of angioedema and antithyroid antibodies, and it responds better to antihistamines. These suggest that CSU becomes more severe and refractory in adolescents and adults. Adolescent CSU shows features similar to adult CSU rather than pediatric CSU.
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