Developing a core outcome set for Netherton syndrome: An international multi-stakeholder e-Delphi consensus study
Netherton syndrome (NS; OMIM#256500) is a rare and severe disorder of epidermal maturation and keratinization caused by pathogenic variants in the serine protease inhibitor Kazal type 5 (SPINK5), leading to severe skin barrier impairment. Although effective treatment is crucial for NS patients, there is a lack of knowledge on what the best treatment options are for these patients. Large heterogeneity in reported outcomes and measurement instruments hinders accurate comparison of treatment results across studies and the development of a treatment guideline. Therefore, we aimed to develop a core outcome set (COS) for NS that can be used in clinical care and research.
Nodular vasculitis - Retrospective study of an uncommon disease in a non-tuberculosis endemic country with focus on treatment modalities and efficacy
Introduction Nodular vasculitis (NV) is a rare form of panniculitis primarily affecting middle-aged females, presenting as painful, sometimes ulcerated nodules on the dorsal lower legs. Erythema induratum Bazin (EIB) is a form of NV and is considered a manifestation of cutaneous tuberculin hypersensitivity. This retrospective study aims to analyze demographics, clinicopathological findings, laboratory results, and treatment outcomes of NV in a non-TB endemic country.
Management strategies and corticophobia among healthcare professionals involved in the care for atopic dermatitis: a Dutch survey
Many healthcare professionals (HCPs) deliver care for patients with atopic dermatitis (AD). Although pivotal, management strategies and the relation with corticophobia among HCPs have not been investigated. This study aimed to investigate management strategies for AD and its relation with corticophobia among HCPs.
A territory wide follow-up of primary and secondary extramammary Paget disease of two decades - effects of local disease on survival
Introduction Extramammary Paget disease (EMPD) is an uncommon malignant cutaneous neoplasm that are divided into primary and secondary forms. In this multicenter study, histologically proven cases of primary and secondary EMPD were reviewed for clinical outcomes with subgroup analysis for secondary EMPD. Methodology Cases of EMPD were identified from pathology report of the involved institutions over a period of over two decades. Cases of secondary EMPD were identified review of case notes, radiology, and pathology reports. Clinicopathological and outcome data were retrieved for statistical analysis. Results A total of 109 cases were retrieved, including 19 cases of secondary EMPD, most commonly associated with colorectal (n=6), anal (n=5) and prostatic carcinomas (n=3). A difference was observed between older age and secondary (versus primary) EMPD (p=0.016), but no differences were seen in other clinico-demographical parameters. Male sex (p=0.018), age over 60 years old (p=0.004) and involvement of margins (resectable) (p=0.018) were associated with shorter OS. For DSS, involvement of margins (p=0.009) was an adverse predictor. Secondary EMPD had a shorter DSS than primary EMPD (p=0.005). Multivariable analysis confirmed all above associations (p<0.05). In subgroup analysis for secondary EMPD, margin involvement remained associated with shorter OS (p = 0.007) and DSS (p = 0.003). Conclusions Secondary EMPD is associated with poorer outcomes. Margin involvement is strong and independent indicator of shorter OS and DSS, including secondary EMPD. Resectability is a strong predictor of favorable outcome and excision with clear margins should be attempted when surgically feasible.
Brodalumab is Effective for Psoriasis Patients with Difficult-to-treat Body Regions: Results from an Observational Clinical Study
Brodalumab, a human monoclonal antibody that selectively inhibits the interleukin (IL)-17 receptor subunit A, has been approved for the treatment of moderate-to-severe plaque psoriasis. The treatment benefit of brodalumab has been clearly demonstrated in multiple clinical studies. However, data on effectiveness for difficult-to-treat body regions, especially in everyday clinical practice, are still limited.
