Maculopapular (Exanthematous) Drug Eruption and DRESS

Maculopapular (exanthematous) drug eruption (MPE), also called morbilliform or maculopapular drug eruption, is the most common type of drug hypersensitivity reaction. They are characterized by a diffuse and symmetric eruption of erythematous macules or small papules occurring approximately one week or, in previously sensitized individuals, as early as one or two days after the initiation of drug treatment. Drug reaction with eosinophilia and systemic symptoms (DRESS) is a rare, potentially life-threatening, drug-induced hypersensitivity reaction that includes skin eruption, hematologic abnormalities (eosinophilia, atypical lymphocytosis), lymphadenopathy, and internal organ involvement (liver, kidney, lung). DRESS is characterized by a long latency (two to eight weeks) between drug exposure and disease onset, a prolonged course with frequent relapses despite the discontinuation of the culprit drug. This topic will discuss the terminology, epidemiology, etiology and risk factors, pathogenesis and histopathology, clinical presentation and course, diagnosis and differential diagnoses, management, prognosis, and prevention of both MPE and DRESS

Retrospective Analysis of Autoimmune Diseases and Immunologic Characteristics of the Adult Primary Immune Deficiency Cohort: 17 Years Experience of the Tertiary Referral Immunology Center in Turkey

Objective: Primary immunodeficiencies (PIDs) consist of genetically heterogeneous disorders. The spectrum can include infectious diseases, malignancy, allergy, and autoimmunity. We aimed to analyze the frequency and variety of autoimmune diseases (ADs) in PIDs and describe their clinical and laboratory features. Materials and Methods: Ninety-two patients with PID followed by Ege University Medical Faculty between 2000 and 2017 were enrolled in this retrospective, cross-sectional study. All patients' medical records were reviewed using the demographic information, type of PIDs and ADs, ADs-related autoantibodies, and basic and immunologic laboratory findings. ADs were diagnosed using clinical and complementary paraclinical findings by an immunologist and/or a subspecialist related to the affected organ or system. Results: We evaluated 50 male and 42 female PID patients with a mean age of 40.92 (18-86). Twenty-nine (32 %) patients (15 females/14 males) with a mean age of 43.8 (19-78) had ADs. In our study group, the most commonly detected type of PID with AD is common variable immune deficiency (CVID) (n=17); followed by combined immune deficiency (CID) (n=3), CTLA4 deficiency (n=2), selective IgA deficiency (sIgAD) (n=2), specific IgG subgroup deficiency (n=1), autoimmune polyglandular syndrome (APS) with hypogammaglobulinemia (n=1), dyskeratosis congenita (DC) (n=1), Osler-Rendu-Weber (ORW) syndrome with CVID-like PID (n=1), and cartilage-hair hypoplasia (CHH) (n=1). According to systematic assessments, ADs resulted in endocrinologic 14%, dermatologic 10.8%, rheumatologic 9.7%, gastroenterological 9.7%, hematological 8.6%, and neurologic disorders 1%. The frequency of ADs was higher in CVID cases than other types of PIDs (p <0.05). Basic and immunologic laboratory findings of the PIDs with and without ADs were analyzed and compared; however, no statical significant difference was obtained between the groups. Conclusion: We have analyzed the frequency and variety of ADs in a adult PID cohort in Turkey. Patients presenting with multiple ADs should be screened for having an underlying PID

COVID-19 in patients with primary and secondary immunodeficiencies: a single-center experience

[No Abstract Available

CoronaVac/Sinovac COVID-19 Vaccine-Related Hypersensitivity Reactions and Second-Dose Vaccine Administration: Tertiary Allergy Center Experience

