Matthew Keller

Ciclosporin Use During Pregnancy

Ciclosporin (cyclosporine) is an immunosuppressive drug first approved for use in organ transplantation to prevent rejection. Ciclosporin is also known to be used for the treatment of psoriasis, rheumatoid arthritis, systemic lupus erythematosus and inflammatory bowel disease, among other indications. While it is recommended that all medications that are not absolutely necessary should be avoided during pregnancy, this may not be an option for many women whose quality of life is significantly impacted without treatment, or for those who must continue immunosuppressive therapy to avoid organ rejection. The purpose of this review is to provide a comprehensive report from the literature of ciclosporin exposure during pregnancy. PubMed, MEDLINE and the Cochrane Database of Systematic Reviews were searched for English-language articles published from 1970 to 2012 that included reports of pregnant women treated at any time during pregnancy with ciclosporin. On an initial search, it was evident that much of the available information is limited to pregnancy after transplant, which suggests that ciclosporin use during pregnancy appears to be associated with premature delivery and low birthweight infants. Comorbidities such as hypertension, pre-eclampsia and gestational diabetes mellitus are also reported at higher incidences than the general population. Medical literature concerning women with autoimmune disorders exposed to ciclosporin during pregnancy are currently limited to case reports and registry data, and, as such, it is difficult to determine if any risks associated with ciclosporin therapy during pregnancy are due to exposure to the drug alone or to pre-existing maternal comorbidities. The literature suggests that ciclosporin therapy during pregnancy should be carefully considered by the treating physician, but may be a safe alternative for patients with autoimmune disease refractory to conventional treatment. Continued monitoring of this patient population remains a key component to understanding the risk factors associated with ciclosporin exposure during pregnancy.

Epidemiologic, Clinicopathologic, Diagnostic, and Management Challenges of Pityriasis Rubra Pilaris: A Case Series of 100 Patients

IMPORTANCE Pityriasis rubra pilaris (PRP) is a rare papulosquamous disorder with limited epidemiologic and clinicopathologic data. Little information is available on long-term outcomes, comorbidities, and treatment efficacy. OBJECTIVE To evaluate objective and subjective disease experience metrics from the perspectives of patients and clinicians. DESIGN, SETTING, AND PARTICIPANTS One hundred patients with a putative diagnosis of PRP and who elected to participate completed a comprehensive survey, followed by acquisition of their medical records, including histopathology slides and reports. The data were analyzed separately from the health care clinician and the patient perspectives. Two academic dermatologists examined clinical notes, pathology reports, and photographs, confirming diagnoses via predetermined criteria. Patients were categorized into 4 levels of diagnostic certainty to allow stratification of the findings for subgroup analysis. Patients with a diagnosis of PRP were solicited through patient support organization websites. MAIN OUTCOMES AND MEASURES Clinical outcomes, unexpected association of comorbidities, and efficacy (or lack of it) of various treatment modalities. RESULTS Among the 100 patients, 50 were diagnosed as having classic, unquestionable PRP. The patients were a median of 61 years old (range, 5-87 years), and 46% were female. Fifty were categorized as level 1 diagnostic certainty, 15 as level 2, 30 as level 3, and 5 as level 4. Of the level 1 patients, 13 (26%) were correctly diagnosed at initial presentation; diagnosis was delayed, on average, by 29 months (range, 0.25-288 months; median, 2 months); and 27 (54%) having undergone 2 or more biopsies. At enrollment, PRP symptoms had persisted in 36 patients (72%) for an average of 58 months (range, 1-300 months; median, 30 months). Thirty-one patients (62%) had comorbidities, including hypothyroidism (20%). Nearly all patients (98%) received some form of therapy. Patients cited topical emollients, corticosteroids, and salicylic acid along with oral retinoids, methotrexate, and tumor necrosis factor inhibitors as most helpful. CONCLUSIONS AND RELEVANCE Pityriasis rubra pilaris remains a challenging diagnosis without established and specific treatment. Our data highlight new potential avenues for research with therapeutic perspective.

Psoriasis Vulgaris: An Evidence-Based Guide for Primary Care

Psoriasis vulgaris is a chronic, sometimes debilitating, inflammatory disorder with multiple pathways of pathogenesis that can be associated with metabolic and cardiovascular disease. This article aims to be a comprehensive, literature-based review of the epidemiology, genetic factors, clinical diagnosis, treatments, and pharmacology for psoriasis as derived from articles published in PubMed. Levels of evidence and recommendations were made according to the strength of recommendation taxonomy. This article is divided into 2 sections: the first is clinical and diagnostic, the second is therapeutic. This review serves as a practical, evidence-based, and unbiased guide for primary care practitioners.

Analysis of CARD14 Polymorphisms in Pityriasis Rubra Pilaris: Activation of NF-κB.

