Search

Introduction and Importance: Coarctation of the aorta (COA) is a rare form of congenital heart disease that is typically diagnosed in children. COA is known to present with hypertension, weak or absent femoral pulses, heart failure in older patients, but the presentation of COA as calf atrophy is extremely rare. This article reports the successful surgical repair of a 27-year-old adult with undiagnosed COA., Case Presentation: A 27-year-old-male has presented with calf atrophy, which was diagnosed as COA transthoracic echocardiography and computed tomography angiography indicate COA, which is treated with successful surgical repair., Clinical Discussion: COA is typically diagnosed in children with a rare incidence in adults. Calf atrophy is an extremely rare presentation and uncommon. He has calf atrophy, which led to the diagnosis of COA in 27 years. The presentation in this medium-aged population with this rare manifestation gives our case significance to be one of the unique reported cases., Conclusion: COA is uncommon to be found in adults and the presentation with calf atrophy is even rare. The authors revealed that COA can be found in adults and with an unexpected manifestation and highlights the significance of early detection, and timely referral to a specialist can enable proper management, which includes surgical correction., Competing Interests: The authors declare that they have no competing interests.Sponsorships or competing interests that may be relevant to content are disclosed at the end of this article., (Copyright © 2024 The Author(s). Published by Wolters Kluwer Health, Inc.)

Introduction: Patients with plaque psoriasis often have nail psoriasis, which is difficult to treat. Ixekizumab (IXE) and ustekinumab (UST) are biologics with established efficacy in nail psoriasis. We present post hoc data from a head-to-head trial of IXE and UST (IXORA-S) to examine the efficacy in nail psoriasis in patients with moderate-to-severe plaque psoriasis over 52 weeks., Methods: In IXORA-S,  randomised patients received IXE (N = 136) or UST (N = 166) per label for 52 weeks. Eighty-four (61.8%) and 105 (63.3%) of the patients treated with IXE or UST, respectively, had baseline fingernail psoriasis (Nail Psoriasis Severity Index [NAPSI] > 0); of these, 54 (64.3%) and 63 (60.0%) patients, respectively, had significant baseline fingernail psoriasis (defined as NAPSI ≥ 16 with ≥ 4 fingernails involved). The proportion of patients achieving NAPSI = 0, a NAPSI score change from baseline and correlations in Psoriasis Area of Severity Index (PASI) and NAPSI improvement over 52 weeks were examined., Results: Progressive improvement occurred in both treatment groups over 52 weeks. Statistically significantly more patients achieved NAPSI = 0 with IXE versus UST by week 16-20, and the proportions continued to increase through week 52 among patients with baseline nail psoriasis (61.9 vs. 28.6%, respectively; P < 0.001), including those with significant nail psoriasis (57.4 vs. 17.5%, respectively; P < 0.001). Similar results were observed for NAPSI score improvement from baseline to week 52. Interestingly, the presence of nail psoriasis was associated with lower skin response with UST but not with IXE., Conclusions: Ixekizumab was superior to UST in the clearance of nail psoriasis, with earlier improvement continued through 52 weeks regardless of baseline nail severity., Trial Registration: ClinicalTrials.gov identifier; NCT02561806.

Background: Interleukin-17A is considered to be central to the pathogenesis of psoriasis. We evaluated secukinumab, a fully human anti-interleukin-17A monoclonal antibody, in patients with moderate-to-severe plaque psoriasis., Methods: In two phase 3, double-blind, 52-week trials, ERASURE (Efficacy of Response and Safety of Two Fixed Secukinumab Regimens in Psoriasis) and FIXTURE (Full Year Investigative Examination of Secukinumab vs. Etanercept Using Two Dosing Regimens to Determine Efficacy in Psoriasis), we randomly assigned 738 patients (in the ERASURE study) and 1306 patients (in the FIXTURE study) to subcutaneous secukinumab at a dose of 300 mg or 150 mg (administered once weekly for 5 weeks, then every 4 weeks), placebo, or (in the FIXTURE study only) etanercept at a dose of 50 mg (administered twice weekly for 12 weeks, then once weekly). The objective of each study was to show the superiority of secukinumab over placebo at week 12 with respect to the proportion of patients who had a reduction of 75% or more from baseline in the psoriasis area-and-severity index score (PASI 75) and a score of 0 (clear) or 1 (almost clear) on a 5-point modified investigator's global assessment (coprimary end points)., Results: The proportion of patients who met the criterion for PASI 75 at week 12 was higher with each secukinumab dose than with placebo or etanercept: in the ERASURE study, the rates were 81.6% with 300 mg of secukinumab, 71.6% with 150 mg of secukinumab, and 4.5% with placebo; in the FIXTURE study, the rates were 77.1% with 300 mg of secukinumab, 67.0% with 150 mg of secukinumab, 44.0% with etanercept, and 4.9% with placebo (P<0.001 for each secukinumab dose vs. comparators). The proportion of patients with a response of 0 or 1 on the modified investigator's global assessment at week 12 was higher with each secukinumab dose than with placebo or etanercept: in the ERASURE study, the rates were 65.3% with 300 mg of secukinumab, 51.2% with 150 mg of secukinumab, and 2.4% with placebo; in the FIXTURE study, the rates were 62.5% with 300 mg of secukinumab, 51.1% with 150 mg of secukinumab, 27.2% with etanercept, and 2.8% with placebo (P<0.001 for each secukinumab dose vs. comparators). The rates of infection were higher with secukinumab than with placebo in both studies and were similar to those with etanercept., Conclusions: Secukinumab was effective for psoriasis in two randomized trials, validating interleukin-17A as a therapeutic target. (Funded by Novartis Pharmaceuticals; ERASURE and FIXTURE ClinicalTrials.gov numbers, NCT01365455 and NCT01358578, respectively.).

