Editor’s note: The Society of Hospital Medicine’s (SHM’s) Physician in Training Committee launched a scholarship program in 2015 for medical students to help transform health care and revolutionize patient care. The program has been expanded for the 2017-18 year, offering two options for students to receive funding and engage in scholarly work during their first, second and third years of medical school. As a part of the longitudinal (18-month) program, recipients are required to write about their experience on a monthly basis.

This phase of the QI project aims at a thorough understanding of the process flow of a patient, from being transferred from outside the hospital, receiving tertiary care services at Dartmouth-Hitchcock Medical Center, to being discharged to a rehabilitation facility/skilled nursing facility.

I am conducting interviews with key stakeholders to understand the current processes and needs for improvement. The key stakeholders include but are not limited to infectious disease, hospital medicine, nursing, case management, and psychiatry services.

Yun Li is an MD/MBA student attending Geisel School of Medicine and Tuck School of Business at Dartmouth. She obtained her Bachelor of Arts degree from Hanover College double-majoring in Economics and Biological Chemistry. Ms. Li participated in research in injury epidemiology and genetics, and has conducted studies on traditional Tibetan medicine, rural health, health NGOs, and digital health. Her career interest is practicing hospital medicine and geriatrics as a clinician/administrator, either in the US or China. Ms. Li is a student member of the Society of Hospital Medicine.

Editor’s note: The Society of Hospital Medicine’s (SHM’s) Physician in Training Committee launched a scholarship program in 2015 for medical students to help transform health care and revolutionize patient care. The program has been expanded for the 2017-18 year, offering two options for students to receive funding and engage in scholarly work during their first, second and third years of medical school. As a part of the longitudinal (18-month) program, recipients are required to write about their experience on a monthly basis.

This phase of the QI project aims at a thorough understanding of the process flow of a patient, from being transferred from outside the hospital, receiving tertiary care services at Dartmouth-Hitchcock Medical Center, to being discharged to a rehabilitation facility/skilled nursing facility.

I am conducting interviews with key stakeholders to understand the current processes and needs for improvement. The key stakeholders include but are not limited to infectious disease, hospital medicine, nursing, case management, and psychiatry services.

Yun Li is an MD/MBA student attending Geisel School of Medicine and Tuck School of Business at Dartmouth. She obtained her Bachelor of Arts degree from Hanover College double-majoring in Economics and Biological Chemistry. Ms. Li participated in research in injury epidemiology and genetics, and has conducted studies on traditional Tibetan medicine, rural health, health NGOs, and digital health. Her career interest is practicing hospital medicine and geriatrics as a clinician/administrator, either in the US or China. Ms. Li is a student member of the Society of Hospital Medicine.

Editor’s note: The Society of Hospital Medicine’s (SHM’s) Physician in Training Committee launched a scholarship program in 2015 for medical students to help transform health care and revolutionize patient care. The program has been expanded for the 2017-18 year, offering two options for students to receive funding and engage in scholarly work during their first, second and third years of medical school. As a part of the longitudinal (18-month) program, recipients are required to write about their experience on a monthly basis.

This phase of the QI project aims at a thorough understanding of the process flow of a patient, from being transferred from outside the hospital, receiving tertiary care services at Dartmouth-Hitchcock Medical Center, to being discharged to a rehabilitation facility/skilled nursing facility.

I am conducting interviews with key stakeholders to understand the current processes and needs for improvement. The key stakeholders include but are not limited to infectious disease, hospital medicine, nursing, case management, and psychiatry services.

Yun Li is an MD/MBA student attending Geisel School of Medicine and Tuck School of Business at Dartmouth. She obtained her Bachelor of Arts degree from Hanover College double-majoring in Economics and Biological Chemistry. Ms. Li participated in research in injury epidemiology and genetics, and has conducted studies on traditional Tibetan medicine, rural health, health NGOs, and digital health. Her career interest is practicing hospital medicine and geriatrics as a clinician/administrator, either in the US or China. Ms. Li is a student member of the Society of Hospital Medicine.

PARIS – Maternal somatic anxiety during pregnancy and early childhood is associated in dose-response fashion with increased hyperactivity symptoms in mothers’ teenage children, according to an update from the landmark Avon Longitudinal Study of Parents and Offspring.

Perhaps the most intriguing finding in the new analysis was that the dose-response effect did not apply to the offspring of mothers in the highest quintile of somatic anxiety as measured during pregnancy and early childhood.

“It’s possible that a ceiling effect exists for those children that are exposed to continuous high levels of anxiety from pregnancy up to 5 years old,” Blanca Bolea-Alamanac, PhD, said at the annual congress of the European College of Neuropsychopharmacology.

Dr. Bolea-Alamanac of the department of psychiatry at the University of Toronto offered a couple possible explanations for this apparent ceiling effect. One is that children continuously exposed to high levels of maternal stress hormones in utero and through breast feeding become desensitized to the effects of those hormones in ways that affect their future behavior.

Another possibility is that children exposed to very high levels of anxiety in early life later develop internalizing-type symptoms rather than externalizing hyperactivity-type symptoms, she continued.

The Avon Longitudinal Study of Parents and Children is a birth cohort study conducted in Southwest England, where women who were pregnant in 1990 and their 14,062 offspring have been followed prospectively for 26 years.

Dr. Bolea-Alamanac’s analysis included the 8,725 women with maternal anxiety assessments obtained at 18 and 32 weeks of pregnancy, as well as at 8 weeks, 8 months, 2.67 years, and 5 years post partum. Maternal anxiety was measured using the Crown-Crisp Experiential Index, which provided a specific indicator of somatic anxiety via responses to questions including, “Are you troubled by dizziness or shortness of breath?” “Do you experience a tingling or prickling sensation in your body, arms, or legs?” “Have you felt that you may faint, feel sick, or have indigestion?” and “Do you experience extra sweating?” The response options to each question were “never,” “sometimes,” “often,” or “very often.”

