Management of Adult Patients With Drug Reaction With Eosinophilia and Systemic Symptoms: A Delphi-Based International Consensus.

Importance: Drug reaction with eosinophilia and systemic symptoms (DRESS) is a rare but potentially fatal drug hypersensitivity reaction. To our knowledge, there is no international consensus on its severity assessment and treatment. Objective: To reach an international, Delphi-based multinational expert consensus on the diagnostic workup, severity assessment, and treatment of patients with DRESS. Design, Setting, and Participants: The Delphi method was used to assess 100 statements related to baseline workup, evaluation of severity, acute phase, and postacute management of DRESS. Fifty-seven international experts in DRESS were invited, and 54 participated in the survey, which took place from July to September 2022. Main Outcomes/Measures: The degree of agreement was calculated with the RAND-UCLA Appropriateness Method. Consensus was defined as a statement with a median appropriateness value of 7 or higher (appropriate) and a disagreement index of lower than 1. Results: In the first Delphi round, consensus was reached on 82 statements. Thirteen statements were revised and assessed in a second round. A consensus was reached for 93 statements overall. The experts agreed on a set of basic diagnostic workup procedures as well as severity- and organ-specific further investigations. They reached a consensus on severity assessment (mild, moderate, and severe) based on the extent of liver, kidney, and blood involvement and the damage of other organs. The panel agreed on the main lines of DRESS management according to these severity grades. General recommendations were generated on the postacute phase follow-up of patients with DRESS and the allergological workup. Conclusions and Relevance: This Delphi exercise represents, to our knowledge, the first international expert consensus on diagnostic workup, severity assessment, and management of DRESS. This should support clinicians in the diagnosis and management of DRESS and constitute the basis for development of future guidelines.

Assessment of Treatment Approaches and Outcomes in Stevens-Johnson Syndrome and Toxic Epidermal Necrolysis: Insights From a Pan-European Multicenter Study.

IMPORTANCE: Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) are severe drug reactions associated with a high rate of mortality and morbidity. There is no consensus on the treatment strategy. OBJECTIVE: To explore treatment approaches across Europe and outcomes associated with the SJS/TEN disease course, as well as risk factors and culprit drugs. DESIGN, SETTING, AND PARTICIPANTS: A retrospective pan-European multicenter cohort study including 13 referral centers belonging to the ToxiTEN ERN-skin subgroup was conducted. A total of 212 adults with SJS/TEN were included between January 1, 2015, and December 31, 2019, and data were collected from a follow-up period of 6 weeks. MAIN OUTCOMES AND MEASURES: Risk factors for severe acute-phase complications (acute kidney failure, septicemia, and need for mechanical ventilation) and mortality 6 weeks following admission were evaluated using a multivariable-adjusted logistic regression model. One tool used in evaluation of severity was the Score of Toxic Epidermal Necrolysis (SCORTEN), which ranges from 0 to 7, with 7 the highest level of severity. RESULTS: Of 212 patients (134 of 211 [63.7%] women; mean [SD] age, 51.0 [19.3] years), the mean (SD) body surface area detachment was 27% (32.8%). In 176 (83.0%) patients, a culprit drug was identified. Antibiotics (21.2%), followed by anticonvulsants (18.9%), nonsteroidal anti-inflammatory drugs (11.8%), allopurinol (11.3%), and sulfonamides (10.4%), were the most common suspected agents. Treatment approaches ranged from best supportive care only (38.2%) to systemic glucocorticoids (35.4%), intravenous immunoglobulins (23.6%), cyclosporine (10.4%), and antitumor necrosis factor agents (3.3%). Most patients (63.7%) developed severe acute-phase complications. The 6-week mortality rate was 20.8%. Maximal body surface area detachment (≥30%) was found to be independently associated with severe acute-phase complications (fully adjusted odds ratio [OR], 2.49; 95% CI, 1.21-5.12; P = .01) and SCORTEN greater than or equal to 2 was significantly associated with mortality (fully adjusted OR, 10.30; 95% CI, 3.82-27.78; P < .001). Cyclosporine was associated with a higher frequency of greater than or equal to 20% increase in body surface area detachment in the acute phase (adjusted OR, 3.44; 95% CI, 1.12-10.52; P = .03) and an increased risk of infections (adjusted OR, 7.16; 95% CI, 1.52-33.74; P = .01). Systemic glucocorticoids and intravenous immunoglobulins were associated with a decreased risk of infections (adjusted OR, 0.40; 95% CI, 0.18-0.88; P = .02). No significant difference in 6-week mortality was found between treatment groups. CONCLUSIONS AND RELEVANCE: This cohort study noted differences in treatment strategies for SJS/TEN in Europe; the findings suggest the need for prospective therapeutic studies to be conducted and registries to be developed.

