Assessment of diagnostic delay, morbidity, and mortality outcomes in 302 calciphylaxis patients over a 17-year period: A retrospective cohort study.

BACKGROUND: Calciphylaxis patients historically have experienced diagnostic challenges and high morbidity; however limited data is available examining these characteristics over time. OBJECTIVE: The primary goals were to a) investigate factors associated with diagnostic delay of calciphylaxis and b) assess morbidity outcomes. The secondary goal was to provide updated mortality rates. METHODS: A retrospective review of 302 adult patients diagnosed with calciphylaxis between January 1, 2006 and December 31, 2022 was conducted. Univariate and multivariate statistical analyses were performed. RESULTS: Nonnephrogenic calciphylaxis (P = .0004) and involvement of the fingers (P = .0001) were significantly associated with an increased diagnostic delay, whereas involvement of the arms (P = .01) and genitalia (P = .022) resulted in fewer days to diagnosis. Almost all patients with genitalia, finger, or toe involvement had nephrogenic disease. The number of complications per patient decreased with time, especially for wound infections (P = .028), increase in lesion number (P = .012), and recurrent hospitalizations (P = .020). Updated 1-year mortality rates were 36.70% and 30.77% for nephrogenic and nonnephrogenic calciphylaxis, respectively. LIMITATIONS: Limitations include the retrospective nature and data from a single institution. CONCLUSION: Diagnostic delay, particularly in nonnephrogenic calciphylaxis, and complications per patient decreased with time, highlighting the importance of continued awareness to expedite diagnosis. Mortality rates have continued to improve in recent years.

Clinical mimickers of calciphylaxis: A retrospective study.

BACKGROUND: Calciphylaxis is an ischemic vasculopathy with high morbidity and mortality. Early and accurate diagnosis is critical to management of calciphylaxis. Clinical mimickers may contribute to delayed or misdiagnosis. OBJECTIVE: To assess the rate and risk factors for misdiagnosis and to identify clinical mimickers of calciphylaxis. METHODS: A retrospective medical record review was conducted of patients with calciphylaxis at a large urban tertiary care hospital between 2006 and 2018. RESULTS: Of 119 patients diagnosed with calciphylaxis, 73.1% were initially misdiagnosed. Of patients not initially misdiagnosed, median time to diagnosis from initial presentation was 4.5 days (interquartile range, 1.0-23.3), compared to 33 days (interquartile range, 13.0-68.8) in patients who were initially misdiagnosed (P = .0002). The most common misdiagnoses were cellulitis (31.0%), unspecified skin infection (8.0%), and peripheral vascular disease (6.9%). Patients who were misdiagnosed frequently received at least 1 course of antibiotics. Patients with end-stage renal disease were less likely to be misdiagnosed than those without this disease (P = .001). LIMITATIONS: Single-center, retrospective study. CONCLUSIONS: Understanding the risk factors for misdiagnosis of calciphylaxis is an opportunity for further education concerning this rare disease.

Assessment of outcomes of calciphylaxis.

BACKGROUND: Calciphylaxis is a rare thrombotic vasculopathy characterized by high morbidity and mortality. There is a paucity of studies examining longitudinal outcomes. OBJECTIVE: To assess mortality, days spent in the hospital, and amputations in patients with calciphylaxis. METHODS: A retrospective medical record review was conducted in 145 patients diagnosed with calciphylaxis at an urban tertiary care hospital from January 2006 to December 2018. RESULTS: Six-month mortality was 37.2%, and 1-year mortality was 44.1%. Patients with nephrogenic calciphylaxis had worse survival than those with nonnephrogenic calciphylaxis (P = .007). This difference in survival disappeared when limiting mortality to deaths due to calciphylaxis. Age (P = .003) and end-stage renal disease (P = .01) were risk factors associated with 1-year mortality. Diabetes mellitus was associated with greater total hospitalization days (coefficient, 1.1; 95% confidence interval, 1.01-1.4); bedside debridement was associated with fewer hospitalization days (coefficient, 0.8; 95% confidence interval, 0.7-0.9). Amputations were not associated with any of the examined risk factors. The use of warfarin followed by a transition to nonwarfarin anticoagulation was associated with decreased hazard of death (P = .01). LIMITATIONS: Retrospective nature. CONCLUSIONS: Calciphylaxis remains a complex, heterogeneous disease. Mortality is lower in patients with nonnephrogenic disease. These findings may be incorporated during discussions regarding the goals of care to facilitate informed shared decision making.

