Mixed Topics

MINNEAPOLIS -- Ignore the big health claims about vitamin supplements, pork, and nitrate-free food products. Meet patients “where they are,” even if that means you focus first on helping a morbidly obese patient maintain her weight instead of losing pounds. And use nutrition to empower patients and reduce the risk of cancer recurrence.

Dianne Piepenburg, MS, RDN, CSO, a certified oncology nutritionist at the Malcolm Randall VA Medical Center in Gainesville, Florida, offered these tips and more in a presentation about nutrition for cancer survivors. She spoke at the annual meeting of the Association of VA Hematology/Oncology (AVAHO).

According to the National Institutes of Health, an estimated 17 million cancer survivors live in the US, accounting for 5% of the population. Nearly two-thirds are aged ≥ 65 years.¹

Piepenburg highlighted the existence of certified specialists in oncology nutrition (CSOs). To be certified, registered dietitian nutritionists must have worked in that job for at least 2 years, have at least 2,000 hours of practice experience within the past 5 years and pass a board exam every 5 years.

Oncology nutritionists seek to empower cancer survivors to regain equilibrium in their lives, she said. “When a patient is told what scan to have next, what blood work they have to have, what treatment they need to be on, they feel they’re losing control,” she said. “Nutrition gives the power back to them, and they feel like there’s something they can do that’s in their control.”

Piepenburg urged colleagues to “meet patients where they are.” She gave the example of a patient with breast cancer whose body mass index is in the 50s, making her morbidly obese. “Our discussion wasn’t, ‘Let’s start [losing weight] today.’ Instead, I said, ‘Can we at least prevent you from gaining any more weight?’ She thought she could at least do that, try to recuperate a bit, and then start looking at a healthy weight loss. We’ll start there and circle back in a few months and see where we’re at.”

Piepenburg urged colleagues to bring exercise into the discussion. “We need people to be physically active no matter what phase of their survivorship journey they are in,” she said.

What about people who say, “I’ve never exercised a day in my life”? Her response: “I tell folks that we need them to move more. Maybe they’re walking to the mailbox or 3 laps around the house that day.”

Oncology patients should also watch sugar, meat, and processed foods. Refined sugar, fast food and processed food should be limited, Piepenburg said, along with red meats, such as beef, pork and lamb.

“Pork is not the ‘other white meat.’ How many of you grew up seeing and hearing that in the 1970s and 1980s? It’s a red meat, and it’s metabolized like a red meat.”

Advise patients to limit bacon, sausage, and lunch meat, she said, “even if they say, ‘I bought the nitrate-free and it’s really healthy for me.’”

It’s okay to eat some red meat, she said, “but there’s a tipping point. Tell them they can have some red meat but have it as a treat and please focus more on plant-based proteins—nuts, beans, legumes. But it’s tough for a lot of our veterans who grew up on meat and potatoes, and the only vegetable they eat is corn.”

It’s tough to limit grilling in a place like Minnesota, Piepenburg said, where the prime grilling season is short, and locals go a bit nuts when it’s nice enough outside. “I tell them to at least marinate the meat and put it on indirect heat.”

Finally, she encouraged oncology care providers to not fall for vitamin hype. Don’t rely on supplements for cancer prevention, she said. With some exceptions, she said, research has suggested they don’t work, and a 1990s study of beta-carotene and retinyl palmitate (vitamin A) in lung cancer was halted because patients actually fared worse on the regimen, although the effects didn’t seem to persist.²

MINNEAPOLIS -- Ignore the big health claims about vitamin supplements, pork, and nitrate-free food products. Meet patients “where they are,” even if that means you focus first on helping a morbidly obese patient maintain her weight instead of losing pounds. And use nutrition to empower patients and reduce the risk of cancer recurrence.

Dianne Piepenburg, MS, RDN, CSO, a certified oncology nutritionist at the Malcolm Randall VA Medical Center in Gainesville, Florida, offered these tips and more in a presentation about nutrition for cancer survivors. She spoke at the annual meeting of the Association of VA Hematology/Oncology (AVAHO).

According to the National Institutes of Health, an estimated 17 million cancer survivors live in the US, accounting for 5% of the population. Nearly two-thirds are aged ≥ 65 years.¹

Piepenburg highlighted the existence of certified specialists in oncology nutrition (CSOs). To be certified, registered dietitian nutritionists must have worked in that job for at least 2 years, have at least 2,000 hours of practice experience within the past 5 years and pass a board exam every 5 years.

Oncology nutritionists seek to empower cancer survivors to regain equilibrium in their lives, she said. “When a patient is told what scan to have next, what blood work they have to have, what treatment they need to be on, they feel they’re losing control,” she said. “Nutrition gives the power back to them, and they feel like there’s something they can do that’s in their control.”

Piepenburg urged colleagues to “meet patients where they are.” She gave the example of a patient with breast cancer whose body mass index is in the 50s, making her morbidly obese. “Our discussion wasn’t, ‘Let’s start [losing weight] today.’ Instead, I said, ‘Can we at least prevent you from gaining any more weight?’ She thought she could at least do that, try to recuperate a bit, and then start looking at a healthy weight loss. We’ll start there and circle back in a few months and see where we’re at.”

Piepenburg urged colleagues to bring exercise into the discussion. “We need people to be physically active no matter what phase of their survivorship journey they are in,” she said.

What about people who say, “I’ve never exercised a day in my life”? Her response: “I tell folks that we need them to move more. Maybe they’re walking to the mailbox or 3 laps around the house that day.”

Oncology patients should also watch sugar, meat, and processed foods. Refined sugar, fast food and processed food should be limited, Piepenburg said, along with red meats, such as beef, pork and lamb.

“Pork is not the ‘other white meat.’ How many of you grew up seeing and hearing that in the 1970s and 1980s? It’s a red meat, and it’s metabolized like a red meat.”

Advise patients to limit bacon, sausage, and lunch meat, she said, “even if they say, ‘I bought the nitrate-free and it’s really healthy for me.’”

It’s okay to eat some red meat, she said, “but there’s a tipping point. Tell them they can have some red meat but have it as a treat and please focus more on plant-based proteins—nuts, beans, legumes. But it’s tough for a lot of our veterans who grew up on meat and potatoes, and the only vegetable they eat is corn.”

It’s tough to limit grilling in a place like Minnesota, Piepenburg said, where the prime grilling season is short, and locals go a bit nuts when it’s nice enough outside. “I tell them to at least marinate the meat and put it on indirect heat.”

Finally, she encouraged oncology care providers to not fall for vitamin hype. Don’t rely on supplements for cancer prevention, she said. With some exceptions, she said, research has suggested they don’t work, and a 1990s study of beta-carotene and retinyl palmitate (vitamin A) in lung cancer was halted because patients actually fared worse on the regimen, although the effects didn’t seem to persist.²

MINNEAPOLIS -- Ignore the big health claims about vitamin supplements, pork, and nitrate-free food products. Meet patients “where they are,” even if that means you focus first on helping a morbidly obese patient maintain her weight instead of losing pounds. And use nutrition to empower patients and reduce the risk of cancer recurrence.

Dianne Piepenburg, MS, RDN, CSO, a certified oncology nutritionist at the Malcolm Randall VA Medical Center in Gainesville, Florida, offered these tips and more in a presentation about nutrition for cancer survivors. She spoke at the annual meeting of the Association of VA Hematology/Oncology (AVAHO).

According to the National Institutes of Health, an estimated 17 million cancer survivors live in the US, accounting for 5% of the population. Nearly two-thirds are aged ≥ 65 years.¹

Piepenburg highlighted the existence of certified specialists in oncology nutrition (CSOs). To be certified, registered dietitian nutritionists must have worked in that job for at least 2 years, have at least 2,000 hours of practice experience within the past 5 years and pass a board exam every 5 years.

Oncology nutritionists seek to empower cancer survivors to regain equilibrium in their lives, she said. “When a patient is told what scan to have next, what blood work they have to have, what treatment they need to be on, they feel they’re losing control,” she said. “Nutrition gives the power back to them, and they feel like there’s something they can do that’s in their control.”

Piepenburg urged colleagues to “meet patients where they are.” She gave the example of a patient with breast cancer whose body mass index is in the 50s, making her morbidly obese. “Our discussion wasn’t, ‘Let’s start [losing weight] today.’ Instead, I said, ‘Can we at least prevent you from gaining any more weight?’ She thought she could at least do that, try to recuperate a bit, and then start looking at a healthy weight loss. We’ll start there and circle back in a few months and see where we’re at.”

Piepenburg urged colleagues to bring exercise into the discussion. “We need people to be physically active no matter what phase of their survivorship journey they are in,” she said.

What about people who say, “I’ve never exercised a day in my life”? Her response: “I tell folks that we need them to move more. Maybe they’re walking to the mailbox or 3 laps around the house that day.”

Oncology patients should also watch sugar, meat, and processed foods. Refined sugar, fast food and processed food should be limited, Piepenburg said, along with red meats, such as beef, pork and lamb.

“Pork is not the ‘other white meat.’ How many of you grew up seeing and hearing that in the 1970s and 1980s? It’s a red meat, and it’s metabolized like a red meat.”

Advise patients to limit bacon, sausage, and lunch meat, she said, “even if they say, ‘I bought the nitrate-free and it’s really healthy for me.’”

It’s okay to eat some red meat, she said, “but there’s a tipping point. Tell them they can have some red meat but have it as a treat and please focus more on plant-based proteins—nuts, beans, legumes. But it’s tough for a lot of our veterans who grew up on meat and potatoes, and the only vegetable they eat is corn.”

It’s tough to limit grilling in a place like Minnesota, Piepenburg said, where the prime grilling season is short, and locals go a bit nuts when it’s nice enough outside. “I tell them to at least marinate the meat and put it on indirect heat.”

Finally, she encouraged oncology care providers to not fall for vitamin hype. Don’t rely on supplements for cancer prevention, she said. With some exceptions, she said, research has suggested they don’t work, and a 1990s study of beta-carotene and retinyl palmitate (vitamin A) in lung cancer was halted because patients actually fared worse on the regimen, although the effects didn’t seem to persist.²

CHICAGO – A care model that uses hospitalists to comanage vascular surgery patients cut myocardial infarction rates by more than half and reduced hospital stays by about 12%, according to results of a study of the hospitalist comanagement model from Loyola University Chicago, Maywood, Ill., presented at the annual meeting of the Midwestern Vascular Surgery Society.

“Hospitalist comanagement was associated with decreased length of stay without affecting readmission for patients undergoing amputation, embolectomy, and infected graft,” said Kaavya Adam, a third-year medical student at Loyola University Chicago. “In the overall population, there was a reduction in cases of MI, 30-day readmissions, and overall length of stay.”

In 2014, Loyola implemented a program that used 11 hospitalists to rotate through the vascular surgery service. The hospitalists call on any patient who stays more than 24 hours on the non-ICU floors. Adam said hospitalist duties include evaluating patient comorbidities, adjusting medication, talking with family about medical management, seeing patients on the day of surgery, ordering preoperative labs, and meeting with the anesthesiology and vascular surgery teams.

The study compared outcomes in 866 patients admitted during 2007-2013, before the comanagement model was put into place, and 572 admitted during 2014-2017.

Rates of diabetes, hypertension, chronic kidney disease, coronary artery disease, hyperlipidemia, and malnutrition were similar between the groups. However, the pre-comanagement group had significantly higher rates of ischemic pain (27.8% vs. 10.7%), gangrene (21.3% vs. 13.6%) and ulceration (30.6% vs. 21.9%), while the comanaged group had significantly higher rates of claudication (34.3% vs. 13.2%). The statistical analysis accounted for these variations, Adam said.

“We did find significant results for the reduction in the odds of MI at 30 days; there was a 61% reduction,” he said.

The reduction in hospital stay was even more pronounced for patients with complex cases, Adam said. In amputation, the length of stay was reduced by 3.77 days (P = .01); in embolectomy, by 7.35 (P = .004); and in infected graft, by 8.35 (P = .007).

Continuing research will evaluate the cost effectiveness of the hospitalist model and define a comanagement model that is most beneficial, Mr. Adam said. He had no relevant financial disclosures.

SOURCE: Adam K et al. Midwestern Vascular 2019, Abstract 14.

CHICAGO – A care model that uses hospitalists to comanage vascular surgery patients cut myocardial infarction rates by more than half and reduced hospital stays by about 12%, according to results of a study of the hospitalist comanagement model from Loyola University Chicago, Maywood, Ill., presented at the annual meeting of the Midwestern Vascular Surgery Society.

“Hospitalist comanagement was associated with decreased length of stay without affecting readmission for patients undergoing amputation, embolectomy, and infected graft,” said Kaavya Adam, a third-year medical student at Loyola University Chicago. “In the overall population, there was a reduction in cases of MI, 30-day readmissions, and overall length of stay.”

In 2014, Loyola implemented a program that used 11 hospitalists to rotate through the vascular surgery service. The hospitalists call on any patient who stays more than 24 hours on the non-ICU floors. Adam said hospitalist duties include evaluating patient comorbidities, adjusting medication, talking with family about medical management, seeing patients on the day of surgery, ordering preoperative labs, and meeting with the anesthesiology and vascular surgery teams.

The study compared outcomes in 866 patients admitted during 2007-2013, before the comanagement model was put into place, and 572 admitted during 2014-2017.

Rates of diabetes, hypertension, chronic kidney disease, coronary artery disease, hyperlipidemia, and malnutrition were similar between the groups. However, the pre-comanagement group had significantly higher rates of ischemic pain (27.8% vs. 10.7%), gangrene (21.3% vs. 13.6%) and ulceration (30.6% vs. 21.9%), while the comanaged group had significantly higher rates of claudication (34.3% vs. 13.2%). The statistical analysis accounted for these variations, Adam said.

“We did find significant results for the reduction in the odds of MI at 30 days; there was a 61% reduction,” he said.

The reduction in hospital stay was even more pronounced for patients with complex cases, Adam said. In amputation, the length of stay was reduced by 3.77 days (P = .01); in embolectomy, by 7.35 (P = .004); and in infected graft, by 8.35 (P = .007).

Continuing research will evaluate the cost effectiveness of the hospitalist model and define a comanagement model that is most beneficial, Mr. Adam said. He had no relevant financial disclosures.

SOURCE: Adam K et al. Midwestern Vascular 2019, Abstract 14.

CHICAGO – A care model that uses hospitalists to comanage vascular surgery patients cut myocardial infarction rates by more than half and reduced hospital stays by about 12%, according to results of a study of the hospitalist comanagement model from Loyola University Chicago, Maywood, Ill., presented at the annual meeting of the Midwestern Vascular Surgery Society.

“Hospitalist comanagement was associated with decreased length of stay without affecting readmission for patients undergoing amputation, embolectomy, and infected graft,” said Kaavya Adam, a third-year medical student at Loyola University Chicago. “In the overall population, there was a reduction in cases of MI, 30-day readmissions, and overall length of stay.”

In 2014, Loyola implemented a program that used 11 hospitalists to rotate through the vascular surgery service. The hospitalists call on any patient who stays more than 24 hours on the non-ICU floors. Adam said hospitalist duties include evaluating patient comorbidities, adjusting medication, talking with family about medical management, seeing patients on the day of surgery, ordering preoperative labs, and meeting with the anesthesiology and vascular surgery teams.

The study compared outcomes in 866 patients admitted during 2007-2013, before the comanagement model was put into place, and 572 admitted during 2014-2017.

Rates of diabetes, hypertension, chronic kidney disease, coronary artery disease, hyperlipidemia, and malnutrition were similar between the groups. However, the pre-comanagement group had significantly higher rates of ischemic pain (27.8% vs. 10.7%), gangrene (21.3% vs. 13.6%) and ulceration (30.6% vs. 21.9%), while the comanaged group had significantly higher rates of claudication (34.3% vs. 13.2%). The statistical analysis accounted for these variations, Adam said.

“We did find significant results for the reduction in the odds of MI at 30 days; there was a 61% reduction,” he said.

The reduction in hospital stay was even more pronounced for patients with complex cases, Adam said. In amputation, the length of stay was reduced by 3.77 days (P = .01); in embolectomy, by 7.35 (P = .004); and in infected graft, by 8.35 (P = .007).

Continuing research will evaluate the cost effectiveness of the hospitalist model and define a comanagement model that is most beneficial, Mr. Adam said. He had no relevant financial disclosures.

SOURCE: Adam K et al. Midwestern Vascular 2019, Abstract 14.

Stanley Patrick Weber, a former Indian Health Service (IHS) pediatrician, was convicted September 27 on 8 counts of sexual abuse of 4 Native American boys under his care at the Pine Ridge Indian Reservation, Oglala Lakota County, South Dakota.

Weber was an employee of the IHS from 1986 until he resigned in 2016. During that time, although suspicions were rampant that he was abusing young patients, he was moved from one reservation to another. An investigation by The Wall Street Journal and PBS/Frontline found officials in the IHS chain of command “ignored warning signs” and tried to silence whistleblowers.

Weber also was convicted last year and served time for abusing 2 boys at another government hospital in Montana before he arrived at Pine Ridge.

The Pine Ridge Reservation is home to some of the poorest communities in the US, with a dropout rate of > 70%, and a teen suicide rate 150% higher than America’s as a whole. Many of the youths testifying in the case described difficult childhoods, according to the Frontline program. In addition to assaulting them during health care visits, Weber lured them to his home with offers of alcohol and cash.

A White House task force was created to address the agency’s failures. In a statement, Rear Adm Michael Weahkee, IHS principal deputy director, said the IHS is “doing all we can throughout our agency to strengthen our protection for patients.” Steps taken include reinforcing with staff the prohibitions against inappropriate contact with children, reminding staff of the protections for whistleblowers, and hiring Integritas Creative Solutions to conduct a medical quality assurance review to examine whether laws, policies and procedures have been followed with regard to protecting patients from sexual abuse. The review complements work done by the Presidential Task Force on Protecting Native American Children in the Indian Health Service System and a separate review by the HHS Office of the Inspector General.

The IHS also is providing professional counseling for victims. Services do not have to be at an IHS facility or with an IHS provider. A confidential hot line has been established at 301-443-0658.

Weahkee calls the verdict “a long-awaited victory.” But, he adds, “we know that it will not completely heal the wounds inflicted on Dr. Weber’s victims.”

Stanley Patrick Weber, a former Indian Health Service (IHS) pediatrician, was convicted September 27 on 8 counts of sexual abuse of 4 Native American boys under his care at the Pine Ridge Indian Reservation, Oglala Lakota County, South Dakota.

Weber was an employee of the IHS from 1986 until he resigned in 2016. During that time, although suspicions were rampant that he was abusing young patients, he was moved from one reservation to another. An investigation by The Wall Street Journal and PBS/Frontline found officials in the IHS chain of command “ignored warning signs” and tried to silence whistleblowers.

Weber also was convicted last year and served time for abusing 2 boys at another government hospital in Montana before he arrived at Pine Ridge.