Biologic Therapy for Hidradenitis Suppurativa Plus Conglobate Acne Associated with SAPHO Syndrome: A Case Report.

is missing (Short Communication).

Clinical Characteristics of Hidradenitis Suppurativa Patients from a Reference Centre in Kaunas, Lithuania.

INTRODUCTION: Clinical data on hidradenitis suppurativa (HS) derived mainly from studies in Western Europe and the USA, raising the risk of potential selection bias. According to the official data, during 2006-2015 the incidence of HS in Lithuania was 23.5 cases per 100,000 population with a female-to-male ratio of 2:1. A more fine-grained data set would however be of great relevance to further understanding this disease. Collection of standardized data enables the comparison of populations of patients across samples. OBJECTIVES: To present a cohort of HS patients with uniformly collected data according to European Hidradenitis Suppurativa Foundation/European Reference Network-Skin recommendations. METHODS: The demographic and clinical data of a total of 37 HS patients, included over a 3-year period (from 2016 to January 2019) in a local database of the reference centre of Rare Skin Diseases in the Hospital of the Lithuanian University of Health Sciences Kauno Klinikos, were analysed. Non-parametric tests such as χ2 and Mann-Whitney were used for assessing interdependence between qualitative data. Logistic regression analysis was performed to find out the factors statistically related with significant diagnostic delay. RESULTS: The mean duration of diagnosis delay was 5.5 (±5.9) years in females and 6.6 (±8.2) years in males. A significant delay (>2 years) was reported in 29/37 (78.4%) cases and was not related with sex, age at HS diagnosis or disease severity. The groin area was more affected in females than in males. There was no statistically significant difference between sex and Hurley stage, HS Physician Global Assessment, International Hidradenitis Suppurativa Severity Score System, visual analogue scale and Dermatology Life Quality Index scores. Combined (medical and surgery) HS treatment was applied to 46.0% and monotherapy (medical or surgery) to 54.0% of patients. CONCLUSIONS: Standardized collection of epidemiological and clinical data is required to improve the quality of HS patient data and allow for international comparisons and pooling of data for research purposes.

Preoperative assessment of skin tumor thickness and structure using 14-MHz ultrasound.

OBJECTIVE: The aim of this study was to compare the relationship between skin tumor thickness and homogeneity and to evaluate the accuracy of 14-MHz ultrasound while measuring the thickness of different skin tumors. MATERIAL AND METHODS: The ultrasonographic and histological analysis of 72 skin tumors was performed. Preoperative vertical tumor thickness (T) and structure of 12 melanomas, 34 melanocytic nevi and 26 basal cell carcinomas was assessed by 14-MHz ultrasonography. After the tumors were excised the vertical thickness measurement (Breslow index, pT) was performed by pathologist. According to the histological thickness all skin tumors were divided to thin (≤1mm) and thick (>1mm). The accuracy of the 14-MHz ultrasound measurements and correlation between the ultrasonographic and histological tumor thickness were estimated. RESULTS: Homogeneous structure was assessed for all thin (≤1mm) and the majority (81.3%) of thick (>1mm) melanocytic skin tumors. Nonhomogeneous structure was estimated in thin and thick basal cell carcinomas, accordingly 42.9% and 31.9%. Measurements of T and pT correlated moderately in thick (>1mm) tumors (r=0.694), while in thin (≤1mm) tumors correlation was low (r=0.336). Moderate correlation between ultrasonographic and histological thickness was computed for melanocytic skin tumors as well as for basal cell carcinomas (r=0.564 and r=0.690). CONCLUSIONS: Medium frequency ultrasound is not a reliable tool for the precise measurement of thin (≤1mm) skin tumors. Ultrasonography using a 14-MHz frequency transducer enables more precisely to measure the thickness of basal cell carcinoma than melanocytic skin tumors. The 14-MHz ultrasound is support tool to suggest the morphologic type of skin tumor.

Cutaneous fungal infection in a neonatal intensive care unit patient: a case report and literature review.