Penile calciphylaxis: A retrospective case-control study.

BACKGROUND: Calciphylaxis is a rare disorder characterized by skin necrosis caused by calcium deposition within vessels, thrombosis, and subsequent tissue ischemia. Penile involvement may rarely occur. OBJECTIVE: To identify risk factors, diagnosis, management, and mortality of patients with penile calciphylaxis. METHODS: A retrospective medical record review was conducted of 16 patients with penile calciphylaxis treated at 2 large urban tertiary care centers between January 2001 and December 2019. A control group of 44 male patients with nonpenile calciphylaxis at the same institution was included. RESULTS: The median survival of patients with penile calciphylaxis was 3.8 months (interquartile range, 27.0 months). Mortality was 50% at 3 months and 62.5% at 6 months for penile calciphylaxis, and 13.6% at 3 months and 29.5% at 6 months for controls (P = .008). Patients with penile calciphylaxis were less likely to be obese (P = .04) but more likely to have hyperparathyroidism (P = .0003) and end-stage renal disease (P = .049). LIMITATIONS: Retrospective study design and small sample size. CONCLUSIONS: This study further defines the disease course of penile calciphylaxis, which has high mortality. Imaging may be used to aid diagnosis. Risk factors include end-stage renal disease, hyperparathyroidism, and normal body mass index.

Clinical factors before or after device implantation in predicting metal hypersensitivity reactions: A retrospective study.

BACKGROUND: Metal hypersensitivity reactions (MHR) as a cause of implant-related complications are highly debated and recommendations regarding pre-procedural allergy evaluation vary dramatically. OBJECTIVE: To examine patients referred before or after device implantation and identify factors that could be useful to guide the value of patch testing. METHODS: Patients who underwent patch testing pre- or post-device implantation between July 2006 and September 2016 were analyzed retrospectively. RESULTS: A series of 127 patients underwent patch testing: pre-implantation (n = 40) and post-implantation (n = 87). In the pre-implant group, a history of metal allergy demonstrated high sensitivity (0.94; 95% CI: 0.83-1.00) and negative likelihood ratio (0.17; 95% CI:0.02-1.29) for diagnosing MHR. No predictive value could be ascribed to any of the clinical symptoms (eg dermatitis, pain, swelling, implant failure, and/or other symptoms) for patients referred following orthopaedic and dental post device implantation. Eight patients in the orthopaedic group and six patients in dental group with relevant patch test reactions underwent implant revisions, and seven and five patients improved, respectively. CONCLUSIONS: Pre-implant patch testing for selected individuals with a history of metal allergy can help guide implant choice. Post-implant patch testing may be helpful in some patients if other causes have been excluded, as patients with confirmed MHR benefited from revisions.

Assessing the validity of self-reported history of rash caused by metal or jewellery.

BACKGROUND: The use of metal-containing bio-devices is becoming increasingly common. Self-reported history of dermatitis with metal exposure is not established as being predictive for metal allergy. OBJECTIVES: To assess the validity of two screening questions addressing metal allergy. METHODS: At Massachusetts General Hospital Contact Dermatitis Clinic, 2132 consecutive patients were asked either 'Do you get rashes when jewellery touches your skin' (Q1; N = 1816) or 'Do you get rashes when metal touches your skin?' (Q2; N = 316) before being patch tested. RESULTS: Testing showed that 20% of subjects had positive reactions to nickel, 7.4% had positive reactions to cobalt, and 5.8% had positive reactions to chromium. Q1 was 40% sensitive (95%CI: 35-45%). The positive predictive value (PPV) was 51%, and the negative predictive value (NPV) was 82%. Q2 was 77% sensitive (95%CI: 68-84%). The PPV was 71%, and the NPV was 84%. Q2 was 37% more sensitive than Q1 (p < 0.0001), with a higher relative risk (RR) (4.75, p < 0.001) than Q1 (RR = 3.01, p < 0.001). CONCLUSIONS: Patient-reported metal allergy, although not perfect, is a reasonable method for metal allergy screening to help identify those needing further objective evaluation by patch testing.