All women understandably experienced increased anxiety during pregnancy, peaking in the weeks shortly prior to delivery. But by examining the totality of data obtained at two time points in pregnancy and four points post pregnancy, the psychiatrist was able to construct a model with five distinct quintiles of maternal anxiety.

Hyperactivity symptoms were assessed in 3,417 of their offspring using the Strengths and Difficulties Questionnaire hyperactivity subscale.

Class 1, the reference class, was comprised of women with low anxiety during pregnancy and after delivery. In a logistic regression analysis adjusted for maternal age, alcohol intake, and education, life events during pregnancy, socioeconomic status, and the child’s sex, the women in class 2 were at 1.44-fold increased risk of having a hyperactive teenager. Those who were more anxious, in class 3, were at 1.87-fold increased risk, and those in class 4 were at 2.5-fold greater risk than those in class 1. All those increased risks were statistically significant.

In contrast, women in class 5 – those who scored highest for somatic anxiety both in pregnancy and during the next 5 years – had only a nonsignificant trend toward an increased risk of having a hyperactive teenager.

The association between prenatal and postnatal maternal somatic anxiety and hyperactivity symptoms in their children seen in the Avon study is consistent with Barker’s theory, according to Dr. Bolea-Alamanac. Barker’s theory, developed by the prominent late British epidemiologist David Barker, MD, PhD, holds that intrauterine influences interact with the environment at birth to produce specific disease risks for the child. Dr. Barker’s theory has gained considerable traction over the years, as evidenced by the creation of the multidisciplinary International Society for Developmental Origins of Health and Disease.

Dr. Bolea-Alamanac reported having no relevant financial conflicts of interest.

[email protected]

PARIS – Maternal somatic anxiety during pregnancy and early childhood is associated in dose-response fashion with increased hyperactivity symptoms in mothers’ teenage children, according to an update from the landmark Avon Longitudinal Study of Parents and Offspring.

Perhaps the most intriguing finding in the new analysis was that the dose-response effect did not apply to the offspring of mothers in the highest quintile of somatic anxiety as measured during pregnancy and early childhood.

“It’s possible that a ceiling effect exists for those children that are exposed to continuous high levels of anxiety from pregnancy up to 5 years old,” Blanca Bolea-Alamanac, PhD, said at the annual congress of the European College of Neuropsychopharmacology.

Dr. Bolea-Alamanac of the department of psychiatry at the University of Toronto offered a couple possible explanations for this apparent ceiling effect. One is that children continuously exposed to high levels of maternal stress hormones in utero and through breast feeding become desensitized to the effects of those hormones in ways that affect their future behavior.

Another possibility is that children exposed to very high levels of anxiety in early life later develop internalizing-type symptoms rather than externalizing hyperactivity-type symptoms, she continued.

The Avon Longitudinal Study of Parents and Children is a birth cohort study conducted in Southwest England, where women who were pregnant in 1990 and their 14,062 offspring have been followed prospectively for 26 years.

Dr. Bolea-Alamanac’s analysis included the 8,725 women with maternal anxiety assessments obtained at 18 and 32 weeks of pregnancy, as well as at 8 weeks, 8 months, 2.67 years, and 5 years post partum. Maternal anxiety was measured using the Crown-Crisp Experiential Index, which provided a specific indicator of somatic anxiety via responses to questions including, “Are you troubled by dizziness or shortness of breath?” “Do you experience a tingling or prickling sensation in your body, arms, or legs?” “Have you felt that you may faint, feel sick, or have indigestion?” and “Do you experience extra sweating?” The response options to each question were “never,” “sometimes,” “often,” or “very often.”

All women understandably experienced increased anxiety during pregnancy, peaking in the weeks shortly prior to delivery. But by examining the totality of data obtained at two time points in pregnancy and four points post pregnancy, the psychiatrist was able to construct a model with five distinct quintiles of maternal anxiety.

Hyperactivity symptoms were assessed in 3,417 of their offspring using the Strengths and Difficulties Questionnaire hyperactivity subscale.

Class 1, the reference class, was comprised of women with low anxiety during pregnancy and after delivery. In a logistic regression analysis adjusted for maternal age, alcohol intake, and education, life events during pregnancy, socioeconomic status, and the child’s sex, the women in class 2 were at 1.44-fold increased risk of having a hyperactive teenager. Those who were more anxious, in class 3, were at 1.87-fold increased risk, and those in class 4 were at 2.5-fold greater risk than those in class 1. All those increased risks were statistically significant.

In contrast, women in class 5 – those who scored highest for somatic anxiety both in pregnancy and during the next 5 years – had only a nonsignificant trend toward an increased risk of having a hyperactive teenager.

The association between prenatal and postnatal maternal somatic anxiety and hyperactivity symptoms in their children seen in the Avon study is consistent with Barker’s theory, according to Dr. Bolea-Alamanac. Barker’s theory, developed by the prominent late British epidemiologist David Barker, MD, PhD, holds that intrauterine influences interact with the environment at birth to produce specific disease risks for the child. Dr. Barker’s theory has gained considerable traction over the years, as evidenced by the creation of the multidisciplinary International Society for Developmental Origins of Health and Disease.

Dr. Bolea-Alamanac reported having no relevant financial conflicts of interest.

[email protected]

PARIS – Maternal somatic anxiety during pregnancy and early childhood is associated in dose-response fashion with increased hyperactivity symptoms in mothers’ teenage children, according to an update from the landmark Avon Longitudinal Study of Parents and Offspring.

Perhaps the most intriguing finding in the new analysis was that the dose-response effect did not apply to the offspring of mothers in the highest quintile of somatic anxiety as measured during pregnancy and early childhood.

“It’s possible that a ceiling effect exists for those children that are exposed to continuous high levels of anxiety from pregnancy up to 5 years old,” Blanca Bolea-Alamanac, PhD, said at the annual congress of the European College of Neuropsychopharmacology.

