Lauren N. Ko

Association Between Hypercoagulable Conditions and Calciphylaxis in Patients With Renal Disease: A Case-Control Study

Importance Calciphylaxis is a rare skin disease with high morbidity and mortality that frequently affects patients with renal disease. Hypercoagulable conditions are frequently observed in both patients with calciphylaxis and those with chronic kidney disease (CKD), complicating our understanding of which hypercoagulable conditions are specific to calciphylaxis. Objective To identify hypercoagulable conditions that are risk factors for developing calciphylaxis while controlling for CKD. Design, Setting, and Participants This was a case-control study, comparing the hypercoagulability status of patients with calciphylaxis and with renal disease with that of a matched control population at 2 large urban academic hospitals in Boston, Massachusetts. Retrospective medical record review of laboratory values was performed to identify patients with hypercoagulable conditions. Case and control patients were further stratified based on both severity of CKD and warfarin. Patients with a dermatologic diagnosis of calciphylaxis between 2006 and 2014 and concomitant CKD were included as cases (n = 38). Three controls (n = 114) per case patient with CKD were included, and were matched by age, sex, and race. Main Outcomes and Measures The rate of various hypercoagulable states (ie, antithrombin III [ATIII] deficiency, protein C and S deficiency, factor V Leiden mutation, prothrombin gene mutation [G20210A], elevated factor VIII level, lupus anticoagulant, anti-IgG or IgM cardiolipin antibodies, heparin-induced thrombocytopenia antibodies, and elevation of homocysteine) in patients with calciphylaxis compared with their matched controls. Results Of the calciphylaxis cohort, 28 (58%) were female and 18 (55%) were non-Hispanic white. Among all patients, lupus anticoagulant (13 [48%] positive in cases vs 1 [5%] in controls; P = .001), protein C deficiency (9 [50%] vs 1 [8%]; P = .02), and combined thrombophilias (18 [62%] vs 10 [31%]; P = .02) were found to be significantly associated with calciphylaxis. In a subanalysis of patients with stage 5 CKD, only lupus anticoagulant (12 [53%] vs 9 [0%]; P = .01) and combined thrombophilia (15 [63%] vs 1 [8%]; P = .004) remained significantly associated with calciphylaxis. In a separate subanalysis of warfarin-unexposed patients, only lupus anticoagulant (7 [50%] vs 1 [6%]; P = .01) and protein C deficiency (5 [46%] vs 10 [0%]; P = .04) remained significantly associated with calciphylaxis. Conclusions and Relevance Presence of lupus anticoagulant and combined thrombophilias are risk factors for the development of calciphylaxis in patients with late-stage renal disease. Clinicians should be aware of these associations in patients with impaired kidney function and may consider increased screening and appropriate anticoagulation treatment to reduce the risk of calciphylaxis development.

Effect of Dermatology Consultation on Outcomes for Patients With Presumed Cellulitis: A Randomized Clinical Trial

Importance Each year, cellulitis leads to 650 000 hospital admissions and is estimated to cost

Clinical Usefulness of Imaging and Blood Cultures in Cellulitis Evaluation

3.7 billion in the United States. Previous literature has demonstrated a high misdiagnosis rate for cellulitis, which results in unnecessary antibiotic use and health care cost. Objective To determine whether dermatologic consultation decreases duration of hospital stay or intravenous antibiotic treatment duration in patients with cellulitis. Design, Setting, and Participants This randomized clinical trial was conducted in a large urban tertiary care hospital between October 2012 and January 2017, with 1-month follow-up duration. Patients were randomized to the control group, which received the standard of care (ie, treatment by primary medicine team), or the intervention group, which received dermatology consultation. Medical chart review of demographic information and hospital courses was performed. Adult patients hospitalized with presumed diagnosis of cellulitis were eligible. A total of 1300 patients were screened, 1125 were excluded, and 175 were included. Statistical analysis was employed to identify significant outcome differences between the 2 groups. Interventions Dermatology consultation within 24 hours of hospitalization. Main Outcomes and Measures Length of hospital stay and duration of intravenous antibiotic treatment. Results Of 175 participants, 70 (40%) were women and 105 (60%) were men. The mean age was 58.8 years. Length of hospital stay was not statistically different between the 2 groups. The duration of intravenous antibiotic treatment (<4 days: 86.4% vs 72.5%; absolute difference, 13.9%; 95% CI, 1.9%-25.9%; P = .04) and duration of total antibiotic treatment was significantly lower in patients who had early dermatology consultation (<10 days: 50.6% vs 32.5%; absolute difference, 18.1%; 95% CI, 3.7%-32.5%; P = .01). Clinical improvement at 2 weeks was significantly higher for those in the intervention group (79 [89.3%] vs 59 [68.3%]; absolute difference, 21.0%; 95% CI, 9.3%-32.7%; P < .001). There was no significant difference in 1-month readmission rate between the groups (4 [4.5%] vs 6 [6.9%]; absolute difference, −2.4%; 95% CI, −9.3% to 4.5%; P = .54). In the intervention group, the rate of cellulitis misdiagnosis was 30.7% (27 of 88 participants). Among the entire cohort, 101 (57.7%) patients were treated with courses of antibiotics longer than what is recommended by guidelines. Conclusions and Relevance Early dermatologic consultation can improve outcomes in patients with suspected cellulitis by identifying alternate diagnoses, treating modifiable risk factors, and decreasing length of antibiotic treatment. Trial Registration clinicaltrials.gov Identifier: NCT01706913

