Horrifying Basal Cell Carcinoma Presenting as Progressive Pyoderma Gangrenosum: Case Report.

Introduction: Skin ulcers can be challenging to diagnose and manage, particularly with comorbid autoimmune and gastrointestinal diseases. Occam's razor encourages the simplest explanation to guide care, but reconsideration must occur when intervention proves futile. Case Presentation: We report the case of a 70-year-old male, with a 17-year history of expanding pretibial skin ulcer, presumed by prior care providers to be pyoderma gangrenosum related to Crohn's disease. A surgical biopsy performed upon presentation to our institution revealed basal cell carcinoma of the skin, invasive to the proximal tibia with associated deep infection, prompting transfemoral amputation. Conclusion: This report is written as a reminder to reconsider a diagnosis and consider seeking additional expertise when a patient's condition progressively worsens despite intervention. Earlier diagnosis likely would have facilitated therapeutic limb salvage care.

Staging System Performance and Clinical Outcomes for Cutaneous Squamous Cell Carcinoma of the Ear: A Single-Center Retrospective Study.

BACKGROUND: Cutaneous squamous cell carcinoma (cSCC) of the ear is associated with poor outcomes. No studies have evaluated current staging system performance in this specific location. OBJECTIVE: Describe clinicopathologic characteristics and outcomes of ear cSCC and evaluate the performance of current staging systems. METHODS: Retrospective study including cases diagnosed and treated at a cancer center from January 2000 to December 2014. Demographic, clinical, and pathologic data were collected from clinical records. Biopsy slides were rereviewed and patients were staged according to the American Joint Committee on Cancer (AJCC) seventh, eighth, and Brigham Women's Hospital (BWH) staging. RESULTS: Of 125 patients, the mean age at diagnosis was 71.9 years (SD 12.5), with most men (89.6%, n = 112). Median follow-up was 22.3 months. Local recurrence and survival risk factors were similar to cSCC outside the ear. The Akaike's Information Criterion (AIC) estimates showed that the BWH system better predicted outcomes than the AJCC seventh, and the AJCC eighth, with AIC values of 189.9, 270.5, and 274.1, respectively. Limitations of the study include retrospective design, single center study, and no control group. CONCLUSION: Current staging systems perform well at stratifying risk in ear cSCC.

Association of interleukin-6 and tumor necrosis factor-α with mortality in hospitalized patients with cancer.

BACKGROUND: Severe cutaneous adverse reactions (SCARs) are associated with high morbidity and mortality in patients with cancer. Early identification and treatment of SCARs may improve outcomes. OBJECTIVE: To identify biomarkers to predict outcomes in hospitalized patients with cancer who developed SCARs. METHODS: Retrospective review of 144 hospitalized patients with cancer with a morbilliform rash, recorded testing for serum cytokines (interleukin [IL]-6, IL-10, and tumor necrosis factor [TNF]-α) or elafin, and a dermatology consultation. Rashes were categorized as simple morbilliform rash without systemic involvement or complex morbilliform rash with systemic involvement. RESULTS: Fifty-four of 144 (37.5%) patients died during follow-up. Elevated levels of IL-6, IL-10, and TNF-α were associated with decreased survival. Overall survivals in patients with elevated levels of IL-6, IL-10, and TNF-α were 53.7%, 56.6%, 53.6%, respectively, compared with 85.7%, 82.5% and 83.6%, respectively, in those with lower levels. Patients with increased levels of both IL-6 and TNF-α had a nearly 6-fold increase in mortality (hazard ratio, 5.82) compared with patients with lower levels. LIMITATIONS: Retrospective design, limited sample size, and high-risk population. CONCLUSIONS: Hospitalized patients with cancer with rash and elevated IL-6 and TNF-α were nearly 6 times more likely to die over the course of follow-up. These biomarkers may serve as prognostic biomarkers and therapeutic targets for this high-risk population.

Patient Concerns in the Immediate Postoperative Period After Mohs Micrographic Surgery.