The Pine Ridge Reservation is home to some of the poorest communities in the US, with a dropout rate of > 70%, and a teen suicide rate 150% higher than America’s as a whole. Many of the youths testifying in the case described difficult childhoods, according to the Frontline program. In addition to assaulting them during health care visits, Weber lured them to his home with offers of alcohol and cash.

A White House task force was created to address the agency’s failures. In a statement, Rear Adm Michael Weahkee, IHS principal deputy director, said the IHS is “doing all we can throughout our agency to strengthen our protection for patients.” Steps taken include reinforcing with staff the prohibitions against inappropriate contact with children, reminding staff of the protections for whistleblowers, and hiring Integritas Creative Solutions to conduct a medical quality assurance review to examine whether laws, policies and procedures have been followed with regard to protecting patients from sexual abuse. The review complements work done by the Presidential Task Force on Protecting Native American Children in the Indian Health Service System and a separate review by the HHS Office of the Inspector General.

The IHS also is providing professional counseling for victims. Services do not have to be at an IHS facility or with an IHS provider. A confidential hot line has been established at 301-443-0658.

Weahkee calls the verdict “a long-awaited victory.” But, he adds, “we know that it will not completely heal the wounds inflicted on Dr. Weber’s victims.”

Stanley Patrick Weber, a former Indian Health Service (IHS) pediatrician, was convicted September 27 on 8 counts of sexual abuse of 4 Native American boys under his care at the Pine Ridge Indian Reservation, Oglala Lakota County, South Dakota.

Weber was an employee of the IHS from 1986 until he resigned in 2016. During that time, although suspicions were rampant that he was abusing young patients, he was moved from one reservation to another. An investigation by The Wall Street Journal and PBS/Frontline found officials in the IHS chain of command “ignored warning signs” and tried to silence whistleblowers.

Weber also was convicted last year and served time for abusing 2 boys at another government hospital in Montana before he arrived at Pine Ridge.

The Pine Ridge Reservation is home to some of the poorest communities in the US, with a dropout rate of > 70%, and a teen suicide rate 150% higher than America’s as a whole. Many of the youths testifying in the case described difficult childhoods, according to the Frontline program. In addition to assaulting them during health care visits, Weber lured them to his home with offers of alcohol and cash.

A White House task force was created to address the agency’s failures. In a statement, Rear Adm Michael Weahkee, IHS principal deputy director, said the IHS is “doing all we can throughout our agency to strengthen our protection for patients.” Steps taken include reinforcing with staff the prohibitions against inappropriate contact with children, reminding staff of the protections for whistleblowers, and hiring Integritas Creative Solutions to conduct a medical quality assurance review to examine whether laws, policies and procedures have been followed with regard to protecting patients from sexual abuse. The review complements work done by the Presidential Task Force on Protecting Native American Children in the Indian Health Service System and a separate review by the HHS Office of the Inspector General.

The IHS also is providing professional counseling for victims. Services do not have to be at an IHS facility or with an IHS provider. A confidential hot line has been established at 301-443-0658.

Weahkee calls the verdict “a long-awaited victory.” But, he adds, “we know that it will not completely heal the wounds inflicted on Dr. Weber’s victims.”

“The American Gastroenterological Association (AGA) has worked diligently and effectively to expand the pool of underrepresented in medicine physicians who provide care for patients with digestive diseases,” remarks Byron Cryer, MD, FORWARD principal investigator and associate dean for the office of faculty diversity & development, University of Texas Southwestern Medical Center, Dallas.

This long history of promoting and encouraging diversity in the field is notable; however, the society recognizes that there is still more to be done to prepare and sponsor underrepresented in medicine physician-scientists to assume leadership positions in the field.

“As of 2017, only 3.2% of gastroenterology fellows were African American and 8.5% were Hispanic,” remarked Sandra Quezada, MD, AGA Chair, Diversity Committee. These statistics closely correspond to AGA’s membership demographics, where only 3.36% of AGA members are African American and 5.53% are Hispanic, among those reporting ethnicity.

Under the leadership of principal investigators Byron Cryer, MD, and Sheila Crowe, MD, and coinvestigators Juanita Merchant, MD, and Jesus Rivera-Nieves, MD, AGA developed the FORWARD (Fostering Opportunities Resulting in Workforce and Research Diversity) program.

This program was funded by the National Institute of Diabetes and Digestive and Kidney Diseases through an education project (R25) grant.

The overall objective of the grant is to enhance the diversity of the biomedical science research workforce in gastroenterology and to prepare individuals to assume leadership positions within the AGA and in academic medicine.

1. Provide skill development in leadership – including general leadership development, executive coaching, and opportunities for leadership experience within the society.

2. Implement a diversity management plan focused on active mentoring approaches, using both one-on-one and networking approaches, to provide opportunities for broad support and sponsorship.

3. Provide training for skill development in research careers including training in research development, management of research groups, scientific manuscript, and grant writing.

The program targets fellows and early-career faculty and was promoted broadly to society membership and to past participants in AGA diversity programs. Through a rigorous selection process, 10 physician-scientists were selected among a qualified pool of applicants. Participants were matched with experienced academic gastroenterologists who committed to serve as their professional mentors throughout the course of their participation in the FORWARD program.

Leadership development

The first FORWARD cohort kicked off in March 2019 and will conclude May 2020. They convened at AGA’s inaugural 2½ day Leadership Development Conference. During the conference, the 10 FORWARD scholars joined with 18 AGA Future Leader program participants and 40 Women’s Leadership Conference attendees to participate in leadership development training that addressed such topics as building resilience, presentation skills, negotiation, career success in an ever-changing scientific environment, emotional intelligence, and other key topics designed to bolster their skills as emerging leaders in gastroenterology. With seven past and current AGA presidents in attendance, the FORWARD participants had the unique opportunity to learn about the career paths and gain advice from key leaders in the field while also networking with fellow emerging leaders in both the Future Leaders program and Women’s Leadership Conference.

To further prepare the FORWARD scholars to assume future leadership positions within the society, they’ve begun “internships” that included shadowing AGA committee meetings during Digestive Disease Week (DDW^®) 2019 in San Diego and participating in networking events with AGA leaders at receptions and various events.
 

Mentoring and coaching

In addition to leadership development training at the conference, each FORWARD scholar identified a research topic that they would work on with their mentor and a grant-writing expert to develop into a full grant proposal by the conclusion of the program. The FORWARD mentors have committed to provide mentoring that addresses scientific research content as well as career guidance. The active mentoring provided to the FORWARD scholars is extensive and includes regular contact with their mentors as well as one-on-one executive coaching by a professional coach committed to learning about their needs and guiding them through an individual development plan.

To facilitate the coaching for their individual development plan, each FORWARD scholar completed a Self-Assessment for Physician Leaders and a 360° assessment, which informed the executive coaching that the scholars receive. For many of the scholars, this is the first time that they have benefited from individual executive coaching.
 

Academic research skills development

In addition to leadership development training and networking opportunities along with executive coaching and mentoring, the FORWARD scholars benefit from direct evaluation and consultation on grant writing. FORWARD scholars have access to a private learning management system where they participate in online grant-writing skills training designed by a grant-writing expert. Based on their training, they are developing various segments of an actual grant proposal and are receiving both virtual peer critiques as well as individualized critiques and guidance from the grant-writing consultant, their program mentor, and their home institution mentor. Future training modules will include lab management and manuscript writing.

Jesus Rivera-Nieves, MD, FORWARD coinvestigator, stated, “I have had the honor of monitoring the progress of our first class of scholars recently. I am incredibly proud of the caliber of these early-career professionals and have no doubt that they will emerge as leaders in our field, where more diversity is greatly needed.”

The scholars and their mentors will reconvene at DDW^® 2020 for an opportunity to give a presentation and participate in a commencement ceremony concluding their involvement in the program. Following the graduation of this first cohort, there will be a period of evaluation prior to recruitment of the second cohort of FORWARD scholars.
 

The 2018-2020 FORWARD Cohort includes:

Yelina Alvarez, MD, PhD University of Pennsylvania Health System, Philadelphia

Dominique Bailey, MD Columbia University Medical Center, New York

Veroushka Ballester, MD, MSc Columbia University Medical Center, New York

Oriana M. Damas, MD University of Miami

Patricia D. Jones, MD, MSCR University of Miami

Folasade (Fola) May, MD, PhD, MPhil University of California, Los Angeles; Veterans Affairs

Antonio Mendoza Ladd, MD, FACG, FASGE Texas Tech Univ. Health Sciences Center, El Paso

Akinbowale Oyalowo, MD University of Pennsylvania, Philadelphia

Nneka Ufere, MD Massachusetts General Hospital, Boston

Eric J. Vargas, MD Mayo Clinic, Rochester, Minn.

The 2018-2020 – FORWARD Program Mentors

Maria Abreu, MD, AGAF University of Miami

C. Rick Boland, MD, AGAF University of California, San Diego

John Carethers, MD, AGAF University of Michigan, Ann Arbor

Darwin Conwell, MD, MS Ohio State University Wexner Medical Center, Columbus

Gail Hecht, MD, MS, AGAF Loyola University Medical Center, Maywood, Ill.

John Inadomi, MD, AGAF University of Washington, Seattle

David Lieberman, MD, AGAF Oregon Health and Science University, Portland

Jorge Marrero, MD, MS, AGAF University of Texas Southwestern Medical Center, Dallas

Robert Sandler, MD, MPH, AGAF University of North Carolina at Chapel Hill

Gary Wu, MD University of Pennsylvania, Philadelphia

Dr. Cryer is associate dean for faculty diversity and development, UT Southwestern Medical Center, Dallas; Dr. Rivera-Nieves, is professor of medicine, University of California, San Diego; and Ms. NuQuay, is senior director, member relations and constituency programs, American Gastroenterological Association, Bethesda, Md.

“The American Gastroenterological Association (AGA) has worked diligently and effectively to expand the pool of underrepresented in medicine physicians who provide care for patients with digestive diseases,” remarks Byron Cryer, MD, FORWARD principal investigator and associate dean for the office of faculty diversity & development, University of Texas Southwestern Medical Center, Dallas.

This long history of promoting and encouraging diversity in the field is notable; however, the society recognizes that there is still more to be done to prepare and sponsor underrepresented in medicine physician-scientists to assume leadership positions in the field.

“As of 2017, only 3.2% of gastroenterology fellows were African American and 8.5% were Hispanic,” remarked Sandra Quezada, MD, AGA Chair, Diversity Committee. These statistics closely correspond to AGA’s membership demographics, where only 3.36% of AGA members are African American and 5.53% are Hispanic, among those reporting ethnicity.

Under the leadership of principal investigators Byron Cryer, MD, and Sheila Crowe, MD, and coinvestigators Juanita Merchant, MD, and Jesus Rivera-Nieves, MD, AGA developed the FORWARD (Fostering Opportunities Resulting in Workforce and Research Diversity) program.

This program was funded by the National Institute of Diabetes and Digestive and Kidney Diseases through an education project (R25) grant.

The overall objective of the grant is to enhance the diversity of the biomedical science research workforce in gastroenterology and to prepare individuals to assume leadership positions within the AGA and in academic medicine.

1. Provide skill development in leadership – including general leadership development, executive coaching, and opportunities for leadership experience within the society.

2. Implement a diversity management plan focused on active mentoring approaches, using both one-on-one and networking approaches, to provide opportunities for broad support and sponsorship.

3. Provide training for skill development in research careers including training in research development, management of research groups, scientific manuscript, and grant writing.

The program targets fellows and early-career faculty and was promoted broadly to society membership and to past participants in AGA diversity programs. Through a rigorous selection process, 10 physician-scientists were selected among a qualified pool of applicants. Participants were matched with experienced academic gastroenterologists who committed to serve as their professional mentors throughout the course of their participation in the FORWARD program.

Leadership development

The first FORWARD cohort kicked off in March 2019 and will conclude May 2020. They convened at AGA’s inaugural 2½ day Leadership Development Conference. During the conference, the 10 FORWARD scholars joined with 18 AGA Future Leader program participants and 40 Women’s Leadership Conference attendees to participate in leadership development training that addressed such topics as building resilience, presentation skills, negotiation, career success in an ever-changing scientific environment, emotional intelligence, and other key topics designed to bolster their skills as emerging leaders in gastroenterology. With seven past and current AGA presidents in attendance, the FORWARD participants had the unique opportunity to learn about the career paths and gain advice from key leaders in the field while also networking with fellow emerging leaders in both the Future Leaders program and Women’s Leadership Conference.

To further prepare the FORWARD scholars to assume future leadership positions within the society, they’ve begun “internships” that included shadowing AGA committee meetings during Digestive Disease Week (DDW^®) 2019 in San Diego and participating in networking events with AGA leaders at receptions and various events.
 

Mentoring and coaching

In addition to leadership development training at the conference, each FORWARD scholar identified a research topic that they would work on with their mentor and a grant-writing expert to develop into a full grant proposal by the conclusion of the program. The FORWARD mentors have committed to provide mentoring that addresses scientific research content as well as career guidance. The active mentoring provided to the FORWARD scholars is extensive and includes regular contact with their mentors as well as one-on-one executive coaching by a professional coach committed to learning about their needs and guiding them through an individual development plan.

To facilitate the coaching for their individual development plan, each FORWARD scholar completed a Self-Assessment for Physician Leaders and a 360° assessment, which informed the executive coaching that the scholars receive. For many of the scholars, this is the first time that they have benefited from individual executive coaching.
 

Academic research skills development

In addition to leadership development training and networking opportunities along with executive coaching and mentoring, the FORWARD scholars benefit from direct evaluation and consultation on grant writing. FORWARD scholars have access to a private learning management system where they participate in online grant-writing skills training designed by a grant-writing expert. Based on their training, they are developing various segments of an actual grant proposal and are receiving both virtual peer critiques as well as individualized critiques and guidance from the grant-writing consultant, their program mentor, and their home institution mentor. Future training modules will include lab management and manuscript writing.

Jesus Rivera-Nieves, MD, FORWARD coinvestigator, stated, “I have had the honor of monitoring the progress of our first class of scholars recently. I am incredibly proud of the caliber of these early-career professionals and have no doubt that they will emerge as leaders in our field, where more diversity is greatly needed.”

The scholars and their mentors will reconvene at DDW^® 2020 for an opportunity to give a presentation and participate in a commencement ceremony concluding their involvement in the program. Following the graduation of this first cohort, there will be a period of evaluation prior to recruitment of the second cohort of FORWARD scholars.
 

The 2018-2020 FORWARD Cohort includes:

Yelina Alvarez, MD, PhD University of Pennsylvania Health System, Philadelphia

Dominique Bailey, MD Columbia University Medical Center, New York

Veroushka Ballester, MD, MSc Columbia University Medical Center, New York

Oriana M. Damas, MD University of Miami

Patricia D. Jones, MD, MSCR University of Miami

Folasade (Fola) May, MD, PhD, MPhil University of California, Los Angeles; Veterans Affairs

Antonio Mendoza Ladd, MD, FACG, FASGE Texas Tech Univ. Health Sciences Center, El Paso

Akinbowale Oyalowo, MD University of Pennsylvania, Philadelphia

Nneka Ufere, MD Massachusetts General Hospital, Boston

Eric J. Vargas, MD Mayo Clinic, Rochester, Minn.

The 2018-2020 – FORWARD Program Mentors

Maria Abreu, MD, AGAF University of Miami

C. Rick Boland, MD, AGAF University of California, San Diego

John Carethers, MD, AGAF University of Michigan, Ann Arbor

Darwin Conwell, MD, MS Ohio State University Wexner Medical Center, Columbus

Gail Hecht, MD, MS, AGAF Loyola University Medical Center, Maywood, Ill.

John Inadomi, MD, AGAF University of Washington, Seattle

David Lieberman, MD, AGAF Oregon Health and Science University, Portland

Jorge Marrero, MD, MS, AGAF University of Texas Southwestern Medical Center, Dallas

Robert Sandler, MD, MPH, AGAF University of North Carolina at Chapel Hill

Gary Wu, MD University of Pennsylvania, Philadelphia

Dr. Cryer is associate dean for faculty diversity and development, UT Southwestern Medical Center, Dallas; Dr. Rivera-Nieves, is professor of medicine, University of California, San Diego; and Ms. NuQuay, is senior director, member relations and constituency programs, American Gastroenterological Association, Bethesda, Md.

“The American Gastroenterological Association (AGA) has worked diligently and effectively to expand the pool of underrepresented in medicine physicians who provide care for patients with digestive diseases,” remarks Byron Cryer, MD, FORWARD principal investigator and associate dean for the office of faculty diversity & development, University of Texas Southwestern Medical Center, Dallas.

This long history of promoting and encouraging diversity in the field is notable; however, the society recognizes that there is still more to be done to prepare and sponsor underrepresented in medicine physician-scientists to assume leadership positions in the field.

“As of 2017, only 3.2% of gastroenterology fellows were African American and 8.5% were Hispanic,” remarked Sandra Quezada, MD, AGA Chair, Diversity Committee. These statistics closely correspond to AGA’s membership demographics, where only 3.36% of AGA members are African American and 5.53% are Hispanic, among those reporting ethnicity.

Under the leadership of principal investigators Byron Cryer, MD, and Sheila Crowe, MD, and coinvestigators Juanita Merchant, MD, and Jesus Rivera-Nieves, MD, AGA developed the FORWARD (Fostering Opportunities Resulting in Workforce and Research Diversity) program.

This program was funded by the National Institute of Diabetes and Digestive and Kidney Diseases through an education project (R25) grant.

The overall objective of the grant is to enhance the diversity of the biomedical science research workforce in gastroenterology and to prepare individuals to assume leadership positions within the AGA and in academic medicine.

1. Provide skill development in leadership – including general leadership development, executive coaching, and opportunities for leadership experience within the society.

2. Implement a diversity management plan focused on active mentoring approaches, using both one-on-one and networking approaches, to provide opportunities for broad support and sponsorship.

3. Provide training for skill development in research careers including training in research development, management of research groups, scientific manuscript, and grant writing.

The program targets fellows and early-career faculty and was promoted broadly to society membership and to past participants in AGA diversity programs. Through a rigorous selection process, 10 physician-scientists were selected among a qualified pool of applicants. Participants were matched with experienced academic gastroenterologists who committed to serve as their professional mentors throughout the course of their participation in the FORWARD program.

Leadership development

The first FORWARD cohort kicked off in March 2019 and will conclude May 2020. They convened at AGA’s inaugural 2½ day Leadership Development Conference. During the conference, the 10 FORWARD scholars joined with 18 AGA Future Leader program participants and 40 Women’s Leadership Conference attendees to participate in leadership development training that addressed such topics as building resilience, presentation skills, negotiation, career success in an ever-changing scientific environment, emotional intelligence, and other key topics designed to bolster their skills as emerging leaders in gastroenterology. With seven past and current AGA presidents in attendance, the FORWARD participants had the unique opportunity to learn about the career paths and gain advice from key leaders in the field while also networking with fellow emerging leaders in both the Future Leaders program and Women’s Leadership Conference.