Hypersensitivity to Hip and Knee Implants

volve a shorter duration of antibiotic exposure.6 Our study has limitations. First, we were not able to account for underlying conditions or other indicators for longer courses of antibiotic therapy. Second, by excluding visits with a diagnosis of unspecified sinusitis from our analysis, we may have excluded some cases of acute sinusitis. However, the findings of a sensitivity analysis that included cases of both acute and unspecified sinusitis were similar (median duration of therapy, 10.0 days; 88.1% [95% CI, 85.1%-91.0%] of nonazithromycin antibiotic courses were 10 days or longer in duration). Outpatient antibiotic stewardship programs can optimize infection management by ensuring guideline-concordant treatment, including the use of minimum effective durations of antibiotic therapy. The durations of most courses of antibiotic therapy for adult outpatients with sinusitis exceed guideline recommendations, which represents an opportunity to reduce the unnecessary use of antibiotics when therapy with antibiotics is indicated.

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

Prosthetic hip and knee implantations rank among the most common elective operations in the United States and Europe. These implants are compositionally complex and have undergone drastic evolutions over the past several decades. Hypersensitivity reactions to hip and knee implant components are well documented in the literature but remain uncommon. They range from localized dermatitis to implant loosening and subsequent failure. Currently, patch testing is the most useful method to evaluate for metal allergy. Preimplantation testing is not necessary unless patients have a significant history of skin rashes after metal contact or previously have experienced device failure. Postoperative complications such as aseptic loosening, chronic pain, or new, unexplained local or regional dermatitis may occasionally benefit from evaluation for metal allergy. Clinical next steps following implantation may be challenging. If the implant is symptomatic, topical or systemic corticosteroids may be tried, but ultimately, the surgeon and patient may need to decide whether replacement with a less allergenic alternative is feasible and safe. Whether pre- or postoperative, clinical decisions should be made by the collaborative efforts of dermatologists, allergists, and surgeons. Prospective trials in this field are still necessary to develop an evidence-based approach to the treatment of patients with allergic reactions to metal, as current clinicians are guided largely by expert opinion.

Assessing the validity of self-reported history of rash caused by metal or jewellery: VALIDITY OF RASH HISTORY IN METAL ALLERGY

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 α6β4 expression was absent or markedly reduced in lethal cases. Integrin α6, plectin-1, cephalic integrin β4 (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.

Fractures in calciphylaxis patients following intravenous sodium thiosulfate therapy

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.

A 25-Year-Old Male with Orogenital Ulcers, Rash, and Difficulty Swallowing

Calciphylaxis is a highly fatal syndrome of small vessel calcification that results in skin ischemia and necrosis.[1] Affected patients present with extremely painful, violaceous skin lesions that progress to non-healing ulcers, and can be complicated by superimposed infections and sepsis.[2] The mortality rate from calciphylaxis is estimated at 60-80%.[2, 3] Sodium thiosulfate (STS), an increasingly popular treatment for calciphylaxis, is theorized to work through vasodilation, antioxidation, and calcium chelation. Known adverse effects include volume overload, metabolic acidosis, and hypotension.[2, 4] We report four patients who experienced unusual fractures during or following treatment with intravenous STS (IVSTS), an adverse effect that has not yet been reported in the literature. This article is protected by copyright. All rights reserved.

Skin Surface Temperatures Measured by Thermal Imaging Aid in the Diagnosis of Cellulitis

A 25-year-old otherwise healthy male presented with new-onset odynophagia, rash, and orogenital ulcers. Despite treatment with antibiotics for presumed bacterial pharyngitis, the patient remained symptomatic, with abnormal vital signs and laboratory values. Upon dermatology consultation and histopathologic correlation, he was diagnosed with Behçet disease. Behçet disease is a rare rheumatologic condition that presents with recurrent oral ulcers and varying degrees of ophthalmic, neurologic, cardiac, and vascular disease. Given its protean nature, the treatment of Behçet disease is tailored to the patients presentation and severity of organ involvement. Following treatment with colchicine and prednisone, the patients symptoms improved rapidly.