Treatment satisfaction in patients with hidradenitis suppurativa: A real-world survey from the EU5 and US
Introduction Hidradenitis suppurativa (HS) is a debilitating, inflammatory skin disorder. Treatment strategies in patients with HS are challenging; real-world evidence in a HS population is warranted for greater disease understanding. The objective of this analysis was to describe real-world treatment patterns and treatment satisfaction in patients with HS. Methods This was a cross-sectional market research survey with retrospective data collection in patients with HS from the United States and five European countries (France, Germany, Italy, Spain, United Kingdom) between November 2020 and April 2021, using physician- and patient-reported surveys. Eligible physicians were general dermatologists actively managing patients with HS; dermatologists were required to have consulted with ≥2 patients with HS in the previous 12 months. Adult (≥18 years) and adolescent (10‒17 years) HS patients visiting a participating dermatologist were included. Outcomes included treatment patterns, flare status, treatments prescribed in response to flares, previous surgeries, barriers to biologics, and patient- and physician-reported satisfaction with the disease control provided by treatment. Results Survey data from 1787 patients were collected from 312 dermatologists. The most frequently prescribed treatments were topicals, oral antibiotics, and antiseptic washes/creams at diagnosis and sampling. At sampling, biologics were more frequently prescribed in patients with more severe disease (prescribed in 26.6%, 31.0% and 52.4% of patients with mild, moderate, and severe disease, respectively); oral antibiotics (48.8%), topicals (37.4%), and biologics (34.3%) were the most frequently prescribed treatment classes in response to a flare. Of patients currently not receiving a biologic, dermatologists reported that 18.9% of patients' condition warranted their use. Approximately one quarter of dermatologists (24.5%) and patients (27.4%) were not satisfied with current treatment; of patients who were dissatisfied, 12.8% reported they would never raise their dissatisfaction with their doctor. Conclusion These real-world data suggest a high disease burden and potential undertreatment in patients with HS. Patients received multiple treatments, and a notable proportion underwent surgery. Robustly integrating the patient voice in HS treatment decisions may lead to better outcomes and improved treatment satisfaction.
Rebound Growth of Infantile Hemangiomas after Propranolol versus Atenolol Treatment: A Retrospective Study
Propranolol is the first-line treatment for complicated infantile hemangioma (IH). Rebound growth following propranolol discontinuation is reported in 6-25% of patients. Atenolol is considered an effective alternative to propranolol. We aimed to compare the incidence of IH rebound growth following discontinuation of atenolol and propranolol and to identify associated risk factors.
Identification of risk factors associated with metabolic dysfunction-associated steatotic liver disease in psoriatic patients
Metabolic dysfunction-associated steatotic liver disease (MASLD) is a common cause of chronic liver disease. Patients suffering from psoriasis are at an increased risk of developing MASLD. Psoriasis and MASLD share a pro-inflammatory cytokine milieu, however it is still unclear whether these conditions are related through shared metainflammatory processes or shared comorbidities such as obesity, diabetes, insulin resistance and metabolic syndrome. The aim of our study was to better characterise the anthropometric and metabolic profile of psoriatic patients with MASLD.
Squamous Metaplasia of Lactiferous Ducts (Zuska's disease) of the Breast: Clinical and Histopathologic Manifestations
Squamous metaplasia of lactiferous ducts (SMOLD), also known as Zuska's disease, is an uncommon, recurrent inflammatory fistulizing disease of the breast that strongly correlates with smoking in premenopausal patients .1 Clinical and imaging findings may overlap with other breast conditions/ SMOLD is well-recognized by breast pathologists, however, the dermatology literature on this condition remains scarce. In this retrospective study, we reviewed 29 patients with SMOLD diagnosed at Mayo Clinic. The mean age of the patient cohort is 50.3 with a range of 30 to 81 years. One patient (3.7%) had hidradenitis suppurativa of the retro-areolar area. Patient smoking history demonstrated prior/current smokers 37.9% (11/29), lifetime nonsmokers with significant secondhand exposure 6.9% (2/29), and unknown smoking status 3.4% (1/29). One patient had a personal history of invasive ductal carcinoma and 10.3% (3/29) had a history of breast cancer in a first-degree relative. The clinical presentation of the patient cohort includes areolar papules, nodules and draining tract/fistula 13.7% (4/29), pustular cyst/abscess on the breast 13.7% (4/29), breast mass 3.4% (1/29), pain breast discomfort/pain 13.7% (4/29), nipple retraction 3.4% (1/29), and asymptomatic with nipple calcifications on mammogram 3.4% (1/29). 77.8% (7/9) patients with bacterial cultures demonstrated polymicrobial growth. 37.9% (11/29) patients received at least one round of antibiotic therapy. 27.6% (8/29) patients underwent invasive intervention. Staphylococcus, Streptococcus, and Cutibacterium species were the most frequent causes of infection in our patient cohort. We confirm previous findings of strong association between SMOLD & current/former smoking status and a potential, novel correlation between extensive secondhand exposure and SMOLD development. While both medical and surgical interventions are employed in patient management, many patients ultimately require complete excision of the involved duct(s). Dermatologists should consider SMOLD in the differential diagnosis of patients presenting with breast abscess, fistulizing tracts with mass, and breast pain.