SNP rs Variant Minor allele frequency cDNA Codon Amino acid rs9895931 c.27C>C/T TCC>TCT p.S9S T: 0.0% rs114688446 c.599G>G/A AGC>AAC p.S200N T: 0.8% rs4889990 c.633G>A GAG>GAA p.E211E A: 37.9% rs28674001 c.676-6G>A A: 37.4% rs142246283 c.683T>T/G CTA>CGA p.L228R no data rs61751629 c.1264G>A GAG>AAG p.E422K A: 2.1% rs11658460 c.1323C>T GAC>GAT p.D441D T: 10.2% rs34367357 c.1753G>A GTC>ATC p.V585I A: 5.2% rs2066964 c.1641G>C AGG>AGC p.R547S C: 65.1% rs117918077 c.2044C>C/T CGG>TGG p.R682W T: 1.2% rs11653893 c.2399-4A>G G: 40.4% no data c.2406C>C/A AGC>AGA p.S802R no data rs11652075 c.2458C>T CGG>TGG p.R820W T: 39.9% rs61757652 c.2481C>T CCC>CCT p.P827P T: 6.2% rs139789664 c.2495C>T, CTC>CTT, p.L832L T: 0.5%

Mohs Micrographic Surgery for Hidradenocarcinoma on a Rhinophymatous Nose: A Histologic Conundrum

Nodular hidradenoma (NH) is a rare tumor of the eccrine sweat glands, first described by Keasbey and Hadley in 1954. NH usually presents as a nonspecific dermal nodule on the head and neck but may also be seen on the trunk and extremities. NH can be difficult to distinguish histologically from its malignant counterpart, nodular hidradenocarcinoma (HC). Originally called a clear cell eccrine carcinoma, HC has been variably termed malignant acrospiroma, malignant clear cell hidradenoma, and clear cell hidradenocarcinoma. NH and HC are characterized by lobular aggregations of cells in the dermis with variable proportions of basaloid polyhedral and round clear cells. Lumina and cystic spaces may also be seen, as well as foci of keratinization. The presence of cellular atypia and an infiltrative pattern suggest HC.

Outdated dermatologic drug samples and obligations to the patient

A 37-year-old man with a slightly raised, red, scaly patch on his left arm is seen in the clinic of an academic teaching hospital by a young dermatologist. Following examination and biopsy, he is found to have squamous cell carcinoma in situ. The patient was recently laid off fromwork and had lost access to his health insurance. He is struggling to make ends meet for his family, so it would be difficult for him to cover any extraneous health-related expenses. Upon relaying this information to the dermatologist, the patient is provided with a full course of 5-fluorouracil (topical cream 5%) from the clinic’s sample cabinet. Later in the day, it is discovered that the same batch of drugs has been outdated for more than 4 months. Because the dermatologist was only recently hired, she worries that she will be severely reprimanded for her failure to inspect the expiry date.

Resident experiences with social media: modernizing dermatology training

nantly lymphocytic, infiltrate in the dermis. Clinical and histological findings are quite different from those of our case; however, endothelial cells in the skin may not function properly because of long-term exposure to VEGFR inhibitor. Furthermore, intravascular occlusive fibrin thrombi were observed, and reparative perivascular fibrosis led to the development of CCV. It is possible that various stimulations, such as an anticancer drug and thrombi, caused microinjury and dysfunction of the small blood vessels of the skin and clinically represented telangiectasia.

Business administration training for dermatology residents: Preparing for the business of medicine

Previous studies have evidenced the lack of practice management and business training components in the residency curriculum, and that satisfaction with this training, when provided, was low. Whether considered good or bad, medicine has been moving increasingly toward becoming more business centric. Dermatology represents a unique field, because most residents choose to pursue private practice, where competent business skills are helpful to running a successful clinic. Our study examines the current state of business administration training for dermatology residents in Accreditation Council for Graduate Medical Education-approved programs as of 2017 to 2018. Our results show that residents both value and desire business administration training; however, the current training provided is considered to be insufficient and inadequate. Promoting and encouraging the teaching of business administration skills, while still balancing core competencies, may help to prepare residents in navigating the future of our field.

An investigational study to assess patient beliefs and informational sources regarding the safety and use of sunscreen

To the Editor, In recent years, the media has paid increasing attention to the safety and efficacy of sunscreens, generating confusion and fear in the general public and prompting comments from the American Academy of Dermatology and Skin Cancer Foundation. A prominent nonprofit organization has made public claims that sunscreens are toxic, can alter hormone levels, and cause birth defects and cancer, which have been perpetuated by the media. To ascertain the impact of these media claims on public opinion, we conducted a survey of 109 patients in a general dermatology clinic. Our results indicate that a majority of patients do not know about the validity of these assertions. In this article, we briefly review the relevant research behind these claims.

Language barriers to informed consent for dermatologic interventions

A n ethical dilemma may present itself when dermatologistsmust obtain informed consent from patients with limited English-language proficiency. With an increasingly diverse populationeboth culturally and linguisticallyeand the significant role that biopsies play in our field, it is crucial that dermatologists learn how to navigate this ethical quandary through recognizing the importance of using trained medical interpreters rather than untrained, biased, and/or minor individuals, eg, most