Dr. Bolea-Alamanac of the department of psychiatry at the University of Toronto offered a couple possible explanations for this apparent ceiling effect. One is that children continuously exposed to high levels of maternal stress hormones in utero and through breast feeding become desensitized to the effects of those hormones in ways that affect their future behavior.

Another possibility is that children exposed to very high levels of anxiety in early life later develop internalizing-type symptoms rather than externalizing hyperactivity-type symptoms, she continued.

The Avon Longitudinal Study of Parents and Children is a birth cohort study conducted in Southwest England, where women who were pregnant in 1990 and their 14,062 offspring have been followed prospectively for 26 years.

Dr. Bolea-Alamanac’s analysis included the 8,725 women with maternal anxiety assessments obtained at 18 and 32 weeks of pregnancy, as well as at 8 weeks, 8 months, 2.67 years, and 5 years post partum. Maternal anxiety was measured using the Crown-Crisp Experiential Index, which provided a specific indicator of somatic anxiety via responses to questions including, “Are you troubled by dizziness or shortness of breath?” “Do you experience a tingling or prickling sensation in your body, arms, or legs?” “Have you felt that you may faint, feel sick, or have indigestion?” and “Do you experience extra sweating?” The response options to each question were “never,” “sometimes,” “often,” or “very often.”

All women understandably experienced increased anxiety during pregnancy, peaking in the weeks shortly prior to delivery. But by examining the totality of data obtained at two time points in pregnancy and four points post pregnancy, the psychiatrist was able to construct a model with five distinct quintiles of maternal anxiety.

Hyperactivity symptoms were assessed in 3,417 of their offspring using the Strengths and Difficulties Questionnaire hyperactivity subscale.

Class 1, the reference class, was comprised of women with low anxiety during pregnancy and after delivery. In a logistic regression analysis adjusted for maternal age, alcohol intake, and education, life events during pregnancy, socioeconomic status, and the child’s sex, the women in class 2 were at 1.44-fold increased risk of having a hyperactive teenager. Those who were more anxious, in class 3, were at 1.87-fold increased risk, and those in class 4 were at 2.5-fold greater risk than those in class 1. All those increased risks were statistically significant.

In contrast, women in class 5 – those who scored highest for somatic anxiety both in pregnancy and during the next 5 years – had only a nonsignificant trend toward an increased risk of having a hyperactive teenager.

The association between prenatal and postnatal maternal somatic anxiety and hyperactivity symptoms in their children seen in the Avon study is consistent with Barker’s theory, according to Dr. Bolea-Alamanac. Barker’s theory, developed by the prominent late British epidemiologist David Barker, MD, PhD, holds that intrauterine influences interact with the environment at birth to produce specific disease risks for the child. Dr. Barker’s theory has gained considerable traction over the years, as evidenced by the creation of the multidisciplinary International Society for Developmental Origins of Health and Disease.

Dr. Bolea-Alamanac reported having no relevant financial conflicts of interest.

[email protected]

Obsessive-compulsive disorder could be detrimental to educational attainment, particularly if diagnosed before the age of 18 years, according to a study published online Nov. 15.

A Swedish population-based birth cohort study identified 15,120 individuals who had been diagnosed with obsessive-compulsive disorder (OCD, 11,482 of whom were in families with full siblings discordant for OCD.

wildpixel/Thinkstock

Obsessive-compulsive disorder could be detrimental to educational attainment, particularly if diagnosed before the age of 18 years, according to a study published online Nov. 15.

A Swedish population-based birth cohort study identified 15,120 individuals who had been diagnosed with obsessive-compulsive disorder (OCD, 11,482 of whom were in families with full siblings discordant for OCD.

wildpixel/Thinkstock

Obsessive-compulsive disorder could be detrimental to educational attainment, particularly if diagnosed before the age of 18 years, according to a study published online Nov. 15.

A Swedish population-based birth cohort study identified 15,120 individuals who had been diagnosed with obsessive-compulsive disorder (OCD, 11,482 of whom were in families with full siblings discordant for OCD.

wildpixel/Thinkstock

Myth: Hypertrophic actinic keratoses are more likely to progress to squamous cell carcinoma

Actinic keratosis (AK) indicates cumulative UV exposure and is the initial lesion in the majority of invasive cutaneous squamous cell carcinomas (SCCs). However, most AKs do not progress to invasive SCC and it currently is not possible to clinically or histopathologically determine which AK lesions will progress to SCC.

The rates of progression of individual AK lesions to SCC vary. In 2011, Feldman and Fleischer summarized 6 studies of 560 to 6691 patients with AK. The AK progression to SCC was found to range from 0.075% per year per lesion to 14% over 5 years.

Criscione et al found that the risk of progression of AK to primary SCC was 0.60% at 1 year and 2.57% at 4 years. In this study, 187 primary SCCs were diagnosed after enrollment, with 65% arising in previously clinically diagnosed and documented AKs. Therefore, although the authors noted low risks of progression of AK to SCC, they observed that the majority of SCCs arose from AKs.

The risk for progression of AK to invasive SCC with the potential for metastasis warrants treatment of AK with lesion- or field-directed therapy or a combined approach when indicated. Dermatologists also should monitor AK patients closely.

Expert Commentary

It’s a myth that hypertrophic lesions are more likely to turn into SCC. In fact, it’s the lesions with follicular extension that are more likely to progress to SCC. These may be hypertrophic or atrophic or simple AKs. The genetics of AKs that become hypertrophic may be different from those that become invasive. These lesions may be more likely to first grow outward before becoming invasive. 

—Gary Goldenberg (New York, New York)

Myth: Hypertrophic actinic keratoses are more likely to progress to squamous cell carcinoma

Actinic keratosis (AK) indicates cumulative UV exposure and is the initial lesion in the majority of invasive cutaneous squamous cell carcinomas (SCCs). However, most AKs do not progress to invasive SCC and it currently is not possible to clinically or histopathologically determine which AK lesions will progress to SCC.