To further prepare the FORWARD scholars to assume future leadership positions within the society, they’ve begun “internships” that included shadowing AGA committee meetings during Digestive Disease Week (DDW^®) 2019 in San Diego and participating in networking events with AGA leaders at receptions and various events.
 

Mentoring and coaching

In addition to leadership development training at the conference, each FORWARD scholar identified a research topic that they would work on with their mentor and a grant-writing expert to develop into a full grant proposal by the conclusion of the program. The FORWARD mentors have committed to provide mentoring that addresses scientific research content as well as career guidance. The active mentoring provided to the FORWARD scholars is extensive and includes regular contact with their mentors as well as one-on-one executive coaching by a professional coach committed to learning about their needs and guiding them through an individual development plan.

To facilitate the coaching for their individual development plan, each FORWARD scholar completed a Self-Assessment for Physician Leaders and a 360° assessment, which informed the executive coaching that the scholars receive. For many of the scholars, this is the first time that they have benefited from individual executive coaching.
 

Academic research skills development

In addition to leadership development training and networking opportunities along with executive coaching and mentoring, the FORWARD scholars benefit from direct evaluation and consultation on grant writing. FORWARD scholars have access to a private learning management system where they participate in online grant-writing skills training designed by a grant-writing expert. Based on their training, they are developing various segments of an actual grant proposal and are receiving both virtual peer critiques as well as individualized critiques and guidance from the grant-writing consultant, their program mentor, and their home institution mentor. Future training modules will include lab management and manuscript writing.

Jesus Rivera-Nieves, MD, FORWARD coinvestigator, stated, “I have had the honor of monitoring the progress of our first class of scholars recently. I am incredibly proud of the caliber of these early-career professionals and have no doubt that they will emerge as leaders in our field, where more diversity is greatly needed.”

The scholars and their mentors will reconvene at DDW^® 2020 for an opportunity to give a presentation and participate in a commencement ceremony concluding their involvement in the program. Following the graduation of this first cohort, there will be a period of evaluation prior to recruitment of the second cohort of FORWARD scholars.
 

The 2018-2020 FORWARD Cohort includes:

Yelina Alvarez, MD, PhD University of Pennsylvania Health System, Philadelphia

Dominique Bailey, MD Columbia University Medical Center, New York

Veroushka Ballester, MD, MSc Columbia University Medical Center, New York

Oriana M. Damas, MD University of Miami

Patricia D. Jones, MD, MSCR University of Miami

Folasade (Fola) May, MD, PhD, MPhil University of California, Los Angeles; Veterans Affairs

Antonio Mendoza Ladd, MD, FACG, FASGE Texas Tech Univ. Health Sciences Center, El Paso

Akinbowale Oyalowo, MD University of Pennsylvania, Philadelphia

Nneka Ufere, MD Massachusetts General Hospital, Boston

Eric J. Vargas, MD Mayo Clinic, Rochester, Minn.

The 2018-2020 – FORWARD Program Mentors

Maria Abreu, MD, AGAF University of Miami

C. Rick Boland, MD, AGAF University of California, San Diego

John Carethers, MD, AGAF University of Michigan, Ann Arbor

Darwin Conwell, MD, MS Ohio State University Wexner Medical Center, Columbus

Gail Hecht, MD, MS, AGAF Loyola University Medical Center, Maywood, Ill.

John Inadomi, MD, AGAF University of Washington, Seattle

David Lieberman, MD, AGAF Oregon Health and Science University, Portland

Jorge Marrero, MD, MS, AGAF University of Texas Southwestern Medical Center, Dallas

Robert Sandler, MD, MPH, AGAF University of North Carolina at Chapel Hill

Gary Wu, MD University of Pennsylvania, Philadelphia

Dr. Cryer is associate dean for faculty diversity and development, UT Southwestern Medical Center, Dallas; Dr. Rivera-Nieves, is professor of medicine, University of California, San Diego; and Ms. NuQuay, is senior director, member relations and constituency programs, American Gastroenterological Association, Bethesda, Md.

AFM cases continue to rise

Cases of Acute Flaccid Myelitis (AFM) are on the rise, with 210 confirmed cases of AFM in 40 states in 2018, up from 35 confirmed cases in 2017. AFM is a rare but serious condition that usually affects children, causing polio-like symptoms – focal extremity weakness, hyporeflexia, and sometimes cranial nerve dysfunction. The Centers for Disease Control and Prevention encourage all health care providers to contact their local health departments with any suspected cases of AFM.

Citation: Centers for Disease Control and Prevention. AFM Investigation. 2019 Jan. https://www.cdc.gov/acute-flaccid-myelitis/afm-surveillance.html.

HHS recommends prescribing naloxone to patients at high risk for opioid overdose

The U.S. Department of Health & Human Services recommends clinicians strongly consider prescribing or coprescribing naloxone to patients at high risk of opioid overdose. This includes patients who are on relatively high doses of opioids, take other medications which enhance opioid complications, or have underlying health conditions. Clinicians are also advised to educate patients and those likely to respond to an overdose on when and how to use naloxone in its variety of forms.

Citation: U.S. Department of Health & Human Services. HHS recommends prescribing or co-prescribing naloxone to patients at high risk for an opioid overdose. 2018 Dec 18. https://www.hhs.gov/about/news/2018/12/19/hhs-recommends-prescribing-or-co-prescribing-naloxone-to-patients-at-high-risk-for-an-opioid-overdose.html.

Fentanyl tops the list of opioid overdose drugs

The total number of drug overdose deaths per year in the United States increased 54%, from 41,340 deaths in 2011 to 63,632 deaths in 2016. Among opioids, mention of fentanyl increased during 2011-2016; that drug took the lead in 2016 with 29% of all drug overdose deaths. Among the drug overdose deaths involving fentanyl, 69% also involved one or more other drugs.

Citation: Hedegaard H et al. Drugs most frequently involved in drug overdose deaths: United States, 2011–2016. Natl Vital Stat Rep. 2018 Dec;67(9):1-14.

AFM cases continue to rise

Cases of Acute Flaccid Myelitis (AFM) are on the rise, with 210 confirmed cases of AFM in 40 states in 2018, up from 35 confirmed cases in 2017. AFM is a rare but serious condition that usually affects children, causing polio-like symptoms – focal extremity weakness, hyporeflexia, and sometimes cranial nerve dysfunction. The Centers for Disease Control and Prevention encourage all health care providers to contact their local health departments with any suspected cases of AFM.

Citation: Centers for Disease Control and Prevention. AFM Investigation. 2019 Jan. https://www.cdc.gov/acute-flaccid-myelitis/afm-surveillance.html.

HHS recommends prescribing naloxone to patients at high risk for opioid overdose

The U.S. Department of Health & Human Services recommends clinicians strongly consider prescribing or coprescribing naloxone to patients at high risk of opioid overdose. This includes patients who are on relatively high doses of opioids, take other medications which enhance opioid complications, or have underlying health conditions. Clinicians are also advised to educate patients and those likely to respond to an overdose on when and how to use naloxone in its variety of forms.

Citation: U.S. Department of Health & Human Services. HHS recommends prescribing or co-prescribing naloxone to patients at high risk for an opioid overdose. 2018 Dec 18. https://www.hhs.gov/about/news/2018/12/19/hhs-recommends-prescribing-or-co-prescribing-naloxone-to-patients-at-high-risk-for-an-opioid-overdose.html.

Fentanyl tops the list of opioid overdose drugs

The total number of drug overdose deaths per year in the United States increased 54%, from 41,340 deaths in 2011 to 63,632 deaths in 2016. Among opioids, mention of fentanyl increased during 2011-2016; that drug took the lead in 2016 with 29% of all drug overdose deaths. Among the drug overdose deaths involving fentanyl, 69% also involved one or more other drugs.

Citation: Hedegaard H et al. Drugs most frequently involved in drug overdose deaths: United States, 2011–2016. Natl Vital Stat Rep. 2018 Dec;67(9):1-14.

AFM cases continue to rise

Cases of Acute Flaccid Myelitis (AFM) are on the rise, with 210 confirmed cases of AFM in 40 states in 2018, up from 35 confirmed cases in 2017. AFM is a rare but serious condition that usually affects children, causing polio-like symptoms – focal extremity weakness, hyporeflexia, and sometimes cranial nerve dysfunction. The Centers for Disease Control and Prevention encourage all health care providers to contact their local health departments with any suspected cases of AFM.

Citation: Centers for Disease Control and Prevention. AFM Investigation. 2019 Jan. https://www.cdc.gov/acute-flaccid-myelitis/afm-surveillance.html.

HHS recommends prescribing naloxone to patients at high risk for opioid overdose

The U.S. Department of Health & Human Services recommends clinicians strongly consider prescribing or coprescribing naloxone to patients at high risk of opioid overdose. This includes patients who are on relatively high doses of opioids, take other medications which enhance opioid complications, or have underlying health conditions. Clinicians are also advised to educate patients and those likely to respond to an overdose on when and how to use naloxone in its variety of forms.

Citation: U.S. Department of Health & Human Services. HHS recommends prescribing or co-prescribing naloxone to patients at high risk for an opioid overdose. 2018 Dec 18. https://www.hhs.gov/about/news/2018/12/19/hhs-recommends-prescribing-or-co-prescribing-naloxone-to-patients-at-high-risk-for-an-opioid-overdose.html.

Fentanyl tops the list of opioid overdose drugs

The total number of drug overdose deaths per year in the United States increased 54%, from 41,340 deaths in 2011 to 63,632 deaths in 2016. Among opioids, mention of fentanyl increased during 2011-2016; that drug took the lead in 2016 with 29% of all drug overdose deaths. Among the drug overdose deaths involving fentanyl, 69% also involved one or more other drugs.

Citation: Hedegaard H et al. Drugs most frequently involved in drug overdose deaths: United States, 2011–2016. Natl Vital Stat Rep. 2018 Dec;67(9):1-14.

The US is in the midst of a chronic disease crisis. According to the latest published data available, 60% of Americans have at least 1 chronic condition, and 42% have ≥ 2 chronic conditions.¹ Estimates by the Health Resources and Services Administration (HRSA) indicate a current shortfall of 13 800 primary care physicians and a projected escalation of that shortage to be between 14 800 and 49 300 physicians by the year 2030.²

The US Public Health Service (USPHS) has used pharmacists since 1930 to provide direct patient care to underserved and vulnerable populations. Clinical pharmacists currently serve in direct patient care roles within the Indian Health Service (IHS), Federal Bureau of Prisons (BOP), Immigration and Customs Enforcement (ICE), and the United States Coast Guard (USCG) in many states (Figure). These pharmacists play a vital role in improving access to care and delivering quality care by managing acute and chronic diseases in collaborative practice settings and pharmacist-managed clinics.

It has previously been reported that in the face of physician shortages and growing demand for primary health care providers, pharmacists are well-equipped and motivated to meet this demand.³ A review of the previous 2 years of outcomes reported by clinical pharmacists certified through the USPHS National Clinical Pharmacy Specialist (NCPS) Committee are presented to demonstrate the impact of pharmacists in advancing the health of the populations they serve and to showcase a model for ameliorating the ongoing physician shortage.

Background

The USPHS NCPS Committee serves to promote uniform competency among clinical pharmacists by establishing national standards for protocols, collaborative practice agreements (CPAs), credentialing and privileging of pharmacists, and by collecting, reviewing, and publishing health care outcomes. The committee, whose constituents include pharmacist and physician subject matter experts from across USPHS agencies, reviews applications and protocols and certifies pharmacists (civilian and uniformed) to recognize an advanced scope of practice in managing various diseases and optimizing medication therapy. NCPScertified pharmacists manage a wide spectrum of diseases, including coagulopathy, asthma, diabetes mellitus (DM), hepatitis C, HIV, hypertension, pain, seizure disorders, and tobacco use disorders.

Clinical pharmacists practicing chronic disease management establish a clinical service in collaboration with 1 or more physicians, physician assistants, or nurse practitioners. In this collaborative practice, the health care practitioner(s) refer patients to be managed by a pharmacist for specific medical needs, such as anticoagulation management, or for holistic medication- focused care (eg, cardiovascular risk reduction, DM management, HIV, hepatitis, or mental health). The pharmacist may order and interpret laboratory tests, check vital signs, perform a limited physical examination, and gather other pertinent information from the patient and the medical record in order to provide the best possible care to the patient.

Medications may be started, stopped, or adjusted, education is provided, and therapeutic lifestyle interventions may be recommended. The pharmacist-run clinic provides the patient more frequent interaction with a health care professional (pharmacist) and focused disease management. As a result, pharmacists increase access to care and allow the medical team to handle a larger panel of patients as the practitioner delegates specified diseases to the pharmacist- managed clinic(s). The number of NCPS-certified pharmacists grew 46% from 2012 (n = 230) to 2017 (n = 336), reflecting an evolution of pharmacists’ practice to better meet the need of patients across the nation.

Methods

The NCPS Committee requires NCPS pharmacists to report data annually from all patients referred for pharmacist management for specific diseases in which they have been certified. The data reflect the patient’s clinical outcome goal status at the time of referral as well as the same status at the end of the reporting period or on release from the pharmacist-run clinic. These data describe the impact prescribing pharmacists have on patients reaching clinical outcome goals acting as the team member specializing in the medication selection and dosing aspect of care.

These records were reviewed for the fiscal year (FY) periods of October 1, 2015 to September 30, 2016 (FY 2016) and October 1, 2016 to September 30, 2017 (FY 2017). A systematic review of submitted reports resulted in 181 reports that included all requested data points for the disease as published here for FYs 2016 and 2017. These include 66 reports from FY 2016 and 115 reports from FY 2017; they cover 76 BOP and IHS facilities located across 24 states. Table 1 shows the number of outcome reports collected from 104 075 patient visits in pharmacist-run clinics in FYs 2016 and 2017.

Results

The following tables represent the standardized outcomes collected by NCPS-certified pharmacists providing direct patient care. Patients on anticoagulants (eg, warfarin) require special monitoring and education for drug interactions and adverse effects. NCPS-certified pharmacists were able to achieve a mean patient time in therapeutic range (TTR) of 67.6% (regardless of indication) over the 2 years (calculated per each facility by Rosendaal method of linear interpolation then combined in a weighted average per visit). The TTR produced by NCPS-certified pharmacists are consistent with Chest Guidelines and Expert Panel Report suggesting that TTR should be between 65% and 70%.⁴ Table 2 shows data from 100 reports with 68 255 patient visits for anticoagulation management.

DM management can be complex and time-intensive. NCPS data indicate pharmacist intervention resulted in a mean decrease in hemoglobin A_1c (HbA1c) of 1.8% from a baseline of 10.2% (decrease calculated per each facility then combined by weighted average per visit). Table 3 shows data from 30 reports with 16 518 patient visits for DM care.

In addition to diet and exercise, medication management plays a vital role in managing hypertension. Patients managed by an NCPS-certified pharmacist experienced a mean decrease in blood pressure from 144/83 to 133/77, putting them in goal for both systolic and diastolic ranges (decrease calculated per each facility then combined by weighted average per visit). Table 4 shows data from 16 reports and 7997 patient visits for treatment of hypertension.

HIV viral suppression is vital in order to best manage patients with HIV and reduce the risk of transmission. Pharmacistled clinics have shown a 32.9% absolute improvement in patients at goal (viral load < 50 copies/mL), from a mean baseline of 46.0% to a mean final assessment of 71.6% of patients at goal (combined by weighted average visits). Table 5 shows data from 6 reports covering 1532 patient encounters for management of HIV.

Nicotine dependence includes the use of cigarettes, cigars, pipe tobacco, chewing tobacco, and vaping products containing nicotine. NCPS-certified pharmacists have successfully helped patients improve their chance of quitting, with a 6-month quit rate of 22.2% (quit rate calculated per each facility then combined by weighted average by visits), which is higher than the national average of 9.4% as reported by the Centers for Disease and Control and Prevention. ⁵ Table 6 shows 29 reports covering 9773 patient visits for treatment of nicotine dependence.

Discussion

These data demonstrate the ability of advanced practice pharmacists in multiple locations within the federal sector to improve targeted clinical outcomes in patients with varying diseases. These results are strengthened by their varied origins as well as the improvements observed across the board. Limitations include the general lack of a comparable dataset, manual method of selfreporting by the individual facilities, and the relatively limited array of diseases reported. Although NCPS-certified pharmacists are currently providing care for patients with hepatitis C, asthma, seizure, pain and other diseases not reported here, there are insufficient data collected for FYs 2016 and 2017 to merit inclusion within this report.

Pharmacists are trusted, readily available medication experts. In a clinical role, NCPS-certified pharmacists have increased access to primary care services and demonstrated beneficial impact on important health outcomes as exhibited by the data reported above. Clinical pharmacy is a growing field, and NCPS has displayed continual growth in both the number of NCPS-certified pharmacists and the number of patient encounters performed by these providers. As more pharmacists in all settings collaborate with medical providers to offer high-quality clinical care, these providers will have more opportunity to delegate disease management. Continued reporting of clinical pharmacy outcomes is expected to increase confidence in pharmacists as primary care providers, increase utilization of pharmacy clinical services, and assist in easing the burden of primary care provider shortages across our nation.

Although these outcomes indicate demonstrable benefit in patient-centered outcomes, the need for ongoing assessment and continued improvement is not obviated. Future efforts may benefit from a comparison of alternative approaches to better facilitate the establishment of best practices. Alignment of clinical outcomes with the Centers for Medicare and Medicaid Services (CMS) Electronic Clinical Quality Measures, where applicable, also may prove beneficial by automating the reporting process and thereby decreasing the burden of reporting as well as providing an avenue for standard comparison across multiple populations. Clinical pharmacy interventions have positive outcomes based on the NCPS model, and the NCPS Committee invites other clinical settings to report outcomes data with which to compare.

Conclusion

The NCPS Committee has documented positive outcomes of clinical pharmacy intervention and anticipates growth of the pharmacy profession as additional states and health systems recognize the capacity of the pharmacist to provide high-quality, multidisciplinary patient care. Clinical pharmacists are prepared to address critical health care needs as the US continues to face a PCP shortage.² The NCPS Committee challenges those participating in clinical pharmacy practice to report outcomes to amplify this body of evidence.

Acknowledgments
NCPS-certified pharmacists provided the outcomes detailed in this report. For document review and edits: Federal Bureau of Prison Publication Review Workgroup; RADM Ty Bingham, USPHS; CAPT Cindy Gunderson, USPHS; CAPT Kevin Brooks, USPHS.