BACKGROUND: Postoperative concerns after Mohs micrographic surgery (MMS) are not well characterized. OBJECTIVE: To better define patient concerns and contributing characteristics in the immediate postoperative period after MMS. MATERIALS AND METHODS: A standardized telephone encounter template was implemented to better assess patient concerns in the 24-hour postoperative period. A review was then performed of patients undergoing MMS from October 2016 to July 2017 to assess for the most common patient concerns and association with clinical characteristics. RESULTS: We included 307 patients. Overall, 60.6% of patients reported a concern. Fifty-four percent of patients reported pain. Most pain was characterized as "a little" (67.7%). On univariate analysis, flap repairs, location on the upper extremities, and swelling were associated with higher mean postoperative pain. Tumor type was not associated with increased pain. On multivariate analyses, patients with larger defects and associated edema were statistically significantly associated with higher degrees of pain. CONCLUSION: Over half of patients experience postoperative concerns after MMS, most commonly pain. The immediate postoperative period may be an optimal time to identify patient concerns allowing for reassurance or early intervention when necessary.

Appearance-related psychosocial distress following facial skin cancer surgery using the FACE-Q Skin Cancer.

Over 2 million facial skin cancers occur globally each year. Facial skin cancer surgery can leave scars that may alter appearance and impact psychosocial functioning. The objective of this study is to assess patient-reported appearance-related psychosocial distress following facial skin cancer surgery, and to identify independent predictors of psychosocial impairment. This was a single-center, cross-sectional study at a tertiary care cancer center including patients who underwent dermatologic surgery on the face from March 1, 2016 to March 31, 2018. Patients completed the FACE-Q Skin Cancer Appearance-related Psychosocial Distress scale postoperatively between May 21, 2018 and October 1, 2018. Patient responses were rated on a 4-point Likert scale and converted on a scale from 0 to 100. In total, 359 patients completed the questionnaire (34.2% response rate). Overall, patients reported a low level of psychosocial distress. Patients most frequently reported items of self-consciousness, unhappiness, and insecurity < 3 months following surgery. Though psychosocial distress significantly improved over time, self-consciousness continued to be reported in the long-term postoperative period. Linear regression analysis determined that younger age, history of anxiety and/or depression, surgery on the nose, and repair by flap were independently predictive of psychosocial distress. Marginal predicted values for distress scores based on age demonstrated an indirect relationship. Patient-reported appearance-related psychosocial distress is low following facial skin cancer surgery, and report of distress decreases over time. The identified predictors of distress may be used as indicators for offering psycho-oncologic support and early interventions to improve scar appearance.

Activation of Ralfuranone/Ralstonin Production by Plant Sugars Functions in the Virulence of Ralstonia solanacearum.

Plant pathogenic bacteria possess sophisticated mechanisms to detect the presence of host plants by sensing host-derived compounds. Ralstonia solanacearum, the causative agent of bacterial wilt on solanaceous plants, employs quorum sensing to control the production of the secondary metabolite ralfuranones/ralstonins, which have been suggested to be involved in virulence. Here, we report that d-galactose and d-glucose, plant sugars, activate the production of ralfuranones/ralstonins in R. solanacearum. As a result, two new derivatives, ralfuranone M (1) and ralstonin C (2), were found in the culture extracts, and their structures were elucidated by spectroscopic and chemical methods. Ralstonin C (2) is a cyclic lipopeptide containing a unique fatty acid, (2S,3S,Z)-3-amino-2-hydroxyicos-13-enoic acid, whereas ralfuranone M (1) has a common aryl-furanone structure with other ralfuranones. d-Galactose and d-glucose activated the expression of the biosynthetic ralfuranone/ralstonin genes and in part became the biosynthetic source of ralfuranones/ralstonins. Ralfuranones and ralstonins were detected from the xylem fluid of the infected tomato plants, and their production-deficient mutants exhibited reduced virulence on tomato and tobacco plants. Taken together, these results suggest that activation of ralfuranone/ralstonin production by host sugars functions in R. solanacearum virulence.

Markers of systemic involvement and death in hospitalized cancer patients with severe cutaneous adverse reactions.