The rates of progression of individual AK lesions to SCC vary. In 2011, Feldman and Fleischer summarized 6 studies of 560 to 6691 patients with AK. The AK progression to SCC was found to range from 0.075% per year per lesion to 14% over 5 years.

Criscione et al found that the risk of progression of AK to primary SCC was 0.60% at 1 year and 2.57% at 4 years. In this study, 187 primary SCCs were diagnosed after enrollment, with 65% arising in previously clinically diagnosed and documented AKs. Therefore, although the authors noted low risks of progression of AK to SCC, they observed that the majority of SCCs arose from AKs.

The risk for progression of AK to invasive SCC with the potential for metastasis warrants treatment of AK with lesion- or field-directed therapy or a combined approach when indicated. Dermatologists also should monitor AK patients closely.

Expert Commentary

It’s a myth that hypertrophic lesions are more likely to turn into SCC. In fact, it’s the lesions with follicular extension that are more likely to progress to SCC. These may be hypertrophic or atrophic or simple AKs. The genetics of AKs that become hypertrophic may be different from those that become invasive. These lesions may be more likely to first grow outward before becoming invasive. 

—Gary Goldenberg (New York, New York)

Myth: Hypertrophic actinic keratoses are more likely to progress to squamous cell carcinoma

Actinic keratosis (AK) indicates cumulative UV exposure and is the initial lesion in the majority of invasive cutaneous squamous cell carcinomas (SCCs). However, most AKs do not progress to invasive SCC and it currently is not possible to clinically or histopathologically determine which AK lesions will progress to SCC.

The rates of progression of individual AK lesions to SCC vary. In 2011, Feldman and Fleischer summarized 6 studies of 560 to 6691 patients with AK. The AK progression to SCC was found to range from 0.075% per year per lesion to 14% over 5 years.

Criscione et al found that the risk of progression of AK to primary SCC was 0.60% at 1 year and 2.57% at 4 years. In this study, 187 primary SCCs were diagnosed after enrollment, with 65% arising in previously clinically diagnosed and documented AKs. Therefore, although the authors noted low risks of progression of AK to SCC, they observed that the majority of SCCs arose from AKs.

The risk for progression of AK to invasive SCC with the potential for metastasis warrants treatment of AK with lesion- or field-directed therapy or a combined approach when indicated. Dermatologists also should monitor AK patients closely.

Expert Commentary

It’s a myth that hypertrophic lesions are more likely to turn into SCC. In fact, it’s the lesions with follicular extension that are more likely to progress to SCC. These may be hypertrophic or atrophic or simple AKs. The genetics of AKs that become hypertrophic may be different from those that become invasive. These lesions may be more likely to first grow outward before becoming invasive. 

—Gary Goldenberg (New York, New York)

As forensic psychiatrists, one of our main roles is to apply the Dusky standard to assess competency. In this regard, multiple times a week, we see pretrial defendants who wait weeks, sometimes months, in jail, for their competency evaluations. Will they be permitted to attend court and continue with their legal proceedings, or will a judge remand them into an involuntary treatment unit to restore their competency? The number of defendants referred for competency evaluation is formally not measured, but estimates suggest it almost doubled from 1973¹ till 2000.²

The intent of ensuring the competency of the accused is fundamentally fair. While all would agree that only those who are convicted of committing crimes be found guilty, not every culture has paid attention to the question of whether those found guilty understand how and why that happened.

Defendant’s background

The defendant is a 40-something-year-old man who vacillates between homelessness and living with friends who partake in his penchant for alcohol. He has committed various crimes, including thefts, disorderly conduct, and possession of controlled substances. He went to prison once for selling narcotics but quickly retorts: “I don’t sell … . This [expletive] cop came and asked if I had any. She was hot. What did you want me to say? It was entrapment.”

Challenging behavior continues

During the course of the interview, he was able to demonstrate that he understands the meaning of making a threat, of committing a crime, and of the roles of the different courtroom personnel. However, the stress of court highlights his interpersonal problems. In this particular case, he recounts: “Court had not yet started; I was talking to my lawyer, and the judge interrupted me, so I answered: Wait your turn [expletive] … not my greatest idea.” When asked about his past referral for competency, he mentions it was in response to trying to fire a public defender because “she was Mexican. I don’t work with those.”

Given his behavior, it is unclear how else a judge or a lawyer could have acted. One could argue that it would be a mistake not to refer this defendant for a competency evaluation, considering his outbursts. On the other hand, he had been evaluated many times before, and the opinion of well-respected forensic psychiatrists was that he did not have a mental illness.

While we reflect on our experience with this defendant, we are unsure of the lessons to be learned. We ponder whether psychiatry does a disservice when not being clearer about what constitutes a serious mental illness. We wonder if we exacerbated the confusion by the removal of “Axis II” categories from the DSM, implying that severe personality disorders are no longer different from, say, schizophrenia and bipolar disorder. Rarely do we hear psychiatrists point out that unusual behaviors do not equal mental illness. We are often too pleased in advocating for more resources by saying that all crimes, all substance misuses, and all annoying behaviors are forms of mental illness when, in reality, the criminal4, the addictive5, and the less common6 are not always biologically based mental disorders or even the real problem, for that matter.

This defendant is difficult. He argues, he yells, he provokes, and he hurts others physically as well as emotionally. While many psychiatrists have decided to codify this pattern of behavior within the B cluster of personality traits, have we misled the public into thinking that patients with personality disorders require the same attention and care as patients with other forms of mental illness, like schizophrenia? Often, we see patients with schizophrenia, bipolar depression, or major depression, who even at their best, are too impaired to file their taxes, apply for an identity card, or understand the complexity of the legal system.

Psychiatry’s difficulty in verbalizing the difference between those disorders harms the public perception of mental disorders. As a result, we have a forensic system similar to the rest of the community health care system – with an abundance of individuals with severe mental illness not referred for treatment or evaluation, and several patients with personality disorders bogging down a system with very limited resources. It is our responsibility not only to educate the public on how to manage and contain the emotions that patients with personality disorders engender in us, but also to educate the public on how to recognize patients with profound mentally ill patients who are quietly suffering.