The US is in the midst of a chronic disease crisis. According to the latest published data available, 60% of Americans have at least 1 chronic condition, and 42% have ≥ 2 chronic conditions.¹ Estimates by the Health Resources and Services Administration (HRSA) indicate a current shortfall of 13 800 primary care physicians and a projected escalation of that shortage to be between 14 800 and 49 300 physicians by the year 2030.²

The US Public Health Service (USPHS) has used pharmacists since 1930 to provide direct patient care to underserved and vulnerable populations. Clinical pharmacists currently serve in direct patient care roles within the Indian Health Service (IHS), Federal Bureau of Prisons (BOP), Immigration and Customs Enforcement (ICE), and the United States Coast Guard (USCG) in many states (Figure). These pharmacists play a vital role in improving access to care and delivering quality care by managing acute and chronic diseases in collaborative practice settings and pharmacist-managed clinics.

It has previously been reported that in the face of physician shortages and growing demand for primary health care providers, pharmacists are well-equipped and motivated to meet this demand.³ A review of the previous 2 years of outcomes reported by clinical pharmacists certified through the USPHS National Clinical Pharmacy Specialist (NCPS) Committee are presented to demonstrate the impact of pharmacists in advancing the health of the populations they serve and to showcase a model for ameliorating the ongoing physician shortage.

Background

The USPHS NCPS Committee serves to promote uniform competency among clinical pharmacists by establishing national standards for protocols, collaborative practice agreements (CPAs), credentialing and privileging of pharmacists, and by collecting, reviewing, and publishing health care outcomes. The committee, whose constituents include pharmacist and physician subject matter experts from across USPHS agencies, reviews applications and protocols and certifies pharmacists (civilian and uniformed) to recognize an advanced scope of practice in managing various diseases and optimizing medication therapy. NCPScertified pharmacists manage a wide spectrum of diseases, including coagulopathy, asthma, diabetes mellitus (DM), hepatitis C, HIV, hypertension, pain, seizure disorders, and tobacco use disorders.

Clinical pharmacists practicing chronic disease management establish a clinical service in collaboration with 1 or more physicians, physician assistants, or nurse practitioners. In this collaborative practice, the health care practitioner(s) refer patients to be managed by a pharmacist for specific medical needs, such as anticoagulation management, or for holistic medication- focused care (eg, cardiovascular risk reduction, DM management, HIV, hepatitis, or mental health). The pharmacist may order and interpret laboratory tests, check vital signs, perform a limited physical examination, and gather other pertinent information from the patient and the medical record in order to provide the best possible care to the patient.

Medications may be started, stopped, or adjusted, education is provided, and therapeutic lifestyle interventions may be recommended. The pharmacist-run clinic provides the patient more frequent interaction with a health care professional (pharmacist) and focused disease management. As a result, pharmacists increase access to care and allow the medical team to handle a larger panel of patients as the practitioner delegates specified diseases to the pharmacist- managed clinic(s). The number of NCPS-certified pharmacists grew 46% from 2012 (n = 230) to 2017 (n = 336), reflecting an evolution of pharmacists’ practice to better meet the need of patients across the nation.

Methods

The NCPS Committee requires NCPS pharmacists to report data annually from all patients referred for pharmacist management for specific diseases in which they have been certified. The data reflect the patient’s clinical outcome goal status at the time of referral as well as the same status at the end of the reporting period or on release from the pharmacist-run clinic. These data describe the impact prescribing pharmacists have on patients reaching clinical outcome goals acting as the team member specializing in the medication selection and dosing aspect of care.

These records were reviewed for the fiscal year (FY) periods of October 1, 2015 to September 30, 2016 (FY 2016) and October 1, 2016 to September 30, 2017 (FY 2017). A systematic review of submitted reports resulted in 181 reports that included all requested data points for the disease as published here for FYs 2016 and 2017. These include 66 reports from FY 2016 and 115 reports from FY 2017; they cover 76 BOP and IHS facilities located across 24 states. Table 1 shows the number of outcome reports collected from 104 075 patient visits in pharmacist-run clinics in FYs 2016 and 2017.

Results

The following tables represent the standardized outcomes collected by NCPS-certified pharmacists providing direct patient care. Patients on anticoagulants (eg, warfarin) require special monitoring and education for drug interactions and adverse effects. NCPS-certified pharmacists were able to achieve a mean patient time in therapeutic range (TTR) of 67.6% (regardless of indication) over the 2 years (calculated per each facility by Rosendaal method of linear interpolation then combined in a weighted average per visit). The TTR produced by NCPS-certified pharmacists are consistent with Chest Guidelines and Expert Panel Report suggesting that TTR should be between 65% and 70%.⁴ Table 2 shows data from 100 reports with 68 255 patient visits for anticoagulation management.

DM management can be complex and time-intensive. NCPS data indicate pharmacist intervention resulted in a mean decrease in hemoglobin A_1c (HbA1c) of 1.8% from a baseline of 10.2% (decrease calculated per each facility then combined by weighted average per visit). Table 3 shows data from 30 reports with 16 518 patient visits for DM care.

In addition to diet and exercise, medication management plays a vital role in managing hypertension. Patients managed by an NCPS-certified pharmacist experienced a mean decrease in blood pressure from 144/83 to 133/77, putting them in goal for both systolic and diastolic ranges (decrease calculated per each facility then combined by weighted average per visit). Table 4 shows data from 16 reports and 7997 patient visits for treatment of hypertension.

HIV viral suppression is vital in order to best manage patients with HIV and reduce the risk of transmission. Pharmacistled clinics have shown a 32.9% absolute improvement in patients at goal (viral load < 50 copies/mL), from a mean baseline of 46.0% to a mean final assessment of 71.6% of patients at goal (combined by weighted average visits). Table 5 shows data from 6 reports covering 1532 patient encounters for management of HIV.

Nicotine dependence includes the use of cigarettes, cigars, pipe tobacco, chewing tobacco, and vaping products containing nicotine. NCPS-certified pharmacists have successfully helped patients improve their chance of quitting, with a 6-month quit rate of 22.2% (quit rate calculated per each facility then combined by weighted average by visits), which is higher than the national average of 9.4% as reported by the Centers for Disease and Control and Prevention. ⁵ Table 6 shows 29 reports covering 9773 patient visits for treatment of nicotine dependence.

Discussion

These data demonstrate the ability of advanced practice pharmacists in multiple locations within the federal sector to improve targeted clinical outcomes in patients with varying diseases. These results are strengthened by their varied origins as well as the improvements observed across the board. Limitations include the general lack of a comparable dataset, manual method of selfreporting by the individual facilities, and the relatively limited array of diseases reported. Although NCPS-certified pharmacists are currently providing care for patients with hepatitis C, asthma, seizure, pain and other diseases not reported here, there are insufficient data collected for FYs 2016 and 2017 to merit inclusion within this report.

Pharmacists are trusted, readily available medication experts. In a clinical role, NCPS-certified pharmacists have increased access to primary care services and demonstrated beneficial impact on important health outcomes as exhibited by the data reported above. Clinical pharmacy is a growing field, and NCPS has displayed continual growth in both the number of NCPS-certified pharmacists and the number of patient encounters performed by these providers. As more pharmacists in all settings collaborate with medical providers to offer high-quality clinical care, these providers will have more opportunity to delegate disease management. Continued reporting of clinical pharmacy outcomes is expected to increase confidence in pharmacists as primary care providers, increase utilization of pharmacy clinical services, and assist in easing the burden of primary care provider shortages across our nation.

Although these outcomes indicate demonstrable benefit in patient-centered outcomes, the need for ongoing assessment and continued improvement is not obviated. Future efforts may benefit from a comparison of alternative approaches to better facilitate the establishment of best practices. Alignment of clinical outcomes with the Centers for Medicare and Medicaid Services (CMS) Electronic Clinical Quality Measures, where applicable, also may prove beneficial by automating the reporting process and thereby decreasing the burden of reporting as well as providing an avenue for standard comparison across multiple populations. Clinical pharmacy interventions have positive outcomes based on the NCPS model, and the NCPS Committee invites other clinical settings to report outcomes data with which to compare.

Conclusion

The NCPS Committee has documented positive outcomes of clinical pharmacy intervention and anticipates growth of the pharmacy profession as additional states and health systems recognize the capacity of the pharmacist to provide high-quality, multidisciplinary patient care. Clinical pharmacists are prepared to address critical health care needs as the US continues to face a PCP shortage.² The NCPS Committee challenges those participating in clinical pharmacy practice to report outcomes to amplify this body of evidence.

Acknowledgments
NCPS-certified pharmacists provided the outcomes detailed in this report. For document review and edits: Federal Bureau of Prison Publication Review Workgroup; RADM Ty Bingham, USPHS; CAPT Cindy Gunderson, USPHS; CAPT Kevin Brooks, USPHS.

The US is in the midst of a chronic disease crisis. According to the latest published data available, 60% of Americans have at least 1 chronic condition, and 42% have ≥ 2 chronic conditions.¹ Estimates by the Health Resources and Services Administration (HRSA) indicate a current shortfall of 13 800 primary care physicians and a projected escalation of that shortage to be between 14 800 and 49 300 physicians by the year 2030.²

The US Public Health Service (USPHS) has used pharmacists since 1930 to provide direct patient care to underserved and vulnerable populations. Clinical pharmacists currently serve in direct patient care roles within the Indian Health Service (IHS), Federal Bureau of Prisons (BOP), Immigration and Customs Enforcement (ICE), and the United States Coast Guard (USCG) in many states (Figure). These pharmacists play a vital role in improving access to care and delivering quality care by managing acute and chronic diseases in collaborative practice settings and pharmacist-managed clinics.

It has previously been reported that in the face of physician shortages and growing demand for primary health care providers, pharmacists are well-equipped and motivated to meet this demand.³ A review of the previous 2 years of outcomes reported by clinical pharmacists certified through the USPHS National Clinical Pharmacy Specialist (NCPS) Committee are presented to demonstrate the impact of pharmacists in advancing the health of the populations they serve and to showcase a model for ameliorating the ongoing physician shortage.

Background

The USPHS NCPS Committee serves to promote uniform competency among clinical pharmacists by establishing national standards for protocols, collaborative practice agreements (CPAs), credentialing and privileging of pharmacists, and by collecting, reviewing, and publishing health care outcomes. The committee, whose constituents include pharmacist and physician subject matter experts from across USPHS agencies, reviews applications and protocols and certifies pharmacists (civilian and uniformed) to recognize an advanced scope of practice in managing various diseases and optimizing medication therapy. NCPScertified pharmacists manage a wide spectrum of diseases, including coagulopathy, asthma, diabetes mellitus (DM), hepatitis C, HIV, hypertension, pain, seizure disorders, and tobacco use disorders.

Clinical pharmacists practicing chronic disease management establish a clinical service in collaboration with 1 or more physicians, physician assistants, or nurse practitioners. In this collaborative practice, the health care practitioner(s) refer patients to be managed by a pharmacist for specific medical needs, such as anticoagulation management, or for holistic medication- focused care (eg, cardiovascular risk reduction, DM management, HIV, hepatitis, or mental health). The pharmacist may order and interpret laboratory tests, check vital signs, perform a limited physical examination, and gather other pertinent information from the patient and the medical record in order to provide the best possible care to the patient.

Medications may be started, stopped, or adjusted, education is provided, and therapeutic lifestyle interventions may be recommended. The pharmacist-run clinic provides the patient more frequent interaction with a health care professional (pharmacist) and focused disease management. As a result, pharmacists increase access to care and allow the medical team to handle a larger panel of patients as the practitioner delegates specified diseases to the pharmacist- managed clinic(s). The number of NCPS-certified pharmacists grew 46% from 2012 (n = 230) to 2017 (n = 336), reflecting an evolution of pharmacists’ practice to better meet the need of patients across the nation.

Methods

The NCPS Committee requires NCPS pharmacists to report data annually from all patients referred for pharmacist management for specific diseases in which they have been certified. The data reflect the patient’s clinical outcome goal status at the time of referral as well as the same status at the end of the reporting period or on release from the pharmacist-run clinic. These data describe the impact prescribing pharmacists have on patients reaching clinical outcome goals acting as the team member specializing in the medication selection and dosing aspect of care.

These records were reviewed for the fiscal year (FY) periods of October 1, 2015 to September 30, 2016 (FY 2016) and October 1, 2016 to September 30, 2017 (FY 2017). A systematic review of submitted reports resulted in 181 reports that included all requested data points for the disease as published here for FYs 2016 and 2017. These include 66 reports from FY 2016 and 115 reports from FY 2017; they cover 76 BOP and IHS facilities located across 24 states. Table 1 shows the number of outcome reports collected from 104 075 patient visits in pharmacist-run clinics in FYs 2016 and 2017.

Results

The following tables represent the standardized outcomes collected by NCPS-certified pharmacists providing direct patient care. Patients on anticoagulants (eg, warfarin) require special monitoring and education for drug interactions and adverse effects. NCPS-certified pharmacists were able to achieve a mean patient time in therapeutic range (TTR) of 67.6% (regardless of indication) over the 2 years (calculated per each facility by Rosendaal method of linear interpolation then combined in a weighted average per visit). The TTR produced by NCPS-certified pharmacists are consistent with Chest Guidelines and Expert Panel Report suggesting that TTR should be between 65% and 70%.⁴ Table 2 shows data from 100 reports with 68 255 patient visits for anticoagulation management.

DM management can be complex and time-intensive. NCPS data indicate pharmacist intervention resulted in a mean decrease in hemoglobin A_1c (HbA1c) of 1.8% from a baseline of 10.2% (decrease calculated per each facility then combined by weighted average per visit). Table 3 shows data from 30 reports with 16 518 patient visits for DM care.

In addition to diet and exercise, medication management plays a vital role in managing hypertension. Patients managed by an NCPS-certified pharmacist experienced a mean decrease in blood pressure from 144/83 to 133/77, putting them in goal for both systolic and diastolic ranges (decrease calculated per each facility then combined by weighted average per visit). Table 4 shows data from 16 reports and 7997 patient visits for treatment of hypertension.

HIV viral suppression is vital in order to best manage patients with HIV and reduce the risk of transmission. Pharmacistled clinics have shown a 32.9% absolute improvement in patients at goal (viral load < 50 copies/mL), from a mean baseline of 46.0% to a mean final assessment of 71.6% of patients at goal (combined by weighted average visits). Table 5 shows data from 6 reports covering 1532 patient encounters for management of HIV.

Nicotine dependence includes the use of cigarettes, cigars, pipe tobacco, chewing tobacco, and vaping products containing nicotine. NCPS-certified pharmacists have successfully helped patients improve their chance of quitting, with a 6-month quit rate of 22.2% (quit rate calculated per each facility then combined by weighted average by visits), which is higher than the national average of 9.4% as reported by the Centers for Disease and Control and Prevention. ⁵ Table 6 shows 29 reports covering 9773 patient visits for treatment of nicotine dependence.

Discussion

These data demonstrate the ability of advanced practice pharmacists in multiple locations within the federal sector to improve targeted clinical outcomes in patients with varying diseases. These results are strengthened by their varied origins as well as the improvements observed across the board. Limitations include the general lack of a comparable dataset, manual method of selfreporting by the individual facilities, and the relatively limited array of diseases reported. Although NCPS-certified pharmacists are currently providing care for patients with hepatitis C, asthma, seizure, pain and other diseases not reported here, there are insufficient data collected for FYs 2016 and 2017 to merit inclusion within this report.

Pharmacists are trusted, readily available medication experts. In a clinical role, NCPS-certified pharmacists have increased access to primary care services and demonstrated beneficial impact on important health outcomes as exhibited by the data reported above. Clinical pharmacy is a growing field, and NCPS has displayed continual growth in both the number of NCPS-certified pharmacists and the number of patient encounters performed by these providers. As more pharmacists in all settings collaborate with medical providers to offer high-quality clinical care, these providers will have more opportunity to delegate disease management. Continued reporting of clinical pharmacy outcomes is expected to increase confidence in pharmacists as primary care providers, increase utilization of pharmacy clinical services, and assist in easing the burden of primary care provider shortages across our nation.

Although these outcomes indicate demonstrable benefit in patient-centered outcomes, the need for ongoing assessment and continued improvement is not obviated. Future efforts may benefit from a comparison of alternative approaches to better facilitate the establishment of best practices. Alignment of clinical outcomes with the Centers for Medicare and Medicaid Services (CMS) Electronic Clinical Quality Measures, where applicable, also may prove beneficial by automating the reporting process and thereby decreasing the burden of reporting as well as providing an avenue for standard comparison across multiple populations. Clinical pharmacy interventions have positive outcomes based on the NCPS model, and the NCPS Committee invites other clinical settings to report outcomes data with which to compare.

Conclusion

The NCPS Committee has documented positive outcomes of clinical pharmacy intervention and anticipates growth of the pharmacy profession as additional states and health systems recognize the capacity of the pharmacist to provide high-quality, multidisciplinary patient care. Clinical pharmacists are prepared to address critical health care needs as the US continues to face a PCP shortage.² The NCPS Committee challenges those participating in clinical pharmacy practice to report outcomes to amplify this body of evidence.

Acknowledgments
NCPS-certified pharmacists provided the outcomes detailed in this report. For document review and edits: Federal Bureau of Prison Publication Review Workgroup; RADM Ty Bingham, USPHS; CAPT Cindy Gunderson, USPHS; CAPT Kevin Brooks, USPHS.

MENSTRUAL PAIN RELIEF THROUGH MICRO-PULSES

Livia, by iPulse Medical Ltd, is a US Food and Drug Administration (FDA) approved, drug-free option to treat menstrual pain through the transmission of electrical pulses. Electrodes are placed on the body at the source of menstrual pain and send a frequency to the nerves to reduce pain. Livia was designed based on the principles of the “gate control” theory of pain, says iPulse Medical. When the nerves are stimulated by the device’s electrodes, the nerve gate is closed, preventing pain signals from being received or felt in the brain.

The device can be worn in public or at home and allows the user to adjust the frequency of the electrical signal to correspond with her pain intensity. According to iPulse Medical, there are no adverse effects and the user will not build up a tolerance; however, the device should not be worn if the user has a pacemaker or is undergoing fertility treatment.

FOR MORE INFORMATION, VISIT: https://mylivia.com/

EXPAREL FOR CESAREAN DELIVERY

Pacira BioSciences an-nounced completion of their Phase 4 study of Exparel (bupivacaine lipsome injectable suspension), a local analgesic given to patients undergoing planned cesarean delivery (CD), aimed at reducing postsurgical pain and total opioid consumption through the first 72 hours postsurgery. Exparel is administered through transversus abdominis plane field block.

Pacira’s multicenter, randomized, double-blind study of 186 patients showed that those receiving Exparel plus bupivacaine HCl had a 52% reduction in total opioid consumption and significantly lower pain scores through the first 72 hours after CD, compared with those receiving only bupivacaine HCl. The most common adverse effects are itching and nausea. Exparel should not be used for patients under the age of 18 and should be used cautiously in patients with hepatic disease.