Fungal skin infections are not uncommon in healthy, premature or immunocompromised newborns. Healthy neonates usually develop fungal skin infections caused by dermatophytes, Candida and Malassezia species, whereas immunocompromised neonates are more susceptible to skin infections with opportunistic pathogens (Aspergilus, Zygomycetes). Therefore neonatal fungal skin infections can range from generally benign superficial lesions to potentially fatal, deep, necrotic forms with dissemination. We present the case of a premature neonate twin with cutaneous fungal infection in a neonatal intensive care unit. Because there were doubts concerning the correspondence of the clinical features with the cultured species in the newborn, a literature review was performed searching for similar clinical cases.

Survey of diagnostic services for genital herpes in fourteen countries in Eastern Europe.

This paper reports survey-based data on the diagnosis and management of genital herpes simplex virus (HSV) infection in 14 countries of the Eastern European Network for Sexual and Reproductive Health (EE SRH). Only 43% of the countries could provide the number of genital HSV cases recorded at national level. Eighty-six percent of countries employed syndromic management in cases of genital ulcer disease. Most countries performed type-specific and/or non-type-specific enzyme immunoassays to detect HSV antibodies. Non-type-specific serology for diagnostic purposes should be actively discouraged. Direct detection methods for HSV, such as PCR, antigen detection and culture, are available in the region, but their usage was extremely low. Their use in Eastern European countries should be actively promoted. The availability of laboratory services must be improved, and countries in the region should implement consensus recommendations for the laboratory diagnosis of genital HSV infections in order to improve clinical practice.

Incontinentia pigmenti in a male infant with Klinefelter syndrome: a case report and review of the literature.

Incontinentia pigmenti, also known as Bloch-Sulzberger syndrome, is a hereditary, X-linked dominant disorder characterized by abnormalities of skin, hair, teeth, eyes, and the central nervous system. It is classically considered a male-lethal disorder leading to recurrent miscarriages of male fetuses. We report a rare case of a surviving baby boy with the classic clinical features of incontinentia pigmenti that can be explained by Klinefelter syndrome.

Home sampling and pooling of vaginal samples are effective tools for genetic screening of Chlamydia trachomatis among high school female students in Lithuania.

The aims were 1) to estimate the prevalence of C. trachomatis infection among sexually active female students in Kaunas, Lithuania; 2) to investigate the usefulness of personal invitation, self-sampling, and pooling of samples for screening; and 3) to evaluate the costs of the approaches used. A cross-sectional study inviting 795 female students (18-31 y of age) from 7 high schools and 1 college in Kaunas was performed. The response rate was 67% (533/795). Self-obtained vaginal samples were analysed, individually and pooled (n = 3), using Digene Hybrid Capture II CT/NG Test. The overall prevalence of C. trachomatis infection was 5.6%. Among the sexually active female students 20-24 y of age (n = 424), the prevalence was 7.1%; however, the prevalence varied from 0% to 14.2% at the different schools. For estimation of the population prevalence based solely on identification of C. trachomatis positive pools, the pooling strategy reduced the costs by 85%. For estimation of population prevalence and for diagnosis of each individual sample, pooling reduced the costs by 70%. Targeted screening, using pooling to reduce the expenses, mainly of 3rd and 4th y Lithuanian female students could be recommended. By extended personal contact and internet-based communication, increased participation rates may be attained.

Prevalence and risk factors of genital Chlamydia trachomatis infection.

The aim of the study was to provide a survey and generalization of literature data on the epidemiological situation of Chlamydia trachomatis infection in various countries, preventive screenings and risk factors of the infection. We performed a survey of articles published during 1998-2005 and selected from bibliographical medical search databases presenting data on the prevalence of Chlamydia trachomatis and the main risk factors for this sexually transmitted infection. Chlamydial infection is the most common among sexually transmitted genital infections worldwide. It has been found that the main risk factors for Chlamydia trachomatis infection are age, irregular and/or accidental sexual relationships and change of sexual partners, failure to use or erratic use of barrier contraception during intercourse, and insufficient knowledge about sexual life and care for one's sexual health. Most countries do not have national preventive screening programs or exhaustive information about the prevalence of Chlamydia trachomatis infection. The comparison of the prevalence and incidence of Chlamydia trachomatis infection among different countries is complicated due to the different diagnostic methods and sample selection techniques applied; however, in order to decrease the prevalence of chlamydial infection and its impact on the reproductive health of the society, significant attention should be paid to sexual education, preventive screening of people in high-risk groups, as well as to early diagnostics and timely treatment.