Introduction: The COVID-19 pandemic has caused a global health crisis. To prevent the disease, the Ministry of Health of Turkey gained approval for the CoronaVac COVID-19 vaccine for emergency use as the first-line. This study aimed to evaluate patients who developed hypersensitivity reactions (HRs) due to the CoronoVac vaccine and to share our experience of administering the second dose of vaccine to these patients. Methods: The study group included the patients who presented to the Ege University Allergy and Immunology Division between January and May 2021. Demographic data, atopic status, allergic reactions to the first dose of the COVID-19 vaccine and the route of second-dose vaccine administrations were recorded. Results: A total of 7 patients (four healthcare professionals), 6 (86%) of whom were women, with an average age of 53.4 years, were included in the study. The rate of allergic reactions among Ege University health workers was 0.036% (2/5,558). Six of our patients had a history of additional allergic diseases and comorbid diseases. None had any allergic reactions to previous vaccinations and latex allergy. Reactions developed commonly on the skin, as generalized urticaria/angioedema and pruritus. The severity of the reactions was evaluated as mild in 2, moderate in 3, and severe in 2 cases. The second-dose CoronaVac was safely administered by using a gradually increase dose in a total of 6 patients. Conclusion: In patients with HRs due to Sinovac in the first dose, the second dose can be safely performed using a gradually increased dose

The Prevalence of Hypersensitivity Reactions to Antirheumatic Biological Agents and Results of the Skin Tests: Experience of a Tertiary Referral Allergy Center in Turkey

WOS:000603358700003Objective: The use of biological agents (BAs) has increased dramatically for inflammatory diseases and this increase has led to a rise in hypersensitivity reactions (HSRs). The symptoms and diagnostic tools for HSRs are not standardized. We aimed to analyze the prevalence of HSRs to anti-rheumatic BAs and to evaluate the usefulness of skin tests (STs) as a diagnostic tool. Materials and Methods: Our study was conducted at the Ege University Medical Faculty, Department of Internal Medicine, Division of Allergy and Clinical Immunology and Division of Rheumatology, Izmir, Turkey. Four hundred sixty patients who received BAs between Jan 1st, 2015, and Jan 1st, 2016, were reviewed in this retrospective cross-sectional study. The prevalence of HSRs was retrospectively evaluated. Ten patients with HSRs were evaluated with STs containing commercially available culprit drugs. The data was collected from hospital records. The age, sex, atopic diseases, primary rheumatic diseases, and anti-rheumatic therapies of the patients were recorded. Results: Two hundred fifty patients were treated with rituximab (RTX), 45 with infliximab, 40 with tocilizumab (TOC), 36 with golimumab, 35 with etanercept, 34 with abatacept, 15 with certolizumab, and 5 with adalimumab. Fifty reactions with RTX and two reactions with TOC were infusion-related (IRRs). Ten HSRs were observed. Eight patients had immediate (7 immediate systemic reactions and 1 local injection site reactions), and 2 had late-onset cutaneous reactions. We detected the ratio of IRRs as 11.3%, immediate HSRs as 1.73%, IgE-mediated reactions as 1.08%, and anaphylaxis as 0.86%. STs were positive in 5 of 8 patients with immediate HSRs. Four of them had anaphylaxis, and remarkably, 3 of these had positive STs. None of the ten patients had high levels of specific IgE and only four had atopic diseases. Total IgE levels were not high and specific IgE levels were not positive in the presence of HSR to BAs (p=0.039). Conclusion: Five of the 8 (62.5%) patients with immediate reactions had positive STs, which suggested IgE-mediated reactions. The prevalence of HSRs to BAs was less than the ones mentioned in the literature

Successful Rapid Drug Desensitization with A Modified Protocol To Alemtuzumab in A Multiple Sclerosis Patient with Severe Immediate-Type Hypersensitivity Reaction