BACKGROUND: Severe cutaneous adverse reactions (SCARs) are frequent in inpatient oncology. Early intervention might reduce morbidity, mortality, and hospitalization costs; however, current clinical and histologic features are unreliable SCAR predictors. There is a need to identify rational markers of SCARs that could lead to effective therapeutic interventions. OBJECTIVE: To characterize the clinical and serologic features of hospitalized patients with cancer who developed SCARs. METHODS: Retrospective review of 49 hospitalized cancer patients with a morbilliform rash, recorded testing for serum cytokines (interleukin [IL] 6, IL-10, and tumor necrosis factor [TNF] α) or elafin, and a prior dermatology consultation. Patients were categorized as having a simple morbilliform rash without systemic involvement or complex morbilliform rash with systemic involvement. RESULTS: Fifteen out of 49 patients (30.6%) were deceased at 6 months from time of dermatologic consultation. Elafin, IL-6, and TNF-α were significantly higher in patients who died compared with patients who were still alive at 6 months. IL-6 and IL-10 were significantly higher in patients with a drug-related complex rash. LIMITATIONS: Retrospective design, limited sample size, and high-risk patient population. CONCLUSION: In cancer patients with SCARs, elafin, IL-6, and TNF-α levels might predict a poor outcome. Agents directed against these targets might represent rational treatments for the prevention of fatal SCARs.

Imatinib Treatment for Locally Advanced or Metastatic Dermatofibrosarcoma Protuberans: A Systematic Review.

Importance: Dermatofibrosarcoma protuberans (DFSP) has the potential for local destruction and recurrence, although it carries a low risk of metastasis. Complete surgical resection with negative margins is considered the gold standard for treatment; however, there are cases that are unresectable owing to tumor extension or size or owing to risk of cosmetic and/or functional impairment. Imatinib treatment has been used for locally advanced or metastatic DFSP. Objective: To evaluate the usefulness of imatinib for treating DFSP. Evidence Review: We conducted a systematic review on the PubMed and Embase databases for articles published from September 2002 through October 2017 using the key words "dermatofibrosarcoma" or "dermatofibrosarcoma protuberans" AND "therapy" AND "imatinib." References within retrieved articles were also reviewed to identify additional studies. Studies of adults with histologically proven DFSP treated with imatinib as monotherapy or as an adjuvant or neoadjuvant therapy to surgery were included. Extracted data were analyzed using descriptive statistics. PRISMA guidelines were followed. All analysis took place October through December 2017. Findings: Nine studies met inclusion criteria; 152 patients were included. The calculated mean patient age was 49.3 years (range, 20-73 years). Calculated mean tumor diameter was 9.9 cm (range, 1.2-49.0 cm). When COL1A1-PDGFß protein translocation (collagen, type 1, alpha 1-platelet-derived growth factor ß) was reported, it was present in 90.9% of patients (111 of 122). Complete response was seen in 5.2% of patients (8 of 152), partial response in 55.2% (84 of 152), stable disease in 27.6% (42 of 152), and progression in 9.2% (14 of 152). Four of the 152 patients (2.6%) were excluded from the analysis owing to unknown or unevaluable response. There were no differences in response rate using 400-mg or 800-mg daily doses (67.5% or 27 of 40 patients for 400-mg dose vs 67.1% or 49 of 73 patients for 800-mg dose complete or partial response; P > .99). Adverse events were present in at least 73.5% of cases (78 of 106); severe adverse events were present in 15.1% of cases (20 of 132). Conclusions and Relevance: Imatinib is a useful directed therapy in patients with DFSP who are not surgical candidates owing to disease extension or significant cosmetic or functional impairment. There seems to be no difference between 400- or 800-mg daily doses.

Patient-reported Aesthetic Satisfaction following Facial Skin Cancer Surgery Using the FACE-Q Skin Cancer Module.

Over 5 million basal and squamous cell skin cancers are diagnosed each year. Seventy to 80% of these cancers occur in the head and neck region, for which surgical excision is the standard treatment. As patient satisfaction and quality of life are among the most important outcomes in plastic and reconstructive surgery, understanding patient perception of aesthetic postoperative outcome is critical. The objective of this study was to assess aesthetic satisfaction following facial skin cancer surgery using the FACE-Q Skin Cancer Module in the context of sociodemographic and clinical factors. METHODS: This is a single-center, cross-sectional study in a tertiary care cancer setting of patients who underwent facial skin cancer surgery from March 1, 2016, to March 31, 2018. Patients completed the FACE-Q Skin Cancer Satisfaction with Facial Appearance and Appraisal of Scar scales postoperatively, between May 21, 2018, and October 1, 2018. RESULTS: Patients completed the Satisfaction with Facial Appearance (n = 405) and Appraisal of Scar scales (n = 408) postoperatively (response rate 39%). Lower postoperative facial appearance and scar satisfaction scores were associated with female gender, younger age (<65 years), surgery location on the lip or nose, repair by flap or graft, and greater defect size. Linear regression models established that younger age, female gender, nose location, and flap repair were independently predictive of lower aesthetic satisfaction. CONCLUSIONS: Sociodemographic factors, central facial location, and repair type strongly contribute to aesthetic satisfaction following facial skin cancer surgery. This patient-reported data may guide counseling regarding postoperative aesthetic outcome and inform patient expectations.