FOR MORE INFORMATION, VISIT: https://www.exparel.com/

M GENITALIUM ASSAY DETECTS THE STI

Hologic’s Aptima^® Mycoplasma genitalium assay is the first FDA-cleared diagnostic test for this sexually transmitted infection (STI), which has been identified by the Centers for Disease Control and Prevention as an emerging public health threat. The assay is an in vitro nucleic acid amplification test that can be used to verify swab or urine samples from women and men. In published studies, the ribosomal RNA-based assay displayed greater sensitivity than lab-developed or CE-marked DNA-based tests. Early detection is important, Hologic asserts, because M genitalium is increasing in prevalence among higher-risk populations; however, it is not well known and often misdiagnosed, leading to incorrect treatment as well as risk for transmission and recurrence.

Hologic cites several studies that have shown M genitalium can be asymptomatic; however, it also can be associated with nongonococcal urethritis in men and cervicitis in women, as well as increased risk for pelvic inflammatory disease, preterm birth, spontaneous abortion, and infertility. A high percentage of infected people have an antibiotic-resistant strain, demonstrating a need for early detection and screening.

FOR MORE INFORMATION, VISIT: https://www.hologic.com

NEW ART OPTION

FOR MORE INFORMATION, VISIT: https://www.ferringusa.com

MENSTRUAL PAIN RELIEF THROUGH MICRO-PULSES

Livia, by iPulse Medical Ltd, is a US Food and Drug Administration (FDA) approved, drug-free option to treat menstrual pain through the transmission of electrical pulses. Electrodes are placed on the body at the source of menstrual pain and send a frequency to the nerves to reduce pain. Livia was designed based on the principles of the “gate control” theory of pain, says iPulse Medical. When the nerves are stimulated by the device’s electrodes, the nerve gate is closed, preventing pain signals from being received or felt in the brain.

The device can be worn in public or at home and allows the user to adjust the frequency of the electrical signal to correspond with her pain intensity. According to iPulse Medical, there are no adverse effects and the user will not build up a tolerance; however, the device should not be worn if the user has a pacemaker or is undergoing fertility treatment.

FOR MORE INFORMATION, VISIT: https://mylivia.com/

EXPAREL FOR CESAREAN DELIVERY

Pacira BioSciences an-nounced completion of their Phase 4 study of Exparel (bupivacaine lipsome injectable suspension), a local analgesic given to patients undergoing planned cesarean delivery (CD), aimed at reducing postsurgical pain and total opioid consumption through the first 72 hours postsurgery. Exparel is administered through transversus abdominis plane field block.

Pacira’s multicenter, randomized, double-blind study of 186 patients showed that those receiving Exparel plus bupivacaine HCl had a 52% reduction in total opioid consumption and significantly lower pain scores through the first 72 hours after CD, compared with those receiving only bupivacaine HCl. The most common adverse effects are itching and nausea. Exparel should not be used for patients under the age of 18 and should be used cautiously in patients with hepatic disease.

FOR MORE INFORMATION, VISIT: https://www.exparel.com/

M GENITALIUM ASSAY DETECTS THE STI

Hologic’s Aptima^® Mycoplasma genitalium assay is the first FDA-cleared diagnostic test for this sexually transmitted infection (STI), which has been identified by the Centers for Disease Control and Prevention as an emerging public health threat. The assay is an in vitro nucleic acid amplification test that can be used to verify swab or urine samples from women and men. In published studies, the ribosomal RNA-based assay displayed greater sensitivity than lab-developed or CE-marked DNA-based tests. Early detection is important, Hologic asserts, because M genitalium is increasing in prevalence among higher-risk populations; however, it is not well known and often misdiagnosed, leading to incorrect treatment as well as risk for transmission and recurrence.

Hologic cites several studies that have shown M genitalium can be asymptomatic; however, it also can be associated with nongonococcal urethritis in men and cervicitis in women, as well as increased risk for pelvic inflammatory disease, preterm birth, spontaneous abortion, and infertility. A high percentage of infected people have an antibiotic-resistant strain, demonstrating a need for early detection and screening.

FOR MORE INFORMATION, VISIT: https://www.hologic.com

NEW ART OPTION

FOR MORE INFORMATION, VISIT: https://www.ferringusa.com

MENSTRUAL PAIN RELIEF THROUGH MICRO-PULSES

Livia, by iPulse Medical Ltd, is a US Food and Drug Administration (FDA) approved, drug-free option to treat menstrual pain through the transmission of electrical pulses. Electrodes are placed on the body at the source of menstrual pain and send a frequency to the nerves to reduce pain. Livia was designed based on the principles of the “gate control” theory of pain, says iPulse Medical. When the nerves are stimulated by the device’s electrodes, the nerve gate is closed, preventing pain signals from being received or felt in the brain.

The device can be worn in public or at home and allows the user to adjust the frequency of the electrical signal to correspond with her pain intensity. According to iPulse Medical, there are no adverse effects and the user will not build up a tolerance; however, the device should not be worn if the user has a pacemaker or is undergoing fertility treatment.

FOR MORE INFORMATION, VISIT: https://mylivia.com/

EXPAREL FOR CESAREAN DELIVERY

Pacira BioSciences an-nounced completion of their Phase 4 study of Exparel (bupivacaine lipsome injectable suspension), a local analgesic given to patients undergoing planned cesarean delivery (CD), aimed at reducing postsurgical pain and total opioid consumption through the first 72 hours postsurgery. Exparel is administered through transversus abdominis plane field block.

Pacira’s multicenter, randomized, double-blind study of 186 patients showed that those receiving Exparel plus bupivacaine HCl had a 52% reduction in total opioid consumption and significantly lower pain scores through the first 72 hours after CD, compared with those receiving only bupivacaine HCl. The most common adverse effects are itching and nausea. Exparel should not be used for patients under the age of 18 and should be used cautiously in patients with hepatic disease.

FOR MORE INFORMATION, VISIT: https://www.exparel.com/

M GENITALIUM ASSAY DETECTS THE STI

Hologic’s Aptima^® Mycoplasma genitalium assay is the first FDA-cleared diagnostic test for this sexually transmitted infection (STI), which has been identified by the Centers for Disease Control and Prevention as an emerging public health threat. The assay is an in vitro nucleic acid amplification test that can be used to verify swab or urine samples from women and men. In published studies, the ribosomal RNA-based assay displayed greater sensitivity than lab-developed or CE-marked DNA-based tests. Early detection is important, Hologic asserts, because M genitalium is increasing in prevalence among higher-risk populations; however, it is not well known and often misdiagnosed, leading to incorrect treatment as well as risk for transmission and recurrence.

Hologic cites several studies that have shown M genitalium can be asymptomatic; however, it also can be associated with nongonococcal urethritis in men and cervicitis in women, as well as increased risk for pelvic inflammatory disease, preterm birth, spontaneous abortion, and infertility. A high percentage of infected people have an antibiotic-resistant strain, demonstrating a need for early detection and screening.

FOR MORE INFORMATION, VISIT: https://www.hologic.com

NEW ART OPTION

FOR MORE INFORMATION, VISIT: https://www.ferringusa.com

To the Editor:

Extramammary Paget disease (EMPD) is a rare intraepidermal neoplasm with glandular differentiation that is classically known as a mimicker of Bowen disease (squamous cell carcinoma in situ of the skin) due to their histologic similarities.^1,2 However, acantholytic anaplastic EMPD (AAEMPD) is a rare variant that can pose a particularly difficult diagnostic challenge because of its histologic similarity to benign acantholytic disorders and other malignant neoplasms. Major histologic features suggestive of AAEMPD include full-thickness atypia of the epidermis, loss of nuclear polarity, marked cytologic anaplasia, intraepidermal acantholysis, and Paget cells.³ The differential diagnosis of EMPD typically includes Bowen disease and pagetoid Bowen disease, but the acantholytic anaplastic variant more often is confused with intraepidermal acantholytic lesions such as acantholytic dyskeratosis of the genitocrural area, familial benign pemphigus (Hailey-Hailey disease), pemphigus vulgaris, and acantholytic Bowen disease. Immunohistochemistry (IHC) studies to assist in the definitive diagnosis of AAEMPD are strongly advised because of these difficulties in diagnosis.⁴ Cases of EMPD with an acantholytic appearance have rarely been reported in the literature.^5-7

A 78-year-old man with a history of arthritis, heart disease, hypertension, and gastrointestinal disease presented for evaluation of a tender lesion of the right genitocrural crease of 5 years’ duration. He had no history of cutaneous or internal malignancy. Previously the lesion had been treated by dermatology with a variety of topical products including antifungal and antibiotic creams with no improvement. Physical examination revealed a well-defined, 7×5-cm, tender, erythematous, macerated plaque on the right upper inner thigh adjacent to the scrotum with an odor possibly due to secondary infection (Figure 1).

The biopsy specimen was examined grossly, serially sectioned, and submitted for routine processing with hematoxylin and eosin, periodic acid–Schiff, and Hale colloidal iron staining. Routine IHC was performed with antibodies to cytokeratin (CK) 7, CK20, carcinoembryonic antigen (CEA), pancytokeratin (CKAE1/AE3), and low- molecular-weight cytokeratin (LMWCK).

Pathologic examination of the biopsy showed prominent acanthosis of the epidermis composed of a proliferation of epithelial cells with associated full-thickness suprabasal acantholysis (Figure 2A). On inspection at higher magnification, the neoplastic cells demonstrated anaplasia as cytologic atypia with prominent and frequently multiple nucleoli, scant cytoplasm, and a high nuclear to cytoplasmic ratio (Figure 2B). There was a marked increase in mitotic activity with as many as 5 mitotic figures per high-power field. A fairly dense mixed inflammatory infiltrate comprised of lymphocytes, plasma cells, neutrophils, and eosinophils was present in the dermis. No fungal elements were observed on periodic acid–Schiff staining. The vast majority of tumor cells demonstrated moderate to abundant cytoplasmic mucin on Hale colloidal iron staining (Figure 3).

Immunohistochemistry staining of tumor cells was positive for CK7, CEA, pancytokeratin (CKAE1/AE3), and LMWCK. The tumor cells were negative for CK20. On the basis of the histopathologic and IHC findings, the patient was diagnosed with AAEMPD.

Extramammary Paget disease is a rare intraepidermal neoplasm with glandular differentiation. The most commonly involved sites are the anogenital areas including the vulvar, perianal, perineal, scrotal, and penile regions, as well as other areas rich in apocrine glands such as the axillae.⁸ Extramammary Paget disease most commonly originates as a primary intraepidermal neoplasm (type 1 EMPD), but an underlying malignant neoplasm that spreads intraepithelially is seen in a minority of cases (types 2 and 3 EMPD). In the vulva, type 1a refers to cutaneous noninvasive Paget disease, type 1b refers to dermal invasion of Paget disease, type 1c refers to vulvar adenocarcinoma–associated Paget disease, type 2 refers to rectal/anal adenocarcinoma–associated Paget disease, and type 3 refers to urogenital neoplasia–associated Paget disease.⁹

The acantholytic anaplastic variant of EMPD can be challenging to diagnose because of its similarities to many other lesions, including acantholytic dyskeratosis of the genitocrural area, familial benign pemphigus (Hailey-Hailey disease), pemphigus vulgaris, Bowen disease, pagetoid Bowen disease, and acantholytic Bowen disease. Major histologic features of AAEMPD include full-thickness atypia of the epidermis, loss of nuclear polarity, marked cytologic anaplasia, intraepidermal acantholysis, and Paget cells.³ The acantholytic anaplastic variant of EMPD can be differentiated from other diagnoses using IHC studies, with findings indicative of AAEMPD outlined below.

The proliferative neoplastic cell in EMPD is the Paget cell, which can be identified as a large round cell located in the epidermis with pale-staining cytoplasm, a large nucleus, and sometimes a prominent nucleolus. Paget cells can be distributed singly or in clusters, nests, or glandular structures within the epidermis and adjacent to adnexal structures.¹⁰ Extramammary Paget disease can have many patterns, including glandular, acantholysis-like, upper nest, tall nest, budding, and sheetlike.¹¹

Immunohistochemically, Paget cells in EMPD typically express pancytokeratins (CKAE1/AE3), low-molecular-weight/simple epithelial type keratins (CK7, CAM 5.2), sweat gland antigens (epithelial membrane antigen, CEA, gross cystic disease fluid protein 15 [GCDFP15]), mucin 5AC (MUC5AC), and often androgen receptor.^12-18 Paget cells contain cytoplasmic mucin and demonstrate prominent cytoplasmic staining with Hale colloidal iron.¹⁷ Paget cells typically do not express high-molecular-weight cytokeratin (eg, CK5/6), melanocytic antigens, estrogen receptor, or progesterone receptor.^15,18

Immunohistochemical staining has been shown to differ between primary cutaneous (type 1) and secondary (types 2 and 3) EMPD. Primary cutaneous EMPD typically expresses sweat gland markers (CK7⁺, CK20⁻, GCDFP15⁺). Secondary EMPD typically expresses an endodermal phenotype (CK7⁺, CK20⁺, GCDFP15⁻).¹²

Acantholytic dyskeratosis of the genitocrural area is a rare lesion included in the spectrum of focal acantholytic dyskeratoses described by Ackerman.¹⁹ It also has been referred to as papular acantholytic dyskeratosis of the vulva, though histologically similar lesions also have been reported in men.^20-22 Histologically, acantholytic dyskeratosis of the genitocrural area has prominent acantholysis and dyskeratosis with corps ronds and grains.¹⁹ Familial benign pemphigus (Hailey-Hailey disease) is caused by mutations of the ATP2C1 gene, which encodes for a secretory pathway Ca²⁺/Mn²⁺-ATPase pump type 1 (SPCA1) in the Golgi apparatus in keratinocytes.²³ Familial benign pemphigus has a histologic appearance similar to acantholytic dyskeratosis of the genitocrural area, but a positive family history of familial benign pemphigus can be used to differentiate the 2 entities from each other due to the autosomal-dominant inheritance pattern of familial benign pemphigus. Both of these disorders can appear similar to AAEMPD because of their extensive intraepidermal acantholysis, but they differ in the lack of Paget cells, intraepidermal atypia, and increased mitotic activity.

Acantholytic Bowen disease is a histologic variant that can be difficult to distinguish from AAEMPD on hematoxylin and eosin–stained sections because of their similar histologic features but can be differentiated by IHC stains.⁵ Acantholytic Bowen disease expresses high-molecular-weight cytokeratin (eg, CK5/6) but is negative for CK7, CAM 5.2, and CEA. Extramammary Paget disease generally has the opposite pattern: positive staining for CK7, CAM 5.2, and CEA, but negative for high-molecular-weight cytokeratin.^13,14,24

Primary cutaneous adenosquamous carcinoma is a rare malignancy of squamous and glandular differentiation known for being locally aggressive and metastatic.²⁵ Histologically, cutaneous adenosquamous carcinoma shows infiltrating nests of neoplastic cells with both squamous and glandular features. It differs notably from AAEMPD in that cutaneous adenosquamous carcinomas tend to arise in the head and arm regions, and their histologic morphology is different. The IHC profiles are similar, with positive staining for CEA, CK7, and mucin; however, they differ in that AAEMPD is negative for high-molecular-weight keratin while cutaneous adenosquamous carcinoma is positive.²⁵

Verrucous carcinoma is an uncommon variant of squamous cell carcinoma with well-differentiated keratinocytes and a blunt pushing border.²⁴ Similar to AAEMPD, this neoplasm can arise in the genital and perineal areas; however, the 2 entities differ considerably in morphology on histologic examination.

Pemphigus vulgaris is an autoimmune intraepidermal blistering disorder of the skin and mucous membranes of which pemphigus vegetans is a subtype.^26,27 Pemphigus vulgaris is another diagnosis that can possibly be mimicked by AAEMPD.²⁸ Histologic features of pemphigus vulgaris include intraepidermal acantholysis of keratinocytes immediately above the basal layer of the epidermis. Pemphigus vegetans is similar with the addition of papillomatosis, hyperkeratosis, and an eosinophilic infiltrate.^26,27 Immunofluorescence typically demonstrates intercellular C3 and IgG deposits.²⁶ These diseases mimic AAEMPD histologically but differ in their relative lack of atypia and Paget cells.

In summary, we report a case of AAEMPD in a 78-year-old man in whom routine histologic specimens showed marked intraepidermal acantholysis and atypical tumor cells with increased mitoses. The latter finding prompted IHC studies that revealed positive CK7, CEA, pancytokeratin, and LMWCK staining with negative CK20 staining. Hale colloidal iron staining showed moderate to abundant cytoplasmic mucin. The patient was diagnosed with AAEMPD. It is imperative to maintain clinical suspicion for AAEMPD and to examine acantholytic disorders with scrutiny. When there is evidence of atypia or mitoses, use of IHC stains can assist in fully characterizing the lesion.

To the Editor:

Extramammary Paget disease (EMPD) is a rare intraepidermal neoplasm with glandular differentiation that is classically known as a mimicker of Bowen disease (squamous cell carcinoma in situ of the skin) due to their histologic similarities.^1,2 However, acantholytic anaplastic EMPD (AAEMPD) is a rare variant that can pose a particularly difficult diagnostic challenge because of its histologic similarity to benign acantholytic disorders and other malignant neoplasms. Major histologic features suggestive of AAEMPD include full-thickness atypia of the epidermis, loss of nuclear polarity, marked cytologic anaplasia, intraepidermal acantholysis, and Paget cells.³ The differential diagnosis of EMPD typically includes Bowen disease and pagetoid Bowen disease, but the acantholytic anaplastic variant more often is confused with intraepidermal acantholytic lesions such as acantholytic dyskeratosis of the genitocrural area, familial benign pemphigus (Hailey-Hailey disease), pemphigus vulgaris, and acantholytic Bowen disease. Immunohistochemistry (IHC) studies to assist in the definitive diagnosis of AAEMPD are strongly advised because of these difficulties in diagnosis.⁴ Cases of EMPD with an acantholytic appearance have rarely been reported in the literature.^5-7

A 78-year-old man with a history of arthritis, heart disease, hypertension, and gastrointestinal disease presented for evaluation of a tender lesion of the right genitocrural crease of 5 years’ duration. He had no history of cutaneous or internal malignancy. Previously the lesion had been treated by dermatology with a variety of topical products including antifungal and antibiotic creams with no improvement. Physical examination revealed a well-defined, 7×5-cm, tender, erythematous, macerated plaque on the right upper inner thigh adjacent to the scrotum with an odor possibly due to secondary infection (Figure 1).

The biopsy specimen was examined grossly, serially sectioned, and submitted for routine processing with hematoxylin and eosin, periodic acid–Schiff, and Hale colloidal iron staining. Routine IHC was performed with antibodies to cytokeratin (CK) 7, CK20, carcinoembryonic antigen (CEA), pancytokeratin (CKAE1/AE3), and low- molecular-weight cytokeratin (LMWCK).