Clinical features and natural course of Hidradenitis Suppurativa in Turkey: a multicentre study
The natural history, the progression of a disease process in an individual over time, has not yet been fully elucidated in hidradenitis suppurativa (HS). In this large multicenter study, we aimed to investigate the natural history of HS and its gender differences.
Systemic Therapy for Atopic Dermatitis in Children and Adolescents: A US Expert Consensus
Atopic dermatitis (AD) is a chronic, type-2 mediated, inflammatory skin disease characterized by intense pruritus, disruption of skin barrier function, and immune dysregulation. Management strategies for AD are routinely determined based on disease severity. First-line treatment begins with basic skin care and topical anti-inflammatory medication, which is typically sufficient for the management of mild-to-moderate disease. For those patients with moderate-to-severe disease, systemic therapy is often required. This can involve off-label treatment with conventional immunosuppressant medications. However, this approach is limited by a lack of robust clinical trial data and safety concerns that necessitate close monitoring. The emergence of novel targeted biologics and small molecules to treat AD presents an opportunity to optimize AD management and patient outcomes by offering greater efficacy than traditional immunosuppressants and a favorable safety profile. As the treatment landscape shifts, clinicians can benefit from a standardized process of patient assessment and treatment, along with resources to help maintain contemporary knowledge of available therapeutic options. This US-based, expert-led consensus used a modified Delphi process to develop core recommendations for the use of systemic medications for the management of pediatric patients <18 years of age with moderate-to-severe AD.
Tetracyclines Revisited: Tetracyclines in the Field of Dermatology
Tetracyclines are a class of broad-spectrum antibiotics favored by dermatologists. Over the last decade, the clinical efficacy of tetracyclines has expanded into various dermatoses.
Assessing Disease Control in Patients with Atopic Dermatitis by Using the Atopic Dermatitis Control Tool in Daily Practice
Atopic dermatitis (AD) is a chronic inflammatory skin disease, placing a significant burden on patients' quality of life (QoL). The validated Atopic Dermatitis Control Tool (ADCT) is recommended to assess AD control in adults. The aim of this study was to assess AD control and explore associations with demographic characteristics, patient-reported outcome measures (PROMs), and treatment.
Association between hidradenitis suppurativa and gout: a propensity-score-matched cohort study
Introduction While an association between hidradenitis suppurativa (HS) and inflammatory arthritis has been reported in clinical studies, the potential link between HS and gout remains uncertain. As HS and gout share common immunological pathways, we conducted a retrospective cohort study to determine whether HS patients are at an increased risk of developing gout in the future. Methods This retrospective multicenter cohort study obtained information through the US collaborative network, a subset of the TriNetX research network. Patients diagnosed with HS between January 01, 2005, and December 31, 2017, were recruited, and a 1:1 propensity score matching was conducted to identify appropriate controls. The hazard ratio for the new-onset of gout in HS patients was subsequently calculated. Results Compared to individuals without HS, those with HS were associated with a 1.39-fold higher risk (95% CI, 1.20, 1.62) of developing new-onset gout within five years after the index date. This association remained significant in shorter follow-up times and sensitivity analyses utilizing different matching models. For both male and female HS patients, the risk of developing new-onset gout within 5 years after the index date was statistically significant, with respective hazard ratios of 1.61 (95% CI, 1.28,2.02) for males and 1.41 (95% CI, 1.11,1.78) for females. Conclusion HS patients are at a high risk of developing gout within five years after an HS diagnosis while comparing with non-HS controls.