Incorporating social media into dermatologic education.

In the current digital age, medical education has slowly evolved from the largely lecture-based teaching style of the past to incorporate more interactive pedagogical techniques, including use of social media. Already used readily by millennial trainees and clinicians, social media can also be used in innovative ways to teach trainees and facilitate continuing education among practicing clinicians. In this commentary, we discuss many learning benefits of social media and review potential pitfalls of employing social media in both trainee and physician dermatological education.

Lower Urinary Tract Dysfunction in Elementary School Children: Results of a Cross-Sectional Teacher Survey.

PURPOSE: Lower urinary tract dysfunction in school-aged children is common and yet data are lacking on current teacher practice regarding bathroom use and daytime incontinence during classroom hours. We determined the prevalence of elementary school teachers who promote lower urinary tract health and identified predictors for and against such behavioral promotion. MATERIALS AND METHODS: We performed an electronic cross-sectional survey among self-identified teachers using targeted social media advertisement during a 1-week period in July 2014. The empirical survey tool consisted of 27 questions and collected data on 5 principal domains, including 1) teacher demographics, 2) rules and regulations on water intake and bathroom use during classroom hours, 3) characteristics of school bathrooms in terms of safety, supervision and suitability for use, 4) experience with and management of students with daytime incontinence and 5) training on the topic of lower urinary tract health. Predictors for promoting lower urinary tract health were identified by multivariable logistic regression. RESULTS: Of the 4,166 teachers who completed the survey 88% indicated that they encourage students to hold urine. Despite strict bathroom protocols 81% of teachers allowed children unlimited access to water. Of the teachers 82% reported never having undergone any professional development on bathroom regulations for children. Overall only 24% of surveyed teachers met criteria for promoting lower urinary tract health. The odds of promoting lower urinary tract health decreased with ascending grade level (OR 0.80, 95% CI 0.76-0.84). Conversely it increased if teaching experience was greater than 5 years (OR 1.66, 95% CI 1.39-1.98) or professional development on the subject had been received (OR 1.42, 95% CI 1.18-1.70). CONCLUSIONS: Of elementary school teachers 76% are not promoting lower urinary tract health in school-aged children. Professional development training on the topics of lower urinary tract dysfunction and/or lower urinary tract health may be beneficial, particularly for educators who teach higher grades and those with less teaching experience.

Risk Factors Predicting Cellulitis Diagnosis in a Prospective Cohort Undergoing Dermatology Consultation in the Emergency Department.

Cellulitis is an infection of the skin and skin-associated structures with many clinical mimickers known collectively as pseudocellulitis. Dermatology or infectious disease consultation is considered the gold standard for diagnosis. We evaluated a prospective cohort of adult patients presenting to the emergency department (ED) with concern for lower extremity cellulitis who received dermatology consultation with conferral of a final diagnosis. Possible risk factors independently associated with cellulitis diagnosis (P<.1) were included in a logistic regression model for prediction of cellulitis diagnosis. Factors having odds ratios with a confidence interval excluding 1 were identified as significant independent predictors. The study identified factors that should be considered in evaluation of patients with suspected uncomplicated lower extremity cellulitis.

Diffuse Reflectance Spectroscopy with Infrared Thermography for Accurate Prediction of Cellulitis.