Alemtuzumab is a humanized monoclonal antibody targeting the CD52 antigen on lymphocyte surfaces. The intravenous administration of alemtuzumab provokes the depletion of lymphocytes by antibody-dependent and complement-mediated cellular cytotoxicity. Resulting cytotoxicity leads to 'first-dose infusion-related reactions in more than 90% of the patients, fewer than 3% being severe cases. We present the first successful modified rapid drug desensitization (RDD) protocol to alemtuzumab in an active relapsing-remitting multiple sclerosis (RRMS) patient. The forty-year-old female patient had an immunologically-mediated mixed-type (co-occurring IgE-mediated and cytokine release syndromes) hypersensitivity reaction (HSR) verified with a drug skin test. As the patient had severe HSR and there was no other option to treat RRMS at that time; two courses of 12 mg alemtuzumab with one-year intervals were administrated successfully using the modified 12-step intravenous RDD protocol. By experience, RDD is known as a safe and effective therapy option allowing alemtuzumab treatment targeted for the aforementioned type of MS

In Vitro Fertilization Using Luteinizing Hormone-Releasing Hormone Injections Resulted in Healthy Triplets without Increased Attack Rates in a Hereditary Angioedema Case

WOS: 000426177100001PubMed ID: 29666724Hereditary angioedema due to C1-inhibitor deficiency (C1-INH-HAE) is a rare, autosomal dominant disorder. The management of pregnant patients with C1-INH-HAE is a challenge for the physician. Intravenous plasma-derived nanofiltered C1-INH (pdC1INH) is the only recommended option throughout pregnancy, postpartum, and breast feeding period. In order to increase pregnancy rates, physicians use fertilization therapies increasing endogen levels of estrogens. Therefore, these techniques can provoke an increase in the number and severity of edema attacks in C1-INH-HAE. Our patient is a 32-year-old female, diagnosed with C1-INH-HAE type 1 since 2004. She had been taking danazol 50-200 mg/day for 9 years. Due to her pregnancy plans in 2013, danazol was discontinued. PdC1INH was prescribed regularly for prophylactic purpose. Triplet pregnancy occurred by in vitro fertilization using luteinizing hormone-releasing hormone (LHRH) injections. In our patient, LHRH injections were done four times without causing any severe attack during in vitro fertilization. Angioedema did not worsen during pregnancy and delivery due to the prophylactic use of intravenous pdC1INH in our patient. According to the attack frequency and severity, there was no difference between the three pregnancy trimesters. To our knowledge, this is the first published case of C1-INH-HAE receiving in vitro fertilization therapies without any angioedema attacks during pregnancy and delivery and eventually having healthy triplets with the prophylactic use of intravenous pdC1INH

Immediate and Late Onset Forms of Insulin Hypersensitivity Presenting with Glucose Dysregulation

WOS: 000452210500009We report the case of a 35-year-old woman allergic to detemir, neutral protamine Hagedorn, and glargine. Initially, local reactions to the insulin preparations occurred, which continued even after the types of insulin and the application areas were changed. When the use of insulin therapy was continued, the local reactions developed into systemic forms. Interestingly, blood glucose levels kept increasing to uncontrolled levels every time the cutaneous reactions occurred. The patient was referred to our clinic for further investigation. The results of the skin prick tests with insulin preparations were negative; however, the intradermal test results were positive with the following dilutions of the insulin preparations: 1/100 detemir, 1/100 glargine, 1/1.000 neutral protamine Hagedorn, and 1/1.000 regular insulin. The intradermal skin test results for glulisine, aspart, and lispro were negative. The levels of immunglobulin E specific to human insulin were high (194 kU/L; N 0-87 kU/L); whereas, the specific immunglobulin G4 levels were normal (35 mg/dL; N 0-125 mg/dL). We attempted to treat the patient with glulisine and aspart; however, similar reactions were observed with these insulin preparations as well. As we considered the levels of the anti-insulin antibodies and the late-onset local reactions, the insulin allergy in our patient was reckoned to be mediated by Type 1 and Type 4 hypersensitivity. The only insulin preparation that had never been used with this patient before was lispro, which also demonstrated negative intradermal skin test results. Therefore, we suggested the use of a continuous subcutaneous insulin infusion pump with lispro. Finally, the insulin hypersensitivity was successfully treated, and glycemic control was achieved