Basal cell carcinoma: Epidemiology; pathophysiology; clinical and histological subtypes; and disease associations.

As the most common human cancer worldwide and continuing to increase in incidence, basal cell carcinoma is associated with significant morbidity and cost. Continued advances in research have refined both our insight and approach to this seemingly ubiquitous disease. This 2-part continuing medical education article will provide a comprehensive and contemporary review of basal cell carcinoma. The first article in this series describes our current understanding of this disease regarding epidemiology, cost, clinical and histopathologic presentations, carcinogenesis, natural history, and disease associations.

Basal cell carcinoma: Contemporary approaches to diagnosis, treatment, and prevention.

As the most common human cancer worldwide and continuing to increase in incidence, basal cell carcinoma is associated with significant morbidity and cost. Continued advances in research have refined both our insight and approach to this seemingly ubiquitous disease. This 2-part continuing medical education series provides a comprehensive and contemporary review of basal cell carcinoma. The second article in this series will present both the current standard of care and newly developed approaches to diagnosis, treatment, and prevention of this disease.

Association of Quality of Life With Surgical Excision of Early-Stage Melanoma of the Head and Neck.

Importance: Surgical excision is the standard-of-care treatment for Tis and T1a melanomas of the head and neck. Currently, however, the association of diagnosis and surgical treatment of these typically slowly progressive and nonfatal melanomas with a patient's health-related quality of life (HRQoL) is unknown. Objective: To characterize and assess HRQoL in patients with Tis and T1a head and neck melanoma, evaluate changes in HRQoL over the surgical treatment course, and identify patient characteristics associated with lower HRQoL. Design, Setting, and Participants: This longitudinal, prospective cohort study involved patients with Tis or T1a melanoma of the head and neck who underwent staged excision at a single tertiary care center (Memorial Sloan Kettering Cancer Center, New York, New York) and were recruited from June 1, 2016, to February 28, 2017. Patients were followed up for 1 year after their surgical procedure. Participants were asked to complete 2 patient-reported outcome measure questionnaires, Skindex-16 and Skin Cancer Index (SCI), at 4 time points: baseline, perioperative (1 to 2 weeks after surgery), and 6-month and 1-year follow-up. Main Outcomes and Measures: Scores on the Skindex-16 and SCI questionnaires. Results: In total, 56 patients were included in the study, among whom 24 (43%) were female and 32 (57%) were male, with a mean (range) age of 67.2 (32-88) years; all patients self-identified as white. Forty-one (73%) questionnaires at perioperative, 49 (88%) at 6-month postoperative, and 41 (73%) at 1-year postoperative time points were completed. At baseline, female patients and those younger than 65 years had statistically significantly worse HRQoL on the Skindex-16 questionnaire (mean score, 14.2 [95% CI, 9.1-21.9] and 16.1 [95% CI, 9.8-26.4]) and on the SCI questionnaire (mean score, 57.2 [95% CI, 48.3-67.6] and 53.2 [95% CI, 44.1-64.3]) compared with males (mean Skindex-16 score, 7.0 [95% CI, 4.8-10.3]; mean SCI score, 73.5 [95% CI, 66.0-81.7]) and those aged 65 years or older (mean Skindex-16 score 7.1 [95% CI, 5.0-10.0]; mean SCI score, 74.3 [95% CI, 67.7-81.6]). Questions that demonstrated the worst scores at baseline were worry about skin condition (Skindex-16) and worry about future skin cancers (SCI). The emotions subscale scores on the Skindex-16 questionnaire showed the greatest improvement from baseline to 1-year follow-up levels (26.6 vs 15.3; P < .001) and so did the appearance subscale scores on the SCI questionnaire (64.0 vs 84.6; P < .001). The score difference in HRQoL by sex diminished over time, whereas the score difference by age persisted through the first year. Conclusions and Relevance: Improvement in HRQoL at the 6-month and 1-year follow-up was associated with surgical excision in patients with early-stage head and neck melanoma, and younger and female patients experienced worse HRQoL. These results may be used in tailoring counseling for this patient population.

