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    Date Issued2021 (1)2020 (1)2016 (2)Author
    St. John, Jessica (4)
    Chakrala, Teja (2)Dobry, Allison S. (2)Ko, Lauren N. (2)Kroshinsky, Daniela (2)View MoreUMass Chan AffiliationDepartment of Dermatology (2)School of Medicine (2)Department of Medicine, Division of Dermatology (1)Department of Pediatrics, Division of Dermatology (1)Department of Pediatrics, Division of Genetics (1)View MoreDocument TypeJournal Article (4)KeywordDermatology (4)calciphylaxis (2)Nutritional and Metabolic Diseases (2)Pathological Conditions, Signs and Symptoms (2)risk factors (2)View MoreJournalDermatologic surgery : official publication for American Society for Dermatologic Surgery [et al.] (1)JAAD case reports (1)Pediatric dermatology (1)

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    The Koebner phenomenon may contribute to the development of calciphylaxis: A case series

    Gabel, Colleen K.; Chakrala, Teja; Dobry, Allison S.; Garza-Mayers, Anna Cristina; Ko, Lauren N.; Nguyen, Emily D.; Shah, Radhika; St. John, Jessica; Nigwekar, Sagar U.; Kroshinsky, Daniela (2021-04-28)
    Calciphylaxis is characterized by calcific occlusion of vessels and subsequent tissue ischemia due to thrombosis. The precise pathogenetic mechanism behind calciphylaxis remains unclear. In the original experiment by Hans Selye and colleagues, soft-tissue calcification was induced in rats by applying a sensitizing agent, followed by a “challenger” agent after a specific time period. Trauma may represent one of these “challenger” agents, serving as an inducer of endothelial dysfunction and subsequent thrombosis, leading from tissue calcification to calciphylaxis. Koebnerization, a term used to describe the appearance of isomorphic lesions in areas of trauma, has been postulated to be a feature of calciphylaxis. This hypothesis arose from reports of patients who developed calciphylaxis following mild skin trauma, such as that caused by chronic resting of elbows on thighs, placement of ice packs, and injections involving various medications such as iron dextran, tobramycin, and especially insulin. Rigorous studies demonstrating the relationship between calciphylaxis and Koebnerization and an underlying mechanism are limited. To better understand this association, this study retrospectively identified characteristics of patients who presented with calciphylaxis in areas of trauma, suggesting the presence of Koebnerization.
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    Penile calciphylaxis: A retrospective case-control study

    Gabel, Colleen; Chakrala, Teja; Shah, Radhika; Danesh, Melissa J.; Dobry, Allison S.; Garza-Mayers, Anna Cristina.; Ko, Lauren N.; Nguyen, Emily; St. John, Jessica; Walls, Andrew C.; et al. (2020-05-15)
    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.
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    Multiple Cafe au Lait Spots in a Group of Fair-Skinned Children without Signs or Symptoms of Neurofibromatosis Type 1

    St. John, Jessica; Summe, Heather S.; Csikesz, Courtney; Wiss, Karen; Hay, Beverly N.; Belazarian, Leah (2016-09-01)
    BACKGROUND: The presence of six or more cafe au lait (CAL) spots is a criterion for the diagnosis of neurofibromatosis type 1 (NF-1). Children with multiple CAL spots are often referred to dermatologists for NF-1 screening. The objective of this case series is to characterize a subset of fair-complected children with red or blond hair and multiple feathery CAL spots who did not meet the criteria for NF-1 at the time of their last evaluation. METHODS: We conducted a chart review of eight patients seen in our pediatric dermatology clinic who were previously identified as having multiple CAL spots and no other signs or symptoms of NF-1. RESULTS: We describe eight patients ages 2 to 9 years old with multiple, irregular CAL spots with feathery borders and no other signs or symptoms of NF-1. Most of these patients had red or blond hair and were fair complected. All patients were evaluated in our pediatric dermatology clinic, some with a geneticist. The number of CAL spots per patient ranged from 5 to 15 (mean 9.4, median 9). CONCLUSION: A subset of children, many with fair complexions and red or blond hair, has an increased number of feathery CAL spots and appears unlikely to develop NF-1, although genetic testing was not conducted. It is important to recognize the benign nature of CAL spots in these patients so that appropriate screening and follow-up recommendations may be made.
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    Avoiding Medical Errors in Cutaneous Site Identification: A Best Practices Review

    St. John, Jessica; Walker, Jennifer; Goldberg, Dori; Maloney, Mary E. (2016-04-01)
    BACKGROUND: Although the field of dermatology has a relatively low incidence of medical errors, dermatologic surgery is a major area where medical errors occur. OBJECTIVE: The purpose of this article is to catalog the many cutaneous site identification techniques used by practitioners and determine which techniques are most evidence based. MATERIALS AND METHODS: A comprehensive literature review of cutaneous surgical site identification techniques and medical errors in dermatology. RESULTS: Wrong-site surgery often occurs because of an inability to identify the surgical site because of factors such as inadequate documentation from referring physicians, well-healed scars obscuring the biopsy site, and a patient's inability to visualize the surgical site. Practitioners use techniques such as photography, dermabrasion, written descriptions using anatomic landmarks, and site identification protocols for surgical site identification. CONCLUSION: Site identification remains a challenge for dermatologists and is a leading cause of medical errors in this field. Patients are often unreliable in their ability to identify biopsy sites; therefore, practitioners must take a proactive role to ensure that medical errors do not occur. This article provides a thorough description and evaluation of current site identification techniques used in dermatology with the aim to improve quality of care and reduce medical errors.
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