Cellulitis is frequently misdiagnosed owing to its clinical mimickers, collectively known as pseudocellulitis. This study investigated diffuse reflectance spectroscopy (DRS) alone and in combination with infrared thermography (IRT) for the differentiation of cellulitis from pseudocellulitis. A prospective cohort study at an urban academic hospital was conducted from March 2017 to March 2018. Patients presenting to the emergency department with presumed cellulitis were screened for eligibility, and 30 adult patients were enrolled. Dermatology consultation conferred a final diagnosis of cellulitis or pseudocellulitis. DRS measurements yielded a spectral ratio between 556 nm (deoxyhemoglobin peak) and 542 nm (oxyhemoglobin peak), and IRT measurements yielded temperature differentials between the affected and unaffected skin. Of the 30 enrolled patients, 30% were diagnosed with pseudocellulitis. DRS revealed higher spectral ratios in patients with cellulitis (P = 0.005). A single parameter model using logistic regression on DRS measurements alone demonstrated a classification accuracy of 77.0%. A dual parameter model using linear discriminant analysis on DRS and IRT measurements combined demonstrated a 95.2% sensitivity, 77.8% specificity, and 90.0% accuracy for cellulitis prediction. DRS and IRT combined diagnoses cellulitis with an accuracy of 90%. DRS and IRT are inexpensive and noninvasive, and their use may reduce cellulitis misdiagnosis.

Patch Testing With an Extended Metal Allergen Series at the Massachusetts General Hospital (2006-2017).

BACKGROUND: Reports of patch test data with an extended metal series that includes rare metals are limited. OBJECTIVE: The aims of the study were to analyze and report patch testing results from an extended metal series, examine associations with sex and age, and highlight concomitant metal reactions. METHODS: This study is a retrospective review of 150 patients referred for suspected metal allergy from January 1, 2007, to December 31, 2016. RESULTS: The most common indications for evaluation referral were those having symptoms after implantation of a metal device (55.3%) and those with a history and concern of metal allergy before implantation of a metal device (22.0%). One or more positive patch test reactions were observed in 87 patients (58.0%). Metals with the highest frequencies were nickel sulfate 2.5% (26.2%), gold sodium thiosulfate 0.5% (23.0%), gold sodium thiosulfate 2.0% (20.7%), palladium chloride 2.0% (19.6%), cobalt chloride 1.0% (12.0%), and manganese chloride 2.0% (10.1%). Of the 45 metals tested, 15 caused no patch test reactions. Female patients were more likely to be sensitized to nickel, gold, and palladium (P < 0.05). Younger patients (≤40 years) had higher reaction rates to nickel, mercury, palladium, and cobalt. Concomitant reactions of the top metals (nickel, palladium, gold, and cobalt) were statistically associated bidirectionally (P < 0.05), except for cobalt and gold. CONCLUSIONS: Allergy to metals, including those not included in standard series, may be more prevalent than previously suspected. Results may help guide future testing for suspected metal allergy, although future studies are warranted.

Renal dysfunction as an independent predictor of outcome after aneurysmal subarachnoid hemorrhage: a single-center cohort study.

BACKGROUND AND PURPOSE: Acute kidney injury occurs in 1% to 25% of critically ill patients with small increases in creatinine adversely affecting outcome. We sought to determine the burden of acute kidney injury in patients with aneurysmal subarachnoid hemorrhage and whether this dysfunction affects outcome. METHODS: Between 1996 and 2008, 787 consecutive patients with aneurysmal subarachnoid hemorrhage were enrolled in our prospective database. Demographics, serum creatinine levels, and discharge modified Rankin scores were recorded, and changes in creatinine clearance were calculated. A multiple logistic regression was performed using known predictors for poor outcome after aneurysmal subarachnoid hemorrhage in addition to burden of contrast-enhanced imaging and change in creatinine clearance. RESULTS: One hundred seventy-nine (23.1%) patients were at risk for renal failure during their hospitalization. In a multivariate model, those patients who developed risk for renal failure were twice as likely to have a poor 3-month outcome (OR, 2.01; P=0.021). Survival curves comparing those not at risk, those at risk (increasing severity classes Risk, Injury, and Failure, and the 2 outcome classes Loss and End-Stage Kidney Disease [RIFLE] R), and those with renal injury or failure (RIFLE I and F) demonstrated that risk of death increases significantly as one progresses through the RIFLE classes (log rank, P<0.0001). CONCLUSIONS: In a large, consecutive series of prospectively enrolled patients with aneurysmal subarachnoid hemorrhage, we demonstrate, using the newly defined RIFLE classification for risk of renal failure, that even seemingly insignificant decreases in creatinine clearance are associated with significantly worse 3-month outcomes. This study highlights the importance of close surveillance of renal function and stresses the value of renal hygiene in the aneurysmal subarachnoid hemorrhage population.