Pathologic examination of the biopsy showed prominent acanthosis of the epidermis composed of a proliferation of epithelial cells with associated full-thickness suprabasal acantholysis (Figure 2A). On inspection at higher magnification, the neoplastic cells demonstrated anaplasia as cytologic atypia with prominent and frequently multiple nucleoli, scant cytoplasm, and a high nuclear to cytoplasmic ratio (Figure 2B). There was a marked increase in mitotic activity with as many as 5 mitotic figures per high-power field. A fairly dense mixed inflammatory infiltrate comprised of lymphocytes, plasma cells, neutrophils, and eosinophils was present in the dermis. No fungal elements were observed on periodic acid–Schiff staining. The vast majority of tumor cells demonstrated moderate to abundant cytoplasmic mucin on Hale colloidal iron staining (Figure 3).

Immunohistochemistry staining of tumor cells was positive for CK7, CEA, pancytokeratin (CKAE1/AE3), and LMWCK. The tumor cells were negative for CK20. On the basis of the histopathologic and IHC findings, the patient was diagnosed with AAEMPD.

Extramammary Paget disease is a rare intraepidermal neoplasm with glandular differentiation. The most commonly involved sites are the anogenital areas including the vulvar, perianal, perineal, scrotal, and penile regions, as well as other areas rich in apocrine glands such as the axillae.⁸ Extramammary Paget disease most commonly originates as a primary intraepidermal neoplasm (type 1 EMPD), but an underlying malignant neoplasm that spreads intraepithelially is seen in a minority of cases (types 2 and 3 EMPD). In the vulva, type 1a refers to cutaneous noninvasive Paget disease, type 1b refers to dermal invasion of Paget disease, type 1c refers to vulvar adenocarcinoma–associated Paget disease, type 2 refers to rectal/anal adenocarcinoma–associated Paget disease, and type 3 refers to urogenital neoplasia–associated Paget disease.⁹

The acantholytic anaplastic variant of EMPD can be challenging to diagnose because of its similarities to many other lesions, including acantholytic dyskeratosis of the genitocrural area, familial benign pemphigus (Hailey-Hailey disease), pemphigus vulgaris, Bowen disease, pagetoid Bowen disease, and acantholytic Bowen disease. Major histologic features of AAEMPD include full-thickness atypia of the epidermis, loss of nuclear polarity, marked cytologic anaplasia, intraepidermal acantholysis, and Paget cells.³ The acantholytic anaplastic variant of EMPD can be differentiated from other diagnoses using IHC studies, with findings indicative of AAEMPD outlined below.

The proliferative neoplastic cell in EMPD is the Paget cell, which can be identified as a large round cell located in the epidermis with pale-staining cytoplasm, a large nucleus, and sometimes a prominent nucleolus. Paget cells can be distributed singly or in clusters, nests, or glandular structures within the epidermis and adjacent to adnexal structures.¹⁰ Extramammary Paget disease can have many patterns, including glandular, acantholysis-like, upper nest, tall nest, budding, and sheetlike.¹¹

Immunohistochemically, Paget cells in EMPD typically express pancytokeratins (CKAE1/AE3), low-molecular-weight/simple epithelial type keratins (CK7, CAM 5.2), sweat gland antigens (epithelial membrane antigen, CEA, gross cystic disease fluid protein 15 [GCDFP15]), mucin 5AC (MUC5AC), and often androgen receptor.^12-18 Paget cells contain cytoplasmic mucin and demonstrate prominent cytoplasmic staining with Hale colloidal iron.¹⁷ Paget cells typically do not express high-molecular-weight cytokeratin (eg, CK5/6), melanocytic antigens, estrogen receptor, or progesterone receptor.^15,18

Immunohistochemical staining has been shown to differ between primary cutaneous (type 1) and secondary (types 2 and 3) EMPD. Primary cutaneous EMPD typically expresses sweat gland markers (CK7⁺, CK20⁻, GCDFP15⁺). Secondary EMPD typically expresses an endodermal phenotype (CK7⁺, CK20⁺, GCDFP15⁻).¹²

Acantholytic dyskeratosis of the genitocrural area is a rare lesion included in the spectrum of focal acantholytic dyskeratoses described by Ackerman.¹⁹ It also has been referred to as papular acantholytic dyskeratosis of the vulva, though histologically similar lesions also have been reported in men.^20-22 Histologically, acantholytic dyskeratosis of the genitocrural area has prominent acantholysis and dyskeratosis with corps ronds and grains.¹⁹ Familial benign pemphigus (Hailey-Hailey disease) is caused by mutations of the ATP2C1 gene, which encodes for a secretory pathway Ca²⁺/Mn²⁺-ATPase pump type 1 (SPCA1) in the Golgi apparatus in keratinocytes.²³ Familial benign pemphigus has a histologic appearance similar to acantholytic dyskeratosis of the genitocrural area, but a positive family history of familial benign pemphigus can be used to differentiate the 2 entities from each other due to the autosomal-dominant inheritance pattern of familial benign pemphigus. Both of these disorders can appear similar to AAEMPD because of their extensive intraepidermal acantholysis, but they differ in the lack of Paget cells, intraepidermal atypia, and increased mitotic activity.

Acantholytic Bowen disease is a histologic variant that can be difficult to distinguish from AAEMPD on hematoxylin and eosin–stained sections because of their similar histologic features but can be differentiated by IHC stains.⁵ Acantholytic Bowen disease expresses high-molecular-weight cytokeratin (eg, CK5/6) but is negative for CK7, CAM 5.2, and CEA. Extramammary Paget disease generally has the opposite pattern: positive staining for CK7, CAM 5.2, and CEA, but negative for high-molecular-weight cytokeratin.^13,14,24

Primary cutaneous adenosquamous carcinoma is a rare malignancy of squamous and glandular differentiation known for being locally aggressive and metastatic.²⁵ Histologically, cutaneous adenosquamous carcinoma shows infiltrating nests of neoplastic cells with both squamous and glandular features. It differs notably from AAEMPD in that cutaneous adenosquamous carcinomas tend to arise in the head and arm regions, and their histologic morphology is different. The IHC profiles are similar, with positive staining for CEA, CK7, and mucin; however, they differ in that AAEMPD is negative for high-molecular-weight keratin while cutaneous adenosquamous carcinoma is positive.²⁵

Verrucous carcinoma is an uncommon variant of squamous cell carcinoma with well-differentiated keratinocytes and a blunt pushing border.²⁴ Similar to AAEMPD, this neoplasm can arise in the genital and perineal areas; however, the 2 entities differ considerably in morphology on histologic examination.

Pemphigus vulgaris is an autoimmune intraepidermal blistering disorder of the skin and mucous membranes of which pemphigus vegetans is a subtype.^26,27 Pemphigus vulgaris is another diagnosis that can possibly be mimicked by AAEMPD.²⁸ Histologic features of pemphigus vulgaris include intraepidermal acantholysis of keratinocytes immediately above the basal layer of the epidermis. Pemphigus vegetans is similar with the addition of papillomatosis, hyperkeratosis, and an eosinophilic infiltrate.^26,27 Immunofluorescence typically demonstrates intercellular C3 and IgG deposits.²⁶ These diseases mimic AAEMPD histologically but differ in their relative lack of atypia and Paget cells.

In summary, we report a case of AAEMPD in a 78-year-old man in whom routine histologic specimens showed marked intraepidermal acantholysis and atypical tumor cells with increased mitoses. The latter finding prompted IHC studies that revealed positive CK7, CEA, pancytokeratin, and LMWCK staining with negative CK20 staining. Hale colloidal iron staining showed moderate to abundant cytoplasmic mucin. The patient was diagnosed with AAEMPD. It is imperative to maintain clinical suspicion for AAEMPD and to examine acantholytic disorders with scrutiny. When there is evidence of atypia or mitoses, use of IHC stains can assist in fully characterizing the lesion.

To the Editor:

Extramammary Paget disease (EMPD) is a rare intraepidermal neoplasm with glandular differentiation that is classically known as a mimicker of Bowen disease (squamous cell carcinoma in situ of the skin) due to their histologic similarities.^1,2 However, acantholytic anaplastic EMPD (AAEMPD) is a rare variant that can pose a particularly difficult diagnostic challenge because of its histologic similarity to benign acantholytic disorders and other malignant neoplasms. Major histologic features suggestive of AAEMPD include full-thickness atypia of the epidermis, loss of nuclear polarity, marked cytologic anaplasia, intraepidermal acantholysis, and Paget cells.³ The differential diagnosis of EMPD typically includes Bowen disease and pagetoid Bowen disease, but the acantholytic anaplastic variant more often is confused with intraepidermal acantholytic lesions such as acantholytic dyskeratosis of the genitocrural area, familial benign pemphigus (Hailey-Hailey disease), pemphigus vulgaris, and acantholytic Bowen disease. Immunohistochemistry (IHC) studies to assist in the definitive diagnosis of AAEMPD are strongly advised because of these difficulties in diagnosis.⁴ Cases of EMPD with an acantholytic appearance have rarely been reported in the literature.^5-7

A 78-year-old man with a history of arthritis, heart disease, hypertension, and gastrointestinal disease presented for evaluation of a tender lesion of the right genitocrural crease of 5 years’ duration. He had no history of cutaneous or internal malignancy. Previously the lesion had been treated by dermatology with a variety of topical products including antifungal and antibiotic creams with no improvement. Physical examination revealed a well-defined, 7×5-cm, tender, erythematous, macerated plaque on the right upper inner thigh adjacent to the scrotum with an odor possibly due to secondary infection (Figure 1).

The biopsy specimen was examined grossly, serially sectioned, and submitted for routine processing with hematoxylin and eosin, periodic acid–Schiff, and Hale colloidal iron staining. Routine IHC was performed with antibodies to cytokeratin (CK) 7, CK20, carcinoembryonic antigen (CEA), pancytokeratin (CKAE1/AE3), and low- molecular-weight cytokeratin (LMWCK).

Pathologic examination of the biopsy showed prominent acanthosis of the epidermis composed of a proliferation of epithelial cells with associated full-thickness suprabasal acantholysis (Figure 2A). On inspection at higher magnification, the neoplastic cells demonstrated anaplasia as cytologic atypia with prominent and frequently multiple nucleoli, scant cytoplasm, and a high nuclear to cytoplasmic ratio (Figure 2B). There was a marked increase in mitotic activity with as many as 5 mitotic figures per high-power field. A fairly dense mixed inflammatory infiltrate comprised of lymphocytes, plasma cells, neutrophils, and eosinophils was present in the dermis. No fungal elements were observed on periodic acid–Schiff staining. The vast majority of tumor cells demonstrated moderate to abundant cytoplasmic mucin on Hale colloidal iron staining (Figure 3).

Immunohistochemistry staining of tumor cells was positive for CK7, CEA, pancytokeratin (CKAE1/AE3), and LMWCK. The tumor cells were negative for CK20. On the basis of the histopathologic and IHC findings, the patient was diagnosed with AAEMPD.

Extramammary Paget disease is a rare intraepidermal neoplasm with glandular differentiation. The most commonly involved sites are the anogenital areas including the vulvar, perianal, perineal, scrotal, and penile regions, as well as other areas rich in apocrine glands such as the axillae.⁸ Extramammary Paget disease most commonly originates as a primary intraepidermal neoplasm (type 1 EMPD), but an underlying malignant neoplasm that spreads intraepithelially is seen in a minority of cases (types 2 and 3 EMPD). In the vulva, type 1a refers to cutaneous noninvasive Paget disease, type 1b refers to dermal invasion of Paget disease, type 1c refers to vulvar adenocarcinoma–associated Paget disease, type 2 refers to rectal/anal adenocarcinoma–associated Paget disease, and type 3 refers to urogenital neoplasia–associated Paget disease.⁹

The acantholytic anaplastic variant of EMPD can be challenging to diagnose because of its similarities to many other lesions, including acantholytic dyskeratosis of the genitocrural area, familial benign pemphigus (Hailey-Hailey disease), pemphigus vulgaris, Bowen disease, pagetoid Bowen disease, and acantholytic Bowen disease. Major histologic features of AAEMPD include full-thickness atypia of the epidermis, loss of nuclear polarity, marked cytologic anaplasia, intraepidermal acantholysis, and Paget cells.³ The acantholytic anaplastic variant of EMPD can be differentiated from other diagnoses using IHC studies, with findings indicative of AAEMPD outlined below.

The proliferative neoplastic cell in EMPD is the Paget cell, which can be identified as a large round cell located in the epidermis with pale-staining cytoplasm, a large nucleus, and sometimes a prominent nucleolus. Paget cells can be distributed singly or in clusters, nests, or glandular structures within the epidermis and adjacent to adnexal structures.¹⁰ Extramammary Paget disease can have many patterns, including glandular, acantholysis-like, upper nest, tall nest, budding, and sheetlike.¹¹

Immunohistochemically, Paget cells in EMPD typically express pancytokeratins (CKAE1/AE3), low-molecular-weight/simple epithelial type keratins (CK7, CAM 5.2), sweat gland antigens (epithelial membrane antigen, CEA, gross cystic disease fluid protein 15 [GCDFP15]), mucin 5AC (MUC5AC), and often androgen receptor.^12-18 Paget cells contain cytoplasmic mucin and demonstrate prominent cytoplasmic staining with Hale colloidal iron.¹⁷ Paget cells typically do not express high-molecular-weight cytokeratin (eg, CK5/6), melanocytic antigens, estrogen receptor, or progesterone receptor.^15,18

Immunohistochemical staining has been shown to differ between primary cutaneous (type 1) and secondary (types 2 and 3) EMPD. Primary cutaneous EMPD typically expresses sweat gland markers (CK7⁺, CK20⁻, GCDFP15⁺). Secondary EMPD typically expresses an endodermal phenotype (CK7⁺, CK20⁺, GCDFP15⁻).¹²

Acantholytic dyskeratosis of the genitocrural area is a rare lesion included in the spectrum of focal acantholytic dyskeratoses described by Ackerman.¹⁹ It also has been referred to as papular acantholytic dyskeratosis of the vulva, though histologically similar lesions also have been reported in men.^20-22 Histologically, acantholytic dyskeratosis of the genitocrural area has prominent acantholysis and dyskeratosis with corps ronds and grains.¹⁹ Familial benign pemphigus (Hailey-Hailey disease) is caused by mutations of the ATP2C1 gene, which encodes for a secretory pathway Ca²⁺/Mn²⁺-ATPase pump type 1 (SPCA1) in the Golgi apparatus in keratinocytes.²³ Familial benign pemphigus has a histologic appearance similar to acantholytic dyskeratosis of the genitocrural area, but a positive family history of familial benign pemphigus can be used to differentiate the 2 entities from each other due to the autosomal-dominant inheritance pattern of familial benign pemphigus. Both of these disorders can appear similar to AAEMPD because of their extensive intraepidermal acantholysis, but they differ in the lack of Paget cells, intraepidermal atypia, and increased mitotic activity.

Acantholytic Bowen disease is a histologic variant that can be difficult to distinguish from AAEMPD on hematoxylin and eosin–stained sections because of their similar histologic features but can be differentiated by IHC stains.⁵ Acantholytic Bowen disease expresses high-molecular-weight cytokeratin (eg, CK5/6) but is negative for CK7, CAM 5.2, and CEA. Extramammary Paget disease generally has the opposite pattern: positive staining for CK7, CAM 5.2, and CEA, but negative for high-molecular-weight cytokeratin.^13,14,24

Primary cutaneous adenosquamous carcinoma is a rare malignancy of squamous and glandular differentiation known for being locally aggressive and metastatic.²⁵ Histologically, cutaneous adenosquamous carcinoma shows infiltrating nests of neoplastic cells with both squamous and glandular features. It differs notably from AAEMPD in that cutaneous adenosquamous carcinomas tend to arise in the head and arm regions, and their histologic morphology is different. The IHC profiles are similar, with positive staining for CEA, CK7, and mucin; however, they differ in that AAEMPD is negative for high-molecular-weight keratin while cutaneous adenosquamous carcinoma is positive.²⁵

Verrucous carcinoma is an uncommon variant of squamous cell carcinoma with well-differentiated keratinocytes and a blunt pushing border.²⁴ Similar to AAEMPD, this neoplasm can arise in the genital and perineal areas; however, the 2 entities differ considerably in morphology on histologic examination.

Pemphigus vulgaris is an autoimmune intraepidermal blistering disorder of the skin and mucous membranes of which pemphigus vegetans is a subtype.^26,27 Pemphigus vulgaris is another diagnosis that can possibly be mimicked by AAEMPD.²⁸ Histologic features of pemphigus vulgaris include intraepidermal acantholysis of keratinocytes immediately above the basal layer of the epidermis. Pemphigus vegetans is similar with the addition of papillomatosis, hyperkeratosis, and an eosinophilic infiltrate.^26,27 Immunofluorescence typically demonstrates intercellular C3 and IgG deposits.²⁶ These diseases mimic AAEMPD histologically but differ in their relative lack of atypia and Paget cells.

In summary, we report a case of AAEMPD in a 78-year-old man in whom routine histologic specimens showed marked intraepidermal acantholysis and atypical tumor cells with increased mitoses. The latter finding prompted IHC studies that revealed positive CK7, CEA, pancytokeratin, and LMWCK staining with negative CK20 staining. Hale colloidal iron staining showed moderate to abundant cytoplasmic mucin. The patient was diagnosed with AAEMPD. It is imperative to maintain clinical suspicion for AAEMPD and to examine acantholytic disorders with scrutiny. When there is evidence of atypia or mitoses, use of IHC stains can assist in fully characterizing the lesion.

These are challenging, and sometimes tragic, times in the history of the United States. The image of a father and child face down in the Rio Grande River, drowning as they tried to cross from Mexico into Texas, is heart breaking. Irrespective of your political affiliation, we can agree that the immigration process is far from ideal and that no one should die in pursuit of a better life.

The United States has a complicated history with equity and inclusion, for all persons, and we are now living in times when the scab is being ripped off and these wounds are raw. What role can the Society of Hospital Medicine play to help heal these wounds?

I am a first-generation immigrant to the United States. I remember walking down the streets of my neighborhood in Uganda when my attention was drawn to a plane flying overhead. I thought to myself, “Some lucky duck is going to the U.S.” The United States was the land of opportunity and I was determined to come here. Through hard work and some luck, I arrived in the United States on June 15, 1991, with a single suitcase packed full of hope, dreams, and $3,000.

Fast-forward 28 years. I am now a hospitalist and faculty at the Johns Hopkins University, Baltimore, the associate director of the division of hospital medicine, and the vice chair for clinical operations at Johns Hopkins Bayview Medical Center. I learned about hospital medicine during my third year of medical school at the University of Minnesota, Minneapolis. While I loved general medicine, I could not see myself practicing anywhere outside of the hospital.