Dr. Badre is affiliated with the county of San Diego, the University of California at San Diego, and the University of San Diego. Dr. Rao is a San Diego–based board-certified psychiatrist with expertise in forensic psychiatry, correctional psychiatry, telepsychiatry, and inpatient psychiatry.
 

References

1. Competency to Stand Trial and Mental Illness: Final Report. Rockville, Md.: National Institute of Mental Health, 1973.

2. Youth on Trial: A Developmental Perspective on Juvenile Justice. Chicago: University of Chicago Press, 2000.

3. J Am Acad Psychiatry Law. 2007;35(4 Suppl):S3-72.

4. Clin Psychiatry News. 2017;45(8):5.

5. Law and Philosophy. 1999;18(6):589-610.

6. Am J Psychiatry. 1981 Feb;138(2):210-5.

As forensic psychiatrists, one of our main roles is to apply the Dusky standard to assess competency. In this regard, multiple times a week, we see pretrial defendants who wait weeks, sometimes months, in jail, for their competency evaluations. Will they be permitted to attend court and continue with their legal proceedings, or will a judge remand them into an involuntary treatment unit to restore their competency? The number of defendants referred for competency evaluation is formally not measured, but estimates suggest it almost doubled from 1973¹ till 2000.²

The intent of ensuring the competency of the accused is fundamentally fair. While all would agree that only those who are convicted of committing crimes be found guilty, not every culture has paid attention to the question of whether those found guilty understand how and why that happened.

Defendant’s background

The defendant is a 40-something-year-old man who vacillates between homelessness and living with friends who partake in his penchant for alcohol. He has committed various crimes, including thefts, disorderly conduct, and possession of controlled substances. He went to prison once for selling narcotics but quickly retorts: “I don’t sell … . This [expletive] cop came and asked if I had any. She was hot. What did you want me to say? It was entrapment.”

Challenging behavior continues

During the course of the interview, he was able to demonstrate that he understands the meaning of making a threat, of committing a crime, and of the roles of the different courtroom personnel. However, the stress of court highlights his interpersonal problems. In this particular case, he recounts: “Court had not yet started; I was talking to my lawyer, and the judge interrupted me, so I answered: Wait your turn [expletive] … not my greatest idea.” When asked about his past referral for competency, he mentions it was in response to trying to fire a public defender because “she was Mexican. I don’t work with those.”

Given his behavior, it is unclear how else a judge or a lawyer could have acted. One could argue that it would be a mistake not to refer this defendant for a competency evaluation, considering his outbursts. On the other hand, he had been evaluated many times before, and the opinion of well-respected forensic psychiatrists was that he did not have a mental illness.

While we reflect on our experience with this defendant, we are unsure of the lessons to be learned. We ponder whether psychiatry does a disservice when not being clearer about what constitutes a serious mental illness. We wonder if we exacerbated the confusion by the removal of “Axis II” categories from the DSM, implying that severe personality disorders are no longer different from, say, schizophrenia and bipolar disorder. Rarely do we hear psychiatrists point out that unusual behaviors do not equal mental illness. We are often too pleased in advocating for more resources by saying that all crimes, all substance misuses, and all annoying behaviors are forms of mental illness when, in reality, the criminal4, the addictive5, and the less common6 are not always biologically based mental disorders or even the real problem, for that matter.

This defendant is difficult. He argues, he yells, he provokes, and he hurts others physically as well as emotionally. While many psychiatrists have decided to codify this pattern of behavior within the B cluster of personality traits, have we misled the public into thinking that patients with personality disorders require the same attention and care as patients with other forms of mental illness, like schizophrenia? Often, we see patients with schizophrenia, bipolar depression, or major depression, who even at their best, are too impaired to file their taxes, apply for an identity card, or understand the complexity of the legal system.

Psychiatry’s difficulty in verbalizing the difference between those disorders harms the public perception of mental disorders. As a result, we have a forensic system similar to the rest of the community health care system – with an abundance of individuals with severe mental illness not referred for treatment or evaluation, and several patients with personality disorders bogging down a system with very limited resources. It is our responsibility not only to educate the public on how to manage and contain the emotions that patients with personality disorders engender in us, but also to educate the public on how to recognize patients with profound mentally ill patients who are quietly suffering.

Dr. Badre is affiliated with the county of San Diego, the University of California at San Diego, and the University of San Diego. Dr. Rao is a San Diego–based board-certified psychiatrist with expertise in forensic psychiatry, correctional psychiatry, telepsychiatry, and inpatient psychiatry.
 

References

1. Competency to Stand Trial and Mental Illness: Final Report. Rockville, Md.: National Institute of Mental Health, 1973.

2. Youth on Trial: A Developmental Perspective on Juvenile Justice. Chicago: University of Chicago Press, 2000.

3. J Am Acad Psychiatry Law. 2007;35(4 Suppl):S3-72.

4. Clin Psychiatry News. 2017;45(8):5.

5. Law and Philosophy. 1999;18(6):589-610.

6. Am J Psychiatry. 1981 Feb;138(2):210-5.

As forensic psychiatrists, one of our main roles is to apply the Dusky standard to assess competency. In this regard, multiple times a week, we see pretrial defendants who wait weeks, sometimes months, in jail, for their competency evaluations. Will they be permitted to attend court and continue with their legal proceedings, or will a judge remand them into an involuntary treatment unit to restore their competency? The number of defendants referred for competency evaluation is formally not measured, but estimates suggest it almost doubled from 1973¹ till 2000.²

The intent of ensuring the competency of the accused is fundamentally fair. While all would agree that only those who are convicted of committing crimes be found guilty, not every culture has paid attention to the question of whether those found guilty understand how and why that happened.