Clinical outcomes and molecular profile of patients with Carmi syndrome: A systematic review and evidence quality assessment.

PURPOSE: Carmi syndrome is a rare genetic disorder characterized by junctional epidermolysis bullosa (JEB) and pyloric atresia (PA). We reviewed the clinicopathologic and molecular features of patients with Carmi syndrome to identify predictors of clinical outcome and guide surgical PA repair. METHODS: A PRISMA-compliant systematic literature review of PubMed, CINAHL, and the Cochrane Library was performed. RESULTS: 63 original studies including a total of 100 patients were included. PA type 1 and 2 were equally prevalent (47.2%, 95% CI: 34.4-60.3). Heineke-Mikulicz pyloroplasty (96%, 95% CI: 78.8-99) and gastroduodenostomy (72%, 95% CI: 52.2-85.9) were the most common type 1 and 2 PA repairs, respectively. Seventy lethal cases were identified (74.5%, 95% CI: 64.8-83.5). Of the 73 patients that received an operation, 49 died (67.1%, 95% CI: 55.7-76.8) and 24 survived (32.9%, 95% CI: 23.2-44.3). Integrin α6ß4 expression was absent or markedly reduced in lethal cases. Integrin α6, plectin-1, cephalic integrin ß4 (exon 3 to intron 11), and premature termination codon mutations were also associated with poor prognosis. CONCLUSIONS: Although Carmi syndrome typically has poor prognosis, 1 in 4 patients exhibits nonlethal phenotypes. Immunofluorescence mapping and genetic consultation can guide surgical intervention and provide valuable family planning information. EVIDENCE RATING/CLASSIFICATION: Prognosis study, Level IV.

Teaching & Learning Tips 5: Making lectures more "active".

Challenge: Although lecturing is an efficient method for the dissemination of information, it has long been criticized for learner passivity and diminished knowledge retention. Active learning strategies to engage the audience in the learning process can facilitate a bidirectional flow of ideas and content between teacher and students during a lecture to keep learners engaged and participating.

Dermatology hospitalists: a multicenter survey study characterizing the infrastructure of consultative dermatology in select American hospitals.

BACKGROUND: Although considered an outpatient specialty, dermatology plays an important role in inpatient medicine. We characterized the activity and structure of dermatology consultation services in select U.S. hospitals. METHODS: In this cross sectional study, a 31-question survey was distributed in person to 32 board-certified dermatologists at the 2017 Society of Dermatology Hospitalists meeting. RESULTS: Thirty participants completed the survey (yield 93.8%). Most dermatology hospitalists spend 41-52 weeks on service (50%), with 37% spending between 11 and 30 weeks. Coverage was organized by continuous weeks (68%) or months (21%). While on service, hospitalists staffed an average of 4 outpatient clinics per week. Consultative teams also included internal medicine residents (43%), medical students (47%), pediatric residents (10%), and fellows from other specialties (27%). Consultation services saw approximately 3.7 new inpatients and 4.2 follow-up inpatients per day, with daily rounds lasting approximately 2.6 hours. CONCLUSIONS: The results suggest that hospital dermatologists in the U.S. consider inpatient care their niche and devote a majority of their time staffing consults over clinic. The diverse composition of inpatient teams and the number of academic duties held by these physicians suggests they play an important role in medical education.