Background: Little is known about the impact of long-term use of immunosuppressive agents on immune response., Objectives: Assess the impact of continuous maintenance ustekinumab treatment on patients' ability to mount immune responses to pneumococcal (T-cell-independent) and tetanus toxoid (T-cell-dependent) vaccines., Patients and Methods: Ustekinumab-treated patients with moderate-to-severe psoriasis treated in the long-term extension of the Phase 3 PHOENIX 2 trial (n=60) were compared with control psoriasis patients not receiving systemic therapy (n=56). Patients were vaccinated with both 23-valent pneumococcal and tetanus toxoid vaccines. Serum samples collected pre-vaccination and 4 weeks post-vaccination were assessed for antibody responses., Results: No differences in the ability of ustekinumab-treated patients to respond to pneumococcal or tetanus toxoid vaccinations were observed compared with controls. A ≥2-fold increase in antibody levels in ≥7 of 14 serotypes of the pneumococcal vaccine was observed in ustekinumab-treated (96.6%) and untreated control (92.6%) patients following vaccination. Ustekinumab-treated patients achieved a ≥4-fold increase (84.7%) in anti-tetanus antibody vs. 77.8% in the control group. No differences were detected in ex-vivo responses to anti-CD3/CD28 or tetanus toxoid between ustekinumab-treated and control groups., Conclusion: Long-term treatment (≥3 years) with ustekinumab does not compromise the immune response to T-cell-dependent/-independent vaccines in patients with moderate-to-severe psoriasis.

Objective: To describe practice patterns for care of Canadian patients with moderate to severe plaque psoriasis., Design: Online survey of a consumer panel., Setting: Participants were drawn from a population-wide Canadian consumer database., Participants: To be eligible to participate, respondents had to have been diagnosed with plaque psoriasis within the past 5 years, and to have had body surface area involvement of 3% or greater in the past 5 years, or to have psoriasis on a sensitive area of the body (hands, feet, scalp, face, or genitals), or to be currently receiving treatment with systemic agents or phototherapy for psoriasis., Main Outcome Measures: Proportion of respondents with psoriasis managed by FPs and other specialists, psoriasis therapies, comorbidities, and patient satisfaction., Results: Invitations were sent to 3845 panelists with self-reported psoriasis, of which 514 qualified to complete the survey. Family physicians were reported to be the primary providers for diagnosis and ongoing care of psoriasis in all provinces except Quebec. Overall physician care was reported to be satisfactory by 62% of respondents. Most respondents receiving over-the-counter therapies (55%) or prescribed topical therapies (61%) reported that their psoriasis was managed by FPs. Respondents receiving prescription oral or injectable medications or phototherapy were mainly managed by dermatologists (42%, 74%, and 71% of respondents, respectively). Ongoing management of respondents with body surface area involvement of 10% or greater was mainly split between dermatologists (47%) and FPs (45%), compared with rheumatologists (4%) or other health care professionals (4%). Of those respondents receiving medications for concomitant health conditions, treatment for high blood pressure was most common (92%), followed by treatment for heart disease (75%) and elevated cholesterol and lipid levels (68%)., Conclusion: Patient-reported practice patterns for the diagnosis and management of moderate to severe psoriasis vary among provinces and in primary and secondary care settings.