Defendant’s background

The defendant is a 40-something-year-old man who vacillates between homelessness and living with friends who partake in his penchant for alcohol. He has committed various crimes, including thefts, disorderly conduct, and possession of controlled substances. He went to prison once for selling narcotics but quickly retorts: “I don’t sell … . This [expletive] cop came and asked if I had any. She was hot. What did you want me to say? It was entrapment.”

Challenging behavior continues

During the course of the interview, he was able to demonstrate that he understands the meaning of making a threat, of committing a crime, and of the roles of the different courtroom personnel. However, the stress of court highlights his interpersonal problems. In this particular case, he recounts: “Court had not yet started; I was talking to my lawyer, and the judge interrupted me, so I answered: Wait your turn [expletive] … not my greatest idea.” When asked about his past referral for competency, he mentions it was in response to trying to fire a public defender because “she was Mexican. I don’t work with those.”

Given his behavior, it is unclear how else a judge or a lawyer could have acted. One could argue that it would be a mistake not to refer this defendant for a competency evaluation, considering his outbursts. On the other hand, he had been evaluated many times before, and the opinion of well-respected forensic psychiatrists was that he did not have a mental illness.

While we reflect on our experience with this defendant, we are unsure of the lessons to be learned. We ponder whether psychiatry does a disservice when not being clearer about what constitutes a serious mental illness. We wonder if we exacerbated the confusion by the removal of “Axis II” categories from the DSM, implying that severe personality disorders are no longer different from, say, schizophrenia and bipolar disorder. Rarely do we hear psychiatrists point out that unusual behaviors do not equal mental illness. We are often too pleased in advocating for more resources by saying that all crimes, all substance misuses, and all annoying behaviors are forms of mental illness when, in reality, the criminal4, the addictive5, and the less common6 are not always biologically based mental disorders or even the real problem, for that matter.

This defendant is difficult. He argues, he yells, he provokes, and he hurts others physically as well as emotionally. While many psychiatrists have decided to codify this pattern of behavior within the B cluster of personality traits, have we misled the public into thinking that patients with personality disorders require the same attention and care as patients with other forms of mental illness, like schizophrenia? Often, we see patients with schizophrenia, bipolar depression, or major depression, who even at their best, are too impaired to file their taxes, apply for an identity card, or understand the complexity of the legal system.

Psychiatry’s difficulty in verbalizing the difference between those disorders harms the public perception of mental disorders. As a result, we have a forensic system similar to the rest of the community health care system – with an abundance of individuals with severe mental illness not referred for treatment or evaluation, and several patients with personality disorders bogging down a system with very limited resources. It is our responsibility not only to educate the public on how to manage and contain the emotions that patients with personality disorders engender in us, but also to educate the public on how to recognize patients with profound mentally ill patients who are quietly suffering.

Dr. Badre is affiliated with the county of San Diego, the University of California at San Diego, and the University of San Diego. Dr. Rao is a San Diego–based board-certified psychiatrist with expertise in forensic psychiatry, correctional psychiatry, telepsychiatry, and inpatient psychiatry.
 

References

1. Competency to Stand Trial and Mental Illness: Final Report. Rockville, Md.: National Institute of Mental Health, 1973.

2. Youth on Trial: A Developmental Perspective on Juvenile Justice. Chicago: University of Chicago Press, 2000.

3. J Am Acad Psychiatry Law. 2007;35(4 Suppl):S3-72.

4. Clin Psychiatry News. 2017;45(8):5.

5. Law and Philosophy. 1999;18(6):589-610.

6. Am J Psychiatry. 1981 Feb;138(2):210-5.

NATIONAL HARBOR, MD. – Elagolix, an oral gonadotropin-releasing hormone antagonist, improved dysmenorrhea and nonmenstrual pelvic pain for a year or more in women with surgically-diagnosed endometriosis in two extension studies.

Women who had participated in two pivotal, 6-month studies of elagolix were given the option to continue in an extension trial for another 6 months, Sukhbir S. Singh, MD, said at the AAGL Global Congress. He noted that “all my patients who completed the 6-month trial continued out to a year.”

Patients tracked their dysmenorrhea and nonmenstrual pelvic pain scores on a 4-point scale daily using an electronic pain impact diary. They were classified as being responders if they experienced reduced dysmenorrhea and nonmenstrual pelvic pain equal to or better than they had during the original trial (much or very much improved on the Patient Global Impression of Change scale).

For each dose of elagolix, rates of response seen at 6 months in the original trial were maintained at a year or longer of continuous treatment, demonstrating long-term efficacy, Dr. Singh said. The average number of analgesic pills taken per month decreased 46%-77% from baseline for all doses in the extension studies.

The most common adverse events were hot flushes, experienced by just over half (52%-55%) of women in the high-dose group and about 25%-30% of women in the lower-dose group. Severity was mild to moderate across the studies.

“The higher the dose you give, the more hot flushes you get,” said Dr. Singh, vice chair of gynecology at the University of Ottawa, Ontario. “But overall, patients often did not complain of this because they were getting pain relief as well.”

Other adverse events included headache in about 25%-30% of high-dose patients and 20% of low-dose patients, as well as nausea in about one-fifth of patients overall. Some decreases from baseline in bone mineral density were seen but there was progressive improvement upon discontinuation of elagolix, Dr. Singh noted.

Importantly, “these were patients who had endometriosis and pain, similar in makeup to groups studied by others. These studies did provide two dosing options to offer individualized approaches and pain results were controlled for use of rescue analgesia. But as extension trials, the studies are limited by having no placebo control and we did not know whether they had deep endometriosis or other pain issues,” Dr. Singh said.

Elagolix represents another treatment option for patients with endometriosis, Dr. Singh said. “One of the objections to taking currently approved [gonadotropin-releasing hormone] antagonists is that they are subcutaneous injections. Patients don’t like that – this is an oral option. It is quick to act and is rapidly reversible as well.”