Tumor Board Conferences for Multidisciplinary Skin Cancer Management: A Survey of US Cancer Centers.

Background: Tumor board conferences (TBCs) are used by oncologic specialists to review patient cases, exchange knowledge, and discuss options for cancer management. These multidisciplinary meetings are often a cornerstone of treatment at leading cancer centers and are required for accreditation by certain groups, such as the American College of Surgeons' Commission on Cancer. Little is known regarding skin cancer TBCs. The objective of this study was to characterize the structure, function, and impact of existing skin cancer TBCs in the United States. Methods: A cross-sectional online survey was administered to physician leaders of skin cancer TBCs at NCI-designated Comprehensive and Clinical Cancer Centers. Results: Of the 59 centers successfully contacted, 14 (24%) reported not having a conference where skin cancer cases were discussed, and 45 (76%) identified 53 physician leaders. A total of 38 physicians (72%) completed the survey. Half of the meeting leaders were medical and/or surgical oncologists, and dermatologists led one-third of meetings. TBCs had a moderate to significant impact on patient care according to 97% of respondents. All respondents indicated that the meetings enhanced communication among physicians and provided an opportunity for involved specialists and professionals to discuss cases. The most frequently cited barrier to organizing TBCs was determining a common available date and time for attendees (62%). The most common suggestion for improvement was to increase attendance, specialists, and/or motivation. Conclusions: Results showed overall consistency in meeting structure but variability in function, which may be a reflection of institutional resources and investment in the conference. Future directions include defining metrics to evaluate changes in diagnosis or management plan after tumor board discussion, attendance, clinical trial enrollment, and cost analysis. Results of this survey may aid other institutions striving to develop and refine skin cancer TBCs.

The Largest Mass Poisoning in History: Arsenic Contamination of Well Water in Bangladesh.

Arsenic is a naturally occurring compound that is widely distributed in trace quantities in the environment. Levels toxic to humans have been found contaminating certain regions of the world and their groundwater, leading to deleterious effects. In fact, an estimated 150 million people are affected by arsenic contamination worldwide.1 Arsenic poisoning leads to several adverse health effects, including cancer of the lung, bladder, and kidney, neurologic disorders, cardiovascular disease, peripheral vascular disease, hypertension, pulmonary disease, and diabetes mellitus.2 Skin lesions are a common manifestation of arsenic poisoning. Early findings include diffuse or spotted melanosis, leukomelanosis, and depigmentation. Years of chronic arsenic poisoning can lead to acral keratoses and squamous cell carcinoma of the skin.3.

Cutaneous Langerhans cell histiocytosis presenting with hypopigmented lesions: Report of two cases and review of literature.

Langerhans cell histiocytosis is a rare group of disorders that results from the abnormal proliferation and accumulation of dendritic-derived cells in various organs of the body, such as the skin and bones. Hypopigmented macules are a rare cutaneous presentation of Langerhans cell histiocytosis that may pose a diagnostic dilemma when no other findings of Langerhans cell histiocytosis are present at the time of examination. We present 2 cases of the hypopigmented variant of Langerhans cell histiocytosis, including a case with histopathologic features of regression, and a review of the literature. These cases highlight the importance of including Langerhans cell histiocytosis in the differential diagnosis of an infant with hypopigmented macules and papules.

Isolation of proanthocyanidins from red wine, and their inhibitory effects on melanin synthesis in vitro.

The red wines made from Vitis vinifera were identified as skin-whitening effectors by using in vitro assays. OPCs in the wine were evaluated for tyrosinase activity and melanogenesis. Strong tyrosinase inhibitory activity was observed in fractions with high oligomeric proanthocyanidin (OPC) content. Among OPC dimers, a strong inhibitory effect on tyrosinase was observed with OPCs which contain (+)-catechin as an upper unit. Melanogenesis inhibitory effect was observed with OPCs which have (-)-epicatechin as upper units. Also, OPC trimers, upper and middle units joined with 4 → 8 bonds, showed stronger effects compared to trimers with 4 → 6 linkages. Interestingly, (-)-epicatechin-(4ß → 8)-(-)-epicatechin 3-O-gallate, which is a unique component of grapes has potent inhibitory effects on both tyrosinase and melanogenesis. Our data provide structural information about such active compounds. These results suggest that red wines containing OPC, have high melanogenesis inhibitory effect and are supposed to have skin-whitening effect.