Background: Although the range of therapeutic options has expanded dramatically in recent years, topical agents remain ubiquitous and indispensable tools for treating psoriasis at all levels of severity. The 2009 Canadian psoriasis guidelines considered evidence supporting various monotherapies and combination regimens., Objective: Here we review all approved topical agents, including corticosteroids, calcineurin inhibitors, vitamin D analogues, and retinoids, used in psoriasis and develop additional treatment recommendations, using the Scottish Intercollegiate Guidelines Network (SIGN) system to evaluate strength of evidence, as in the original guidelines., Conclusion: We propose that topical treatments have a place in the long-term management of patients with moderate to severe plaque psoriasis, including those receiving concomitant photo- or systemic therapy. Topical agents are effective and appropriate treatments for psoriasis as long as the physician is attentive to signs of local adverse events and seeks opportunities to reduce the dose or treatment frequency during chronic use.

Objective: To determine the efficacy, safety, and sustainability of response to adalimumab therapy for moderate to severe chronic plaque psoriasis involving hands and/or feet., Design: Sixteen-week, randomized, double-blind, placebo-controlled evaluation of adalimumab therapy for moderate to severe chronic plaque psoriasis involving the hands and/or feet with a 12-week open-label extension (Randomized Controlled Evaluation of Adalimumab in Treatment of Chronic Plaque Psoriasis of the Hands and Feet [REACH])., Setting: Multicenter outpatient study in the United States and Canada., Participants: Patients with chronic plaque psoriasis on the hands and/or feet with a Physician's Global Assessment of hands and/or feet (hfPGA) score of "moderate" or above., Intervention: Patients were randomized 2:1 to adalimumab (80 mg at week 0, then 40 mg every other week starting at week 1) or to matching placebo., Main Outcome Measure: Percentage of patients achieving an hfPGA score of "clear" or "almost clear" at week 16., Results: Seventy-two patients (adalimumab [n = 49];placebo [n = 23]) were evaluated. Baseline percentages of patients with moderate and severe hfPGA scores were 76% and 24%, respectively, for the adalimumab group and 74% and 26%, respectively, for the placebo group. At week 16, 31% and 4% of patients randomized to adalimumab and placebo, respectively, achieved an hfPGA score of clear or almost clear (P = .01). At week 28, 80% of the hfPGA clear or almost clear response was maintained from week 16 (25% for patients randomized to adalimumab). Adverse events in both groups were generally mild to moderate. In both periods combined, nasopharyngitis (27% and 13% for adalimumab- and placebo-treated patients, respectively) was most frequently reported., Conclusion: Adalimumab is efficacious and well tolerated for treatment of chronic plaque psoriasis of hands and/or feet, with efficacy largely maintained to 28 weeks. Trial Registration clinicaltrials.gov Identifier: NCT00735787.

Background: limited data are available on the epidemiologic features of psoriasis in Canada., Objective: to investigate the epidemiologic features and burden of moderate-to-severe psoriasis in a Canadian population., Methods: an online survey was conducted using a consumer panel. Eligible respondents indicated a diagnosis of psoriasis and plaque-type psoriasis of at least moderate severity. Eligibility was validated according to self-reported body surface area (BSA) involvement, sensitive areas affected, and/or current treatment., Results: of the 514 respondents who completed the survey, 62% estimated a BSA involvement of >/= 3% within the past 5 years. Onset of psoriasis occurred earlier in females than in males. Nail involvement was more commonly reported in individuals with psoriatic arthritis compared to those without. Several symptoms were more likely described as "constantly" or "near constantly" experienced by females than by males. Comorbidities commonly reported were hypertension, dyslipidemia, and overweight or obesity., Conclusions: the findings are consistent with a substantial burden attributed to moderate-to-severe plaque psoriasis in a Canadian population.

Background: Few population studies of individuals living with psoriasis have been performed in Canada., Objective: The objective of this survey was to understand the severity and impact of psoriasis on the lives of Canadian patients., Methods: An online survey was conducted using a consumer panel. Eligible subjects reported a diagnosis of psoriasis and provided a self-reported level of severity. In addition, subjects had to either (a) have psoriasis covering at least 3% of their body surface area; (b) have psoriasis on a sensitive area of the body; or (c) be currently undergoing treatment for their psoriasis with systemic medication and/or phototherapy., Results: A total of 514 panelists met the inclusion criteria and completed the survey. Current moderate, severe, or very severe psoriasis was reported by 65% of respondents. Nearly all subjects (96%) had psoriasis affecting a sensitive area of the body. At the time of the survey, 18% were taking systemic medication and/or phototherapy. Comorbidities, such as obesity and high blood pressure, were highly prevalent, with 75% of respondents reporting at least one other diagnosis. Data from the SF-8 and Dermatology Life Quality Index instruments indicated that psoriasis negatively impacted quality of life., Conclusion: Moderate-to-severe psoriasis places a burden on Canadian patients, some of whom may be receiving suboptimal treatment or treatment not appropriate for the severity of their condition.