Dr. Singh serves as a principal investigator and speaker for Allergan, AbbVie, and Bayer. The studies were sponsored by AbbVie.
 

[email protected]

On Twitter @denisefulton

NATIONAL HARBOR, MD. – Elagolix, an oral gonadotropin-releasing hormone antagonist, improved dysmenorrhea and nonmenstrual pelvic pain for a year or more in women with surgically-diagnosed endometriosis in two extension studies.

Women who had participated in two pivotal, 6-month studies of elagolix were given the option to continue in an extension trial for another 6 months, Sukhbir S. Singh, MD, said at the AAGL Global Congress. He noted that “all my patients who completed the 6-month trial continued out to a year.”

Patients tracked their dysmenorrhea and nonmenstrual pelvic pain scores on a 4-point scale daily using an electronic pain impact diary. They were classified as being responders if they experienced reduced dysmenorrhea and nonmenstrual pelvic pain equal to or better than they had during the original trial (much or very much improved on the Patient Global Impression of Change scale).

For each dose of elagolix, rates of response seen at 6 months in the original trial were maintained at a year or longer of continuous treatment, demonstrating long-term efficacy, Dr. Singh said. The average number of analgesic pills taken per month decreased 46%-77% from baseline for all doses in the extension studies.

The most common adverse events were hot flushes, experienced by just over half (52%-55%) of women in the high-dose group and about 25%-30% of women in the lower-dose group. Severity was mild to moderate across the studies.

“The higher the dose you give, the more hot flushes you get,” said Dr. Singh, vice chair of gynecology at the University of Ottawa, Ontario. “But overall, patients often did not complain of this because they were getting pain relief as well.”

Other adverse events included headache in about 25%-30% of high-dose patients and 20% of low-dose patients, as well as nausea in about one-fifth of patients overall. Some decreases from baseline in bone mineral density were seen but there was progressive improvement upon discontinuation of elagolix, Dr. Singh noted.

Importantly, “these were patients who had endometriosis and pain, similar in makeup to groups studied by others. These studies did provide two dosing options to offer individualized approaches and pain results were controlled for use of rescue analgesia. But as extension trials, the studies are limited by having no placebo control and we did not know whether they had deep endometriosis or other pain issues,” Dr. Singh said.

Elagolix represents another treatment option for patients with endometriosis, Dr. Singh said. “One of the objections to taking currently approved [gonadotropin-releasing hormone] antagonists is that they are subcutaneous injections. Patients don’t like that – this is an oral option. It is quick to act and is rapidly reversible as well.”

Dr. Singh serves as a principal investigator and speaker for Allergan, AbbVie, and Bayer. The studies were sponsored by AbbVie.
 

[email protected]

On Twitter @denisefulton

NATIONAL HARBOR, MD. – Elagolix, an oral gonadotropin-releasing hormone antagonist, improved dysmenorrhea and nonmenstrual pelvic pain for a year or more in women with surgically-diagnosed endometriosis in two extension studies.

Women who had participated in two pivotal, 6-month studies of elagolix were given the option to continue in an extension trial for another 6 months, Sukhbir S. Singh, MD, said at the AAGL Global Congress. He noted that “all my patients who completed the 6-month trial continued out to a year.”

Patients tracked their dysmenorrhea and nonmenstrual pelvic pain scores on a 4-point scale daily using an electronic pain impact diary. They were classified as being responders if they experienced reduced dysmenorrhea and nonmenstrual pelvic pain equal to or better than they had during the original trial (much or very much improved on the Patient Global Impression of Change scale).

For each dose of elagolix, rates of response seen at 6 months in the original trial were maintained at a year or longer of continuous treatment, demonstrating long-term efficacy, Dr. Singh said. The average number of analgesic pills taken per month decreased 46%-77% from baseline for all doses in the extension studies.

The most common adverse events were hot flushes, experienced by just over half (52%-55%) of women in the high-dose group and about 25%-30% of women in the lower-dose group. Severity was mild to moderate across the studies.

“The higher the dose you give, the more hot flushes you get,” said Dr. Singh, vice chair of gynecology at the University of Ottawa, Ontario. “But overall, patients often did not complain of this because they were getting pain relief as well.”

Other adverse events included headache in about 25%-30% of high-dose patients and 20% of low-dose patients, as well as nausea in about one-fifth of patients overall. Some decreases from baseline in bone mineral density were seen but there was progressive improvement upon discontinuation of elagolix, Dr. Singh noted.

Importantly, “these were patients who had endometriosis and pain, similar in makeup to groups studied by others. These studies did provide two dosing options to offer individualized approaches and pain results were controlled for use of rescue analgesia. But as extension trials, the studies are limited by having no placebo control and we did not know whether they had deep endometriosis or other pain issues,” Dr. Singh said.

Elagolix represents another treatment option for patients with endometriosis, Dr. Singh said. “One of the objections to taking currently approved [gonadotropin-releasing hormone] antagonists is that they are subcutaneous injections. Patients don’t like that – this is an oral option. It is quick to act and is rapidly reversible as well.”

Dr. Singh serves as a principal investigator and speaker for Allergan, AbbVie, and Bayer. The studies were sponsored by AbbVie.
 

[email protected]

On Twitter @denisefulton

NATIONAL HARBOR, MD. –Genetic testing could provide a useful noninvasive diagnostic tool in identifying patients with endometriosis, according to a study conducted by Nick Fogelson, MD, of Pearl Women’s Center in Portland, Ore., and his colleagues.

Dr. Fogelson presented the findings from a blinded, randomized pilot study at the AAGL Global Congress. The study included two groups of 200 women each. The first group of women had previously been diagnosed with endometriosis. The second group comprised women with no evidence of endometriosis. In the group with endometriosis, the test correctly identified endometriosis in 189 of the 200 women (95%). The women with no evidence of endometriosis were accurately identified as having a low risk of developing endometriosis in 176 of 200 women (88%).