Efficacy and Safety of Upadacitinib Versus Dupilumab Treatment for Moderate to Severe Atopic Dermatitis in Four Body Regions: Analysis From the Heads-Up Study
Upadacitinib has demonstrated high and rapid rates of efficacy in adolescent and adult patients with moderate-to-severe atopic dermatitis (AD) as assessed by the Eczema Area and Severity Index (EASI). This post hoc analysis assessed the EASI response in four anatomical regions for patients with moderate-to-severe AD treated with upadacitinib compared to dupilumab over 24 weeks.
Artificial Intelligence-Driven Skin Aging Simulation as a Novel Skin Cancer Prevention
Skin cancer, a prevalent cancer type among fair-skinned patients globally, poses a relevant public health concern due to rising incidence rates. Ultraviolet (UV) radiation poses a major risk factor for skin cancer. However, intentional tanning associated with sunburns remains a common practice, notably among female adults. Appropriate prevention campaigns targeting children and adolescents are needed to improve sun protection behavior particularly in these age groups. The aim of our study was to investigate if an AI-based simulation of facial skin aging can enhance sun protection behavior in female adults.
Twenty Five Years of Medical Discoveries Aboard the International Space Station: A Retrospective Discussion of Medical Translations from Space
The Copenhagen Hidradenitis Suppurativa Cohort: Insights from the First 8 Years
Factors Associated with Severe Hidradenitis Suppurativa, Using Hurley Staging and Metascore
Hidradenitis suppurativa (HS) is a chronic inflammatory skin disease for which certain risk factors are well known: obesity and smoking (in particular). However, the factors associated with more severe conditions, and therefore potential aggravators of the disease, remain a matter of debate. Our study aims to determine the clinical factors associated with severe HS using several severity scores.
Identification of Novel Genetic Risk Variants Associated with Hidradenitis Suppurativa in an Exome Sequencing Cohort of 92,455 Individuals
Hidradenitis suppurativa (HS) is a prevalent and persistent inflammatory skin disorder, lacking a known cure or effective biomarkers for early diagnosis at present. The genetic determinants of HS have not been fully documented, but it is believed to result from a combination of genetic and environmental factors.
Hidradenitis suppurativa prevalence in Algeria: A multicenter cross-sectional study
Introduction Although there has been an expansion of knowledge on hidradenitis suppurativa (HS), data about the disease is largely based on Western population and no relevant African or Asian studies are available. Methods We conducted a descriptive, cross-sectional, multicenter study, as part of GHiSA (Global HS Atlas) initiative, to assess the epidemiologic profile of HS in Algerian population. Healthy adults accompanying patients undergoing care in a non-dermatological wards were approached and invited to complete a self-administered questionnaire. Subsequently, a clinical assessment was performed by an in-person dermatologists for all screen-positive participants and ten percent of the screen-negative ones. Results A total of 1434 participants were included in this study. The prevalence of HS among Algerian adults was 0.78%. Compared to non HS group, no significant difference was found regarding gender, age, body mass index and smoker status. Both the sensitivity (100%) and the specificity (97%) of the HS screening questionnaire were excellent. Conclusion The prevalence of HS in Algeria is very close to that of Australia (0.8%) and Europe (0.7%) and almost the same prevalence found by Ghanaian study (other GHiSA study from Africa). The results of this study demonstrate also the reliability and validity of GHiSA questionnaire as HS data collection instrument.