Background: The pathogenesis of lupus vulgaris, a form of cutaneous tuberculosis, is not always clear, especially in patients who do not have coexistent extracutaneous tuberculosis and in patients with single lesions., Objectives: To report a case of lupus vulgaris in a locus minoris resistentiae (a site of reduced resistance) and to use a unique set of clinical circumstances and laboratory tests to reconstruct the pathogenesis of the lesion and the response to treatment., Conclusion: Lupus vulgaris can occur in a locus minoris resistentiae; local trauma and possibly other factors, such as increased temperature, topical corticosteroids, and the virulence of the infecting strain, may facilitate the growth of Mycobacterium tuberculosis present at a locus minoris resistentiae as a result of a silent bacillemia.

Background: Alopecia areata of the eyebrows can be difficult to treat. Intralesional triamcinolone or potent topical steroids are considered the mainstay of medical therapy. This case illustrates the results of an experimental hair transplant to the eyebrows following years of modest response to intralesional triamcinolone., Objective: The aim of this study was to ascertain the benefits of a hair transplant for chronic eyebrow alopecia areata not responding to appropriate medical therapy., Methods: A hair transplant was performed with tumescent anesthesia and a total of 85 mini and micrografts placed in the right eyebrow. Followup after the hair transplant occurs every 8 weeks., Results: The patient was free of eyebrow alopecia areata for 8 months following the initial hair transplant. Although the disease relapsed, hair growth is now manageable with intralesional cortisone injection performed six times per year., Conclusions: For the first time in years, this patient was given 8 months of reprieve from his eyebrow alopecia areata and is currently well-maintained on monthly intralesional cortisone which originally was of only modest benefit. The patient is pleased with the outcome.

We present an infant girl who was born with pyloric stenosis and epidermolysis bullosa (EB). Electron microscopy of a skin biopsy specimen showed findings suggestive of EB simplex, but immunofluorescence (IF) mapping of the same specimen established the diagnosis of junctional EB. Because electron microscopy findings may sometimes be misleading, an EB patient with pyloric stenosis and electron microscopy findings suggestive of EB simplex should have a biopsy specimen examined by immunofluorescence mapping, which may confirm that the patient in fact has junctional EB.

Background: Minocycline-induced cutaneous pigmentation is an adverse effect that may be more common than is generally realized. It is usually reported in patients undergoing chronic minocycline therapy for acne vulgaris., Objective: The case of a 69-year-old woman taking minocycline for rheumatoid arthritis is presented, and its differential diagnosis discussed in order to characterize the clinical features of minocycline-induced cutaneous pigmentation., Conclusion: Patients undergoing minocycline therapy for rheumatoid arthritis may develop bluish-grey pigmentation over the legs and forearms. Cutaneous pigmentation is a well recognized adverse effect of minocycline therapy that is usually reported in young patients on chronic therapy for acne vulgaris. However, the antiinflammatory properties of minocycline have also made it useful in the management of various inflammatory conditions such as rheumatoid arthritis.1 We report the case of a 69-year-old woman who developed progressive cutaneous pigmentation, affecting mainly the legs, approximately 3 months after beginning minocycline therapy for rheumatoid arthritis.

Tay-Sachs disease (TSD) results from mutations in HEXA that cause Hex A deficiency. Heterozygote-screening programs have been applied in groups with an increased TSD incidence, such as Ashkenazi Jews and French Canadians in Quebec. These programs are complicated by benign mutations that cause apparent Hex A deficiency but not TSD. Benign mutations account for only approximately 2% of Jewish and approximately 36% of non-Jewish enzyme-defined carriers. A carrier frequency of 1/53 (n = 1,434) was found in an ongoing prospective analysis of persons of French Canadian background living in New England by using an enzyme-based assay. DNA from enzyme-defined carriers from this population was analyzed to determine the molecular basis of Hex A deficiency. Samples (36) were tested for common mutations, and samples that were negative for these were screened for uncommon or novel mutations by using SSCP analysis. Exons showing mobility shifts were sequenced, and most mutations were confirmed by restriction enzyme digestion. Known disease-causing mutations were found in nine samples (four had a 7.6-kb deletion found in 80% of French Canadian TSD alleles), and known benign mutations were found in four samples. Seven novel changes were identified, including G748A in four samples. The molecular basis of Hex A deficiency in this carrier population differs from that of French Canadian TSD patients. Screening centers should be aware of the presence of benign mutations among U.S. French Canadians or Franco-Americans.(ABSTRACT TRUNCATED AT 250 WORDS)