The samples were collected from around the United States as part of ongoing research by the Utah-based genetics company Juneau Biosciences.

Both groups were genotyped for 1,067 low-frequency DNA variants associated with endometriosis using a proprietary algorithm. The researchers then compared genotype results with a large dataset of 1,000 genotyped endometriosis patients and 33,000 published controls and assessed patient risk of developing endometriosis by weighting each genotype by the logarithm of the odds ratio.

“We’re getting toward a time when you will be able to tell if someone has endometriosis by looking at their genetics,” Dr. Fogelson said.

The genetic analysis hold potential in a disease state where misdiagnosis by nonexpert physicians can be high. Dr. Fogelson estimated that the misdiagnosis rate in endometriosis based on physical exam and patient history alone is about 50%. Compounding this issue, many insurers have reduced payment for diagnostic laparoscopy leading to surgeons’ placing patients on long-term medication treatments when they would benefit from surgery, Dr. Fogelson said.

“Noninvasive DNA testing may help to direct symptomatic patients to specialists who can effectively treat the disease state.” Dr. Fogelson and his colleagues wrote in the study abstract. “DNA markers might have better correlation to the subtypes and extent of disease than histology alone.”

Additional trials are currently underway using the genetic marker test; one is a prospective study and is expected to be completed in early 2018.

Dr. Fogelson reported having no conflicts of interest. Other researchers on the study work for Juneau Biosciences and receive stock options as part of their compensation.

[email protected]

NATIONAL HARBOR, MD. –Genetic testing could provide a useful noninvasive diagnostic tool in identifying patients with endometriosis, according to a study conducted by Nick Fogelson, MD, of Pearl Women’s Center in Portland, Ore., and his colleagues.

Dr. Fogelson presented the findings from a blinded, randomized pilot study at the AAGL Global Congress. The study included two groups of 200 women each. The first group of women had previously been diagnosed with endometriosis. The second group comprised women with no evidence of endometriosis. In the group with endometriosis, the test correctly identified endometriosis in 189 of the 200 women (95%). The women with no evidence of endometriosis were accurately identified as having a low risk of developing endometriosis in 176 of 200 women (88%).

The samples were collected from around the United States as part of ongoing research by the Utah-based genetics company Juneau Biosciences.

Both groups were genotyped for 1,067 low-frequency DNA variants associated with endometriosis using a proprietary algorithm. The researchers then compared genotype results with a large dataset of 1,000 genotyped endometriosis patients and 33,000 published controls and assessed patient risk of developing endometriosis by weighting each genotype by the logarithm of the odds ratio.

“We’re getting toward a time when you will be able to tell if someone has endometriosis by looking at their genetics,” Dr. Fogelson said.

The genetic analysis hold potential in a disease state where misdiagnosis by nonexpert physicians can be high. Dr. Fogelson estimated that the misdiagnosis rate in endometriosis based on physical exam and patient history alone is about 50%. Compounding this issue, many insurers have reduced payment for diagnostic laparoscopy leading to surgeons’ placing patients on long-term medication treatments when they would benefit from surgery, Dr. Fogelson said.

“Noninvasive DNA testing may help to direct symptomatic patients to specialists who can effectively treat the disease state.” Dr. Fogelson and his colleagues wrote in the study abstract. “DNA markers might have better correlation to the subtypes and extent of disease than histology alone.”

Additional trials are currently underway using the genetic marker test; one is a prospective study and is expected to be completed in early 2018.

Dr. Fogelson reported having no conflicts of interest. Other researchers on the study work for Juneau Biosciences and receive stock options as part of their compensation.

[email protected]

NATIONAL HARBOR, MD. –Genetic testing could provide a useful noninvasive diagnostic tool in identifying patients with endometriosis, according to a study conducted by Nick Fogelson, MD, of Pearl Women’s Center in Portland, Ore., and his colleagues.

Dr. Fogelson presented the findings from a blinded, randomized pilot study at the AAGL Global Congress. The study included two groups of 200 women each. The first group of women had previously been diagnosed with endometriosis. The second group comprised women with no evidence of endometriosis. In the group with endometriosis, the test correctly identified endometriosis in 189 of the 200 women (95%). The women with no evidence of endometriosis were accurately identified as having a low risk of developing endometriosis in 176 of 200 women (88%).

The samples were collected from around the United States as part of ongoing research by the Utah-based genetics company Juneau Biosciences.

Both groups were genotyped for 1,067 low-frequency DNA variants associated with endometriosis using a proprietary algorithm. The researchers then compared genotype results with a large dataset of 1,000 genotyped endometriosis patients and 33,000 published controls and assessed patient risk of developing endometriosis by weighting each genotype by the logarithm of the odds ratio.

“We’re getting toward a time when you will be able to tell if someone has endometriosis by looking at their genetics,” Dr. Fogelson said.

The genetic analysis hold potential in a disease state where misdiagnosis by nonexpert physicians can be high. Dr. Fogelson estimated that the misdiagnosis rate in endometriosis based on physical exam and patient history alone is about 50%. Compounding this issue, many insurers have reduced payment for diagnostic laparoscopy leading to surgeons’ placing patients on long-term medication treatments when they would benefit from surgery, Dr. Fogelson said.

“Noninvasive DNA testing may help to direct symptomatic patients to specialists who can effectively treat the disease state.” Dr. Fogelson and his colleagues wrote in the study abstract. “DNA markers might have better correlation to the subtypes and extent of disease than histology alone.”

Additional trials are currently underway using the genetic marker test; one is a prospective study and is expected to be completed in early 2018.

Dr. Fogelson reported having no conflicts of interest. Other researchers on the study work for Juneau Biosciences and receive stock options as part of their compensation.

[email protected]

To the Editor:

Primary mucinous carcinoma (PMC) is an exceedingly rare adnexal tumor with an incidence of 0.07 cases per million individuals.^1,2 First described by Lennox et al³ in 1952, this entity often presents as slow-growing, solitary nodules that often