Following residency at Johns Hopkins Bayview, I still felt that a hospital-based practice was tailor-made for me. As I matured professionally, I worked to improve the provision of care within my hospital, and then started developing educational and practice programs in hospital medicine, both locally and internationally. My passion for hospital medicine led me to serve on committees for SHM, and this year, I was honored to join the SHM Board of Directors.

It is hard to answer the question of why, or how, one person immigrates to the United States and finds success while another loses their life. A quote attributed to Edmund Burke says, “the only thing necessary for the triumph of evil is for good [wo]men to do nothing.” One of SHM’s core values is to promote diversity and inclusion. A major step taken by the society to promote work in this area was to establish the diversity and inclusion Special Interest Group in 2018. I am the board liaison for the diversity and inclusion SIG and will work alongside this group, which aims to:

• Foster diversity, equity, and inclusion in SHM.
• Increase visibility of diversity, equity, and inclusion to the broader hospital medicine community.
• Support hospital medicine groups in matching their work forces to their diverse patient populations.
• Develop tool kits to improve the provision of care for our diverse patient population.
• Engender diversity among hospitalists.
• Develop opportunities for expanding the fund of knowledge on diversity in hospital medicine through research and discovery.
• Participate in SHM’s advocacy efforts related to diversity and inclusion.
• Develop partnerships with other key organizations to advance diversity, equity, and inclusion platforms so as to increase the scalability of SHM’s efforts.

We have been successful at Hopkins with diversity and inclusion, but that did not occur by chance. I believe that diversity and inclusion is a team sport and that everyone can be an important part of that team. In my hospitalist group, we actively engage women, men, doctors, NPs, PAs, administrators, minorities, and nonminorities. We recruit to – and cherish members of – our group irrespective of religious beliefs or sexual orientation. We believe that a heterogeneous group of people leads to an engaged and high-performing culture.

I have traveled a convoluted path since my arrival in 1991. Along the way, I was blessed with a husband and son who anchor me. Every day they remind me that the hard work I do is to build on the past to improve the future. My husband, an immigrant from Uganda like me, reminds me that we are lucky to have made it to the United States and that the ability and freedom to work hard and be rewarded for that hard work is a great privilege. My son reminds me of the many other children who look at me and know that they too can dare to dream. Occasionally, I still look up and see a plane, and I am reminded of that day many years ago. Hospital medicine is my suitcase packed with hopes and dreams for me, for this specialty, and for this country.

Dr. Kisuule is associate director of the division of hospital medicine at Johns Hopkins Bayview and assistant professor at Johns Hopkins University, both in Baltimore, and a member of the SHM Board of Directors.

These are challenging, and sometimes tragic, times in the history of the United States. The image of a father and child face down in the Rio Grande River, drowning as they tried to cross from Mexico into Texas, is heart breaking. Irrespective of your political affiliation, we can agree that the immigration process is far from ideal and that no one should die in pursuit of a better life.

The United States has a complicated history with equity and inclusion, for all persons, and we are now living in times when the scab is being ripped off and these wounds are raw. What role can the Society of Hospital Medicine play to help heal these wounds?

I am a first-generation immigrant to the United States. I remember walking down the streets of my neighborhood in Uganda when my attention was drawn to a plane flying overhead. I thought to myself, “Some lucky duck is going to the U.S.” The United States was the land of opportunity and I was determined to come here. Through hard work and some luck, I arrived in the United States on June 15, 1991, with a single suitcase packed full of hope, dreams, and $3,000.

Fast-forward 28 years. I am now a hospitalist and faculty at the Johns Hopkins University, Baltimore, the associate director of the division of hospital medicine, and the vice chair for clinical operations at Johns Hopkins Bayview Medical Center. I learned about hospital medicine during my third year of medical school at the University of Minnesota, Minneapolis. While I loved general medicine, I could not see myself practicing anywhere outside of the hospital.

Following residency at Johns Hopkins Bayview, I still felt that a hospital-based practice was tailor-made for me. As I matured professionally, I worked to improve the provision of care within my hospital, and then started developing educational and practice programs in hospital medicine, both locally and internationally. My passion for hospital medicine led me to serve on committees for SHM, and this year, I was honored to join the SHM Board of Directors.

It is hard to answer the question of why, or how, one person immigrates to the United States and finds success while another loses their life. A quote attributed to Edmund Burke says, “the only thing necessary for the triumph of evil is for good [wo]men to do nothing.” One of SHM’s core values is to promote diversity and inclusion. A major step taken by the society to promote work in this area was to establish the diversity and inclusion Special Interest Group in 2018. I am the board liaison for the diversity and inclusion SIG and will work alongside this group, which aims to:

• Foster diversity, equity, and inclusion in SHM.
• Increase visibility of diversity, equity, and inclusion to the broader hospital medicine community.
• Support hospital medicine groups in matching their work forces to their diverse patient populations.
• Develop tool kits to improve the provision of care for our diverse patient population.
• Engender diversity among hospitalists.
• Develop opportunities for expanding the fund of knowledge on diversity in hospital medicine through research and discovery.
• Participate in SHM’s advocacy efforts related to diversity and inclusion.
• Develop partnerships with other key organizations to advance diversity, equity, and inclusion platforms so as to increase the scalability of SHM’s efforts.

We have been successful at Hopkins with diversity and inclusion, but that did not occur by chance. I believe that diversity and inclusion is a team sport and that everyone can be an important part of that team. In my hospitalist group, we actively engage women, men, doctors, NPs, PAs, administrators, minorities, and nonminorities. We recruit to – and cherish members of – our group irrespective of religious beliefs or sexual orientation. We believe that a heterogeneous group of people leads to an engaged and high-performing culture.

I have traveled a convoluted path since my arrival in 1991. Along the way, I was blessed with a husband and son who anchor me. Every day they remind me that the hard work I do is to build on the past to improve the future. My husband, an immigrant from Uganda like me, reminds me that we are lucky to have made it to the United States and that the ability and freedom to work hard and be rewarded for that hard work is a great privilege. My son reminds me of the many other children who look at me and know that they too can dare to dream. Occasionally, I still look up and see a plane, and I am reminded of that day many years ago. Hospital medicine is my suitcase packed with hopes and dreams for me, for this specialty, and for this country.

Dr. Kisuule is associate director of the division of hospital medicine at Johns Hopkins Bayview and assistant professor at Johns Hopkins University, both in Baltimore, and a member of the SHM Board of Directors.

These are challenging, and sometimes tragic, times in the history of the United States. The image of a father and child face down in the Rio Grande River, drowning as they tried to cross from Mexico into Texas, is heart breaking. Irrespective of your political affiliation, we can agree that the immigration process is far from ideal and that no one should die in pursuit of a better life.

The United States has a complicated history with equity and inclusion, for all persons, and we are now living in times when the scab is being ripped off and these wounds are raw. What role can the Society of Hospital Medicine play to help heal these wounds?

I am a first-generation immigrant to the United States. I remember walking down the streets of my neighborhood in Uganda when my attention was drawn to a plane flying overhead. I thought to myself, “Some lucky duck is going to the U.S.” The United States was the land of opportunity and I was determined to come here. Through hard work and some luck, I arrived in the United States on June 15, 1991, with a single suitcase packed full of hope, dreams, and $3,000.

Fast-forward 28 years. I am now a hospitalist and faculty at the Johns Hopkins University, Baltimore, the associate director of the division of hospital medicine, and the vice chair for clinical operations at Johns Hopkins Bayview Medical Center. I learned about hospital medicine during my third year of medical school at the University of Minnesota, Minneapolis. While I loved general medicine, I could not see myself practicing anywhere outside of the hospital.

Following residency at Johns Hopkins Bayview, I still felt that a hospital-based practice was tailor-made for me. As I matured professionally, I worked to improve the provision of care within my hospital, and then started developing educational and practice programs in hospital medicine, both locally and internationally. My passion for hospital medicine led me to serve on committees for SHM, and this year, I was honored to join the SHM Board of Directors.

It is hard to answer the question of why, or how, one person immigrates to the United States and finds success while another loses their life. A quote attributed to Edmund Burke says, “the only thing necessary for the triumph of evil is for good [wo]men to do nothing.” One of SHM’s core values is to promote diversity and inclusion. A major step taken by the society to promote work in this area was to establish the diversity and inclusion Special Interest Group in 2018. I am the board liaison for the diversity and inclusion SIG and will work alongside this group, which aims to:

• Foster diversity, equity, and inclusion in SHM.
• Increase visibility of diversity, equity, and inclusion to the broader hospital medicine community.
• Support hospital medicine groups in matching their work forces to their diverse patient populations.
• Develop tool kits to improve the provision of care for our diverse patient population.
• Engender diversity among hospitalists.
• Develop opportunities for expanding the fund of knowledge on diversity in hospital medicine through research and discovery.
• Participate in SHM’s advocacy efforts related to diversity and inclusion.
• Develop partnerships with other key organizations to advance diversity, equity, and inclusion platforms so as to increase the scalability of SHM’s efforts.

We have been successful at Hopkins with diversity and inclusion, but that did not occur by chance. I believe that diversity and inclusion is a team sport and that everyone can be an important part of that team. In my hospitalist group, we actively engage women, men, doctors, NPs, PAs, administrators, minorities, and nonminorities. We recruit to – and cherish members of – our group irrespective of religious beliefs or sexual orientation. We believe that a heterogeneous group of people leads to an engaged and high-performing culture.

I have traveled a convoluted path since my arrival in 1991. Along the way, I was blessed with a husband and son who anchor me. Every day they remind me that the hard work I do is to build on the past to improve the future. My husband, an immigrant from Uganda like me, reminds me that we are lucky to have made it to the United States and that the ability and freedom to work hard and be rewarded for that hard work is a great privilege. My son reminds me of the many other children who look at me and know that they too can dare to dream. Occasionally, I still look up and see a plane, and I am reminded of that day many years ago. Hospital medicine is my suitcase packed with hopes and dreams for me, for this specialty, and for this country.

Dr. Kisuule is associate director of the division of hospital medicine at Johns Hopkins Bayview and assistant professor at Johns Hopkins University, both in Baltimore, and a member of the SHM Board of Directors.

To the Editor:

Leiomyomas are benign smooth muscle tumors. There are 3 types of cutaneous leiomyomas: (1) piloleiomyomas, arising from the arrector pili muscles; (2) angioleiomyomas, arising from the muscles surrounding dermal blood vessels; and (3) leiomyomas of the external genitalia, arising from the dartoic, vulvar, or mammary smooth muscles.¹ There is no gender predilection for cutaneous leiomyomas, and lesions present on average at approximately 40 to 45 years of age.²

Piloleiomyomas are the most common type of cutaneous leiomyomas and typically present as red-brown papules and nodules on the trunk, arms, and legs.³ Piloleiomyomas often are associated with spontaneous or induced pain (eg, with cold exposure). The pain associated with piloleiomyomas can be severely debilitating to patients and may have a considerable impact on their quality of life.

A 40-year-old woman presented to our clinic with numerous widespread, painful, red-brown papules and nodules on the head, neck, chest, abdomen, back, arms, and legs of 6 years’ duration that were increasing in number (Figure 1). She had a history of uterine leiomyomas and type 2 renal papillary carcinoma following a left nephrectomy at 38 years of age. The patient’s mother had a history of similar skin lesions as well as uterine cancer. Multiple excisional biopsies were performed, all of which showed piloleiomyomas on histopathology (Figure 2). The pain associated with the patient’s extensive cutaneous leiomyomas considerably impaired her quality of life. Although she experienced pain in all affected areas of the body, the pain was the worst in the upper arms. She reported having requested a nerve ablation procedure from an outside pain management clinic, which was denied for unknown reasons.

Two years prior to the current presentation, the patient had been treated by a pain management specialist with gabapentin 300 mg twice daily as needed for pain associated with leiomyomas. The patient followed this regimen approximately 3 times weekly for the preceding 1 to 2 years with reduction in her pain symptoms; however, the painful episodes became more frequent and severe over time. The patient reported being unable to further increase the gabapentin dosing frequency because it made her too drowsy and impacted her ability to work a job that required heavy lifting. Thus, the patient requested additional therapy and was subsequently treated at our clinic with numerous excisional biopsies of the most painful lesions during the 2 years prior to her current presentation.

When the patient re-presented to our clinic, she requested additional lesion excisions given that she had experienced some pain relief from this treatment modality in the past; however, these prior excisions only resulted in local pain relief limited to the site of the excision. Because of the extent of the lesions and the patient’s inability to tolerate pain from the lidocaine injections, we did not feel multiple excisions were a practical treatment option. The patient subsequently was offered a trial of cryotherapy for symptom relief based on a reported case in which this modality was successfully used.⁴ After discussing the risks and benefits associated with this treatment, cryotherapy was attempted on a few of the leiomyomas on the patient’s right shoulder; however, she experienced severe pain during cryotherapy treatment, and the procedure had to be aborted.

We then increased the patient’s gabapentin regimen to 300 mg in the morning and 600 mg in the evening, as tolerated. The patient reported that she was better able to tolerate the sedating side effects of the increased dose of gabapentin because she had stopped working due to her severe pain episodes. We also added oral nifedipine 10 mg 3 times daily, as needed. Within 30 minutes of starting this treatment regimen, the pain associated with the lesions remarkably improved (10/10 severity before starting treatment vs 3/10 after starting treatment). Her pain levels remained stable (3/10 severity) during 3 weeks of treatment with this combination regimen, but unfortunately she developed headaches and malaise, which she associated with the nifedipine at the 3 times daily dose.

The patient was able to better tolerate the nifedipine after reducing the dose to once daily on an as-needed basis. On average, the patient took nifedipine once every 3 days; however, she reported that she had to periodically increase the frequency of the nifedipine to once daily for up to 2 weeks at a time for periods of more frequent pain flares. The patient reported a consistent pattern of the breakthrough symptoms rapidly improving with each dose of nifedipine, though she did feel that taking consistent gabapentin enhanced baseline symptom control. The patient also noticed on a few occasions when she did not have access to her nifedipine that her pain would flare to 10/10 severity and would decrease to 4/10 severity 30 minutes after restarting nifedipine at 10 mg once daily. She experienced breakthrough pain due to exacerbating factors including her menstrual cycle; exposure to the sun and cold temperatures or water; excessive physical activity; and mild trauma. Due to exacerbations from sun exposure, the patient often wore long-sleeved shirts, which helped reduce the severity of the pain episodes while she was outdoors.

The exact mechanism for the pain associated with cutaneous leiomyomas is unknown but is thought to be due to infringement of the lesion on the surrounding cutaneous nerves. In addition, norepinephrine activates alpha receptors on the smooth muscle to contract through an influx of ions such as calcium. When smooth muscle contracts, the compression of nerves likely is worsened.

There are a limited number of case reports in the literature that have demonstrated successful treatment of the pain associated with cutaneous leiomyomas. Previously reported treatment modalities have included phenoxybenzamine, an alpha-blocking agent that may reduce pain through its antiadrenergic effects²; nitroglycerin, a venous and arterial dilator that may reduce pain by decreasing muscle oxygen requirements²; gabapentin, an antiepileptic and analgesic medication with structural similarity to the gamma-aminobutyric acid neurotransmitter for which the exact mechanism of action is unknown³; botulinum toxin, a neuromuscular blocker that prevents the release of presynaptic acetylcholine and may decrease neuropathic pain by reducing hyperactive nerves^5,6; hyoscine butylbromide and topical hyoscine hydrobromide, both antispasmodics that may reduce pain through their anticholinergic effects, which relax smooth muscle^7,8; and the CO₂ laser, a treatment that has been utilized for its resurfacing, excisional, and ablative properties.^9,10

Calcium channel blockers such as amlodipine, verapamil, and nifedipine also have been used to treat the pain associated with piloleiomyomas.¹¹ Calcium ion channel antagonists inhibit the influx of calcium ions across the cell membrane; therefore, nifedipine and other calcium channel blockers may prevent the smooth muscle contraction that is hypothesized to cause pain in patients with cutaneous leiomyomas.¹²

Mean plasma concentration of nifedipine has been shown to reach maximum values of 160 +/− 49 µg/L after 30 to 60 minutes following oral administration of 10 mg of nifedipine.¹³ After 8 hours, the mean concentration drops to 3.4 +/− 1.2 µg/L. The clinical response in our patient appeared consistent with the reported pharmacokinetics of the drug, as she was able to consistently obtain considerable reduction in her pain symptoms within 30 minutes of starting nifedipine, coinciding with the period of time it takes for the nifedipine to reach maximum plasma concentrations.¹³

Interestingly, our patient had worsening pain episodes associated with sun exposure, which typically is not reported as one of the usual triggers for cutaneous leiomyomas. We are not aware of any described mechanisms that would explain this phenomenon.

Importantly, any patient presenting with multiple cutaneous and uterine (if female) leiomyomas should be screened for hereditary leiomyomatosis and renal cell carcinoma syndrome (HLRCC), an autosomal-dominant disorder linked to a mutation in the fumarate hydratase tumor suppressor gene. Clinically, HLRCC patients typically present with multiple cutaneous leiomyomas, uterine leiomyomas, and renal cell cancer (most often type 2 papillary renal cell carcinoma).¹⁴ Hereditary leiomyomatosis and renal cell carcinoma syndrome (also known as multiple cutaneous and uterine leiomyomatosis syndrome) previously was thought to be a separate disease entity from Reed syndrome; however, after the same mutation in the fumarate hydratase tumor suppressor gene was found to be responsible for both Reed syndrome and HLRCC, they are now thought to be the same disease process.¹⁵

Diagnosis of HLRCC is likely when the patient meets the major criterion of multiple cutaneous piloleiomyomas confirmed histopathologically. Clinical diagnosis of HLRCC is suspected if 2 or more of the following minor criteria are present: type 2 papillary renal cell carcinoma before 40 years of age; onset of severely symptomatic (requiring surgery) uterine fibroids before 40 years of age in females; and first-degree family member who meets 1 or more of these criteria.¹⁵ At the time of presentation, the patient met clinical criteria for HLRCC, including multiple cutaneous leiomyomas (major criterion) and type 2 papillary renal cell carcinoma before 40 years of age (minor criterion). The patient also had a history of uterine leiomyomas, but these lesions did not fulfill the criterion of being severely symptomatic requiring surgery. Furthermore, the patient’s mother had similar cutaneous leiomyomas and a history of uterine cancer, which fulfilled additional minor criterion, consistent with an autosomal-dominant inheritance pattern (with variable penetrance) seen in HLRCC. An important issue for counseling and monitoring patients is that premenopausal women with HLRCC are at an increased risk of developing uterine leiomyosarcoma.¹⁵ Our patient followed up with an oncologist for tumor surveillance and subsequently underwent genetic testing, which revealed a mutation in the fumarate hydratase gene.

Treatment of painful cutaneous leiomyomas, particularly in patients with HLRCC, remains a therapeutic challenge. Although surgical and/or destructive treatments can provide pain relief for patients who have a limited number of lesions, these options are impracticable when a patient has numerous widespread leiomyomas; therefore, systemic therapies may be more beneficial. Clinicians should be aware of nifedipine, which may be used in combination with gabapentin as a viable treatment option in the management of acute and breakthrough pain associated with cutaneous leiomyomas.

Acknowledgment
The authors thank Alejandra Encalada, MA, for her assistance in the care and follow-up of the patient.

To the Editor:

Leiomyomas are benign smooth muscle tumors. There are 3 types of cutaneous leiomyomas: (1) piloleiomyomas, arising from the arrector pili muscles; (2) angioleiomyomas, arising from the muscles surrounding dermal blood vessels; and (3) leiomyomas of the external genitalia, arising from the dartoic, vulvar, or mammary smooth muscles.¹ There is no gender predilection for cutaneous leiomyomas, and lesions present on average at approximately 40 to 45 years of age.²

Piloleiomyomas are the most common type of cutaneous leiomyomas and typically present as red-brown papules and nodules on the trunk, arms, and legs.³ Piloleiomyomas often are associated with spontaneous or induced pain (eg, with cold exposure). The pain associated with piloleiomyomas can be severely debilitating to patients and may have a considerable impact on their quality of life.

A 40-year-old woman presented to our clinic with numerous widespread, painful, red-brown papules and nodules on the head, neck, chest, abdomen, back, arms, and legs of 6 years’ duration that were increasing in number (Figure 1). She had a history of uterine leiomyomas and type 2 renal papillary carcinoma following a left nephrectomy at 38 years of age. The patient’s mother had a history of similar skin lesions as well as uterine cancer. Multiple excisional biopsies were performed, all of which showed piloleiomyomas on histopathology (Figure 2). The pain associated with the patient’s extensive cutaneous leiomyomas considerably impaired her quality of life. Although she experienced pain in all affected areas of the body, the pain was the worst in the upper arms. She reported having requested a nerve ablation procedure from an outside pain management clinic, which was denied for unknown reasons.

Two years prior to the current presentation, the patient had been treated by a pain management specialist with gabapentin 300 mg twice daily as needed for pain associated with leiomyomas. The patient followed this regimen approximately 3 times weekly for the preceding 1 to 2 years with reduction in her pain symptoms; however, the painful episodes became more frequent and severe over time. The patient reported being unable to further increase the gabapentin dosing frequency because it made her too drowsy and impacted her ability to work a job that required heavy lifting. Thus, the patient requested additional therapy and was subsequently treated at our clinic with numerous excisional biopsies of the most painful lesions during the 2 years prior to her current presentation.

When the patient re-presented to our clinic, she requested additional lesion excisions given that she had experienced some pain relief from this treatment modality in the past; however, these prior excisions only resulted in local pain relief limited to the site of the excision. Because of the extent of the lesions and the patient’s inability to tolerate pain from the lidocaine injections, we did not feel multiple excisions were a practical treatment option. The patient subsequently was offered a trial of cryotherapy for symptom relief based on a reported case in which this modality was successfully used.⁴ After discussing the risks and benefits associated with this treatment, cryotherapy was attempted on a few of the leiomyomas on the patient’s right shoulder; however, she experienced severe pain during cryotherapy treatment, and the procedure had to be aborted.

We then increased the patient’s gabapentin regimen to 300 mg in the morning and 600 mg in the evening, as tolerated. The patient reported that she was better able to tolerate the sedating side effects of the increased dose of gabapentin because she had stopped working due to her severe pain episodes. We also added oral nifedipine 10 mg 3 times daily, as needed. Within 30 minutes of starting this treatment regimen, the pain associated with the lesions remarkably improved (10/10 severity before starting treatment vs 3/10 after starting treatment). Her pain levels remained stable (3/10 severity) during 3 weeks of treatment with this combination regimen, but unfortunately she developed headaches and malaise, which she associated with the nifedipine at the 3 times daily dose.

The patient was able to better tolerate the nifedipine after reducing the dose to once daily on an as-needed basis. On average, the patient took nifedipine once every 3 days; however, she reported that she had to periodically increase the frequency of the nifedipine to once daily for up to 2 weeks at a time for periods of more frequent pain flares. The patient reported a consistent pattern of the breakthrough symptoms rapidly improving with each dose of nifedipine, though she did feel that taking consistent gabapentin enhanced baseline symptom control. The patient also noticed on a few occasions when she did not have access to her nifedipine that her pain would flare to 10/10 severity and would decrease to 4/10 severity 30 minutes after restarting nifedipine at 10 mg once daily. She experienced breakthrough pain due to exacerbating factors including her menstrual cycle; exposure to the sun and cold temperatures or water; excessive physical activity; and mild trauma. Due to exacerbations from sun exposure, the patient often wore long-sleeved shirts, which helped reduce the severity of the pain episodes while she was outdoors.

The exact mechanism for the pain associated with cutaneous leiomyomas is unknown but is thought to be due to infringement of the lesion on the surrounding cutaneous nerves. In addition, norepinephrine activates alpha receptors on the smooth muscle to contract through an influx of ions such as calcium. When smooth muscle contracts, the compression of nerves likely is worsened.

There are a limited number of case reports in the literature that have demonstrated successful treatment of the pain associated with cutaneous leiomyomas. Previously reported treatment modalities have included phenoxybenzamine, an alpha-blocking agent that may reduce pain through its antiadrenergic effects²; nitroglycerin, a venous and arterial dilator that may reduce pain by decreasing muscle oxygen requirements²; gabapentin, an antiepileptic and analgesic medication with structural similarity to the gamma-aminobutyric acid neurotransmitter for which the exact mechanism of action is unknown³; botulinum toxin, a neuromuscular blocker that prevents the release of presynaptic acetylcholine and may decrease neuropathic pain by reducing hyperactive nerves^5,6; hyoscine butylbromide and topical hyoscine hydrobromide, both antispasmodics that may reduce pain through their anticholinergic effects, which relax smooth muscle^7,8; and the CO₂ laser, a treatment that has been utilized for its resurfacing, excisional, and ablative properties.^9,10

Calcium channel blockers such as amlodipine, verapamil, and nifedipine also have been used to treat the pain associated with piloleiomyomas.¹¹ Calcium ion channel antagonists inhibit the influx of calcium ions across the cell membrane; therefore, nifedipine and other calcium channel blockers may prevent the smooth muscle contraction that is hypothesized to cause pain in patients with cutaneous leiomyomas.¹²

Mean plasma concentration of nifedipine has been shown to reach maximum values of 160 +/− 49 µg/L after 30 to 60 minutes following oral administration of 10 mg of nifedipine.¹³ After 8 hours, the mean concentration drops to 3.4 +/− 1.2 µg/L. The clinical response in our patient appeared consistent with the reported pharmacokinetics of the drug, as she was able to consistently obtain considerable reduction in her pain symptoms within 30 minutes of starting nifedipine, coinciding with the period of time it takes for the nifedipine to reach maximum plasma concentrations.¹³

Interestingly, our patient had worsening pain episodes associated with sun exposure, which typically is not reported as one of the usual triggers for cutaneous leiomyomas. We are not aware of any described mechanisms that would explain this phenomenon.

Importantly, any patient presenting with multiple cutaneous and uterine (if female) leiomyomas should be screened for hereditary leiomyomatosis and renal cell carcinoma syndrome (HLRCC), an autosomal-dominant disorder linked to a mutation in the fumarate hydratase tumor suppressor gene. Clinically, HLRCC patients typically present with multiple cutaneous leiomyomas, uterine leiomyomas, and renal cell cancer (most often type 2 papillary renal cell carcinoma).¹⁴ Hereditary leiomyomatosis and renal cell carcinoma syndrome (also known as multiple cutaneous and uterine leiomyomatosis syndrome) previously was thought to be a separate disease entity from Reed syndrome; however, after the same mutation in the fumarate hydratase tumor suppressor gene was found to be responsible for both Reed syndrome and HLRCC, they are now thought to be the same disease process.¹⁵

Diagnosis of HLRCC is likely when the patient meets the major criterion of multiple cutaneous piloleiomyomas confirmed histopathologically. Clinical diagnosis of HLRCC is suspected if 2 or more of the following minor criteria are present: type 2 papillary renal cell carcinoma before 40 years of age; onset of severely symptomatic (requiring surgery) uterine fibroids before 40 years of age in females; and first-degree family member who meets 1 or more of these criteria.¹⁵ At the time of presentation, the patient met clinical criteria for HLRCC, including multiple cutaneous leiomyomas (major criterion) and type 2 papillary renal cell carcinoma before 40 years of age (minor criterion). The patient also had a history of uterine leiomyomas, but these lesions did not fulfill the criterion of being severely symptomatic requiring surgery. Furthermore, the patient’s mother had similar cutaneous leiomyomas and a history of uterine cancer, which fulfilled additional minor criterion, consistent with an autosomal-dominant inheritance pattern (with variable penetrance) seen in HLRCC. An important issue for counseling and monitoring patients is that premenopausal women with HLRCC are at an increased risk of developing uterine leiomyosarcoma.¹⁵ Our patient followed up with an oncologist for tumor surveillance and subsequently underwent genetic testing, which revealed a mutation in the fumarate hydratase gene.

Treatment of painful cutaneous leiomyomas, particularly in patients with HLRCC, remains a therapeutic challenge. Although surgical and/or destructive treatments can provide pain relief for patients who have a limited number of lesions, these options are impracticable when a patient has numerous widespread leiomyomas; therefore, systemic therapies may be more beneficial. Clinicians should be aware of nifedipine, which may be used in combination with gabapentin as a viable treatment option in the management of acute and breakthrough pain associated with cutaneous leiomyomas.

Acknowledgment
The authors thank Alejandra Encalada, MA, for her assistance in the care and follow-up of the patient.

To the Editor:

Leiomyomas are benign smooth muscle tumors. There are 3 types of cutaneous leiomyomas: (1) piloleiomyomas, arising from the arrector pili muscles; (2) angioleiomyomas, arising from the muscles surrounding dermal blood vessels; and (3) leiomyomas of the external genitalia, arising from the dartoic, vulvar, or mammary smooth muscles.¹ There is no gender predilection for cutaneous leiomyomas, and lesions present on average at approximately 40 to 45 years of age.²

Piloleiomyomas are the most common type of cutaneous leiomyomas and typically present as red-brown papules and nodules on the trunk, arms, and legs.³ Piloleiomyomas often are associated with spontaneous or induced pain (eg, with cold exposure). The pain associated with piloleiomyomas can be severely debilitating to patients and may have a considerable impact on their quality of life.

A 40-year-old woman presented to our clinic with numerous widespread, painful, red-brown papules and nodules on the head, neck, chest, abdomen, back, arms, and legs of 6 years’ duration that were increasing in number (Figure 1). She had a history of uterine leiomyomas and type 2 renal papillary carcinoma following a left nephrectomy at 38 years of age. The patient’s mother had a history of similar skin lesions as well as uterine cancer. Multiple excisional biopsies were performed, all of which showed piloleiomyomas on histopathology (Figure 2). The pain associated with the patient’s extensive cutaneous leiomyomas considerably impaired her quality of life. Although she experienced pain in all affected areas of the body, the pain was the worst in the upper arms. She reported having requested a nerve ablation procedure from an outside pain management clinic, which was denied for unknown reasons.

Two years prior to the current presentation, the patient had been treated by a pain management specialist with gabapentin 300 mg twice daily as needed for pain associated with leiomyomas. The patient followed this regimen approximately 3 times weekly for the preceding 1 to 2 years with reduction in her pain symptoms; however, the painful episodes became more frequent and severe over time. The patient reported being unable to further increase the gabapentin dosing frequency because it made her too drowsy and impacted her ability to work a job that required heavy lifting. Thus, the patient requested additional therapy and was subsequently treated at our clinic with numerous excisional biopsies of the most painful lesions during the 2 years prior to her current presentation.

When the patient re-presented to our clinic, she requested additional lesion excisions given that she had experienced some pain relief from this treatment modality in the past; however, these prior excisions only resulted in local pain relief limited to the site of the excision. Because of the extent of the lesions and the patient’s inability to tolerate pain from the lidocaine injections, we did not feel multiple excisions were a practical treatment option. The patient subsequently was offered a trial of cryotherapy for symptom relief based on a reported case in which this modality was successfully used.⁴ After discussing the risks and benefits associated with this treatment, cryotherapy was attempted on a few of the leiomyomas on the patient’s right shoulder; however, she experienced severe pain during cryotherapy treatment, and the procedure had to be aborted.

We then increased the patient’s gabapentin regimen to 300 mg in the morning and 600 mg in the evening, as tolerated. The patient reported that she was better able to tolerate the sedating side effects of the increased dose of gabapentin because she had stopped working due to her severe pain episodes. We also added oral nifedipine 10 mg 3 times daily, as needed. Within 30 minutes of starting this treatment regimen, the pain associated with the lesions remarkably improved (10/10 severity before starting treatment vs 3/10 after starting treatment). Her pain levels remained stable (3/10 severity) during 3 weeks of treatment with this combination regimen, but unfortunately she developed headaches and malaise, which she associated with the nifedipine at the 3 times daily dose.

The patient was able to better tolerate the nifedipine after reducing the dose to once daily on an as-needed basis. On average, the patient took nifedipine once every 3 days; however, she reported that she had to periodically increase the frequency of the nifedipine to once daily for up to 2 weeks at a time for periods of more frequent pain flares. The patient reported a consistent pattern of the breakthrough symptoms rapidly improving with each dose of nifedipine, though she did feel that taking consistent gabapentin enhanced baseline symptom control. The patient also noticed on a few occasions when she did not have access to her nifedipine that her pain would flare to 10/10 severity and would decrease to 4/10 severity 30 minutes after restarting nifedipine at 10 mg once daily. She experienced breakthrough pain due to exacerbating factors including her menstrual cycle; exposure to the sun and cold temperatures or water; excessive physical activity; and mild trauma. Due to exacerbations from sun exposure, the patient often wore long-sleeved shirts, which helped reduce the severity of the pain episodes while she was outdoors.

The exact mechanism for the pain associated with cutaneous leiomyomas is unknown but is thought to be due to infringement of the lesion on the surrounding cutaneous nerves. In addition, norepinephrine activates alpha receptors on the smooth muscle to contract through an influx of ions such as calcium. When smooth muscle contracts, the compression of nerves likely is worsened.

There are a limited number of case reports in the literature that have demonstrated successful treatment of the pain associated with cutaneous leiomyomas. Previously reported treatment modalities have included phenoxybenzamine, an alpha-blocking agent that may reduce pain through its antiadrenergic effects²; nitroglycerin, a venous and arterial dilator that may reduce pain by decreasing muscle oxygen requirements²; gabapentin, an antiepileptic and analgesic medication with structural similarity to the gamma-aminobutyric acid neurotransmitter for which the exact mechanism of action is unknown³; botulinum toxin, a neuromuscular blocker that prevents the release of presynaptic acetylcholine and may decrease neuropathic pain by reducing hyperactive nerves^5,6; hyoscine butylbromide and topical hyoscine hydrobromide, both antispasmodics that may reduce pain through their anticholinergic effects, which relax smooth muscle^7,8; and the CO₂ laser, a treatment that has been utilized for its resurfacing, excisional, and ablative properties.^9,10

Calcium channel blockers such as amlodipine, verapamil, and nifedipine also have been used to treat the pain associated with piloleiomyomas.¹¹ Calcium ion channel antagonists inhibit the influx of calcium ions across the cell membrane; therefore, nifedipine and other calcium channel blockers may prevent the smooth muscle contraction that is hypothesized to cause pain in patients with cutaneous leiomyomas.¹²

Mean plasma concentration of nifedipine has been shown to reach maximum values of 160 +/− 49 µg/L after 30 to 60 minutes following oral administration of 10 mg of nifedipine.¹³ After 8 hours, the mean concentration drops to 3.4 +/− 1.2 µg/L. The clinical response in our patient appeared consistent with the reported pharmacokinetics of the drug, as she was able to consistently obtain considerable reduction in her pain symptoms within 30 minutes of starting nifedipine, coinciding with the period of time it takes for the nifedipine to reach maximum plasma concentrations.¹³

Interestingly, our patient had worsening pain episodes associated with sun exposure, which typically is not reported as one of the usual triggers for cutaneous leiomyomas. We are not aware of any described mechanisms that would explain this phenomenon.

Importantly, any patient presenting with multiple cutaneous and uterine (if female) leiomyomas should be screened for hereditary leiomyomatosis and renal cell carcinoma syndrome (HLRCC), an autosomal-dominant disorder linked to a mutation in the fumarate hydratase tumor suppressor gene. Clinically, HLRCC patients typically present with multiple cutaneous leiomyomas, uterine leiomyomas, and renal cell cancer (most often type 2 papillary renal cell carcinoma).¹⁴ Hereditary leiomyomatosis and renal cell carcinoma syndrome (also known as multiple cutaneous and uterine leiomyomatosis syndrome) previously was thought to be a separate disease entity from Reed syndrome; however, after the same mutation in the fumarate hydratase tumor suppressor gene was found to be responsible for both Reed syndrome and HLRCC, they are now thought to be the same disease process.¹⁵

Diagnosis of HLRCC is likely when the patient meets the major criterion of multiple cutaneous piloleiomyomas confirmed histopathologically. Clinical diagnosis of HLRCC is suspected if 2 or more of the following minor criteria are present: type 2 papillary renal cell carcinoma before 40 years of age; onset of severely symptomatic (requiring surgery) uterine fibroids before 40 years of age in females; and first-degree family member who meets 1 or more of these criteria.¹⁵ At the time of presentation, the patient met clinical criteria for HLRCC, including multiple cutaneous leiomyomas (major criterion) and type 2 papillary renal cell carcinoma before 40 years of age (minor criterion). The patient also had a history of uterine leiomyomas, but these lesions did not fulfill the criterion of being severely symptomatic requiring surgery. Furthermore, the patient’s mother had similar cutaneous leiomyomas and a history of uterine cancer, which fulfilled additional minor criterion, consistent with an autosomal-dominant inheritance pattern (with variable penetrance) seen in HLRCC. An important issue for counseling and monitoring patients is that premenopausal women with HLRCC are at an increased risk of developing uterine leiomyosarcoma.¹⁵ Our patient followed up with an oncologist for tumor surveillance and subsequently underwent genetic testing, which revealed a mutation in the fumarate hydratase gene.

Treatment of painful cutaneous leiomyomas, particularly in patients with HLRCC, remains a therapeutic challenge. Although surgical and/or destructive treatments can provide pain relief for patients who have a limited number of lesions, these options are impracticable when a patient has numerous widespread leiomyomas; therefore, systemic therapies may be more beneficial. Clinicians should be aware of nifedipine, which may be used in combination with gabapentin as a viable treatment option in the management of acute and breakthrough pain associated with cutaneous leiomyomas.

Acknowledgment
The authors thank Alejandra Encalada, MA, for her assistance in the care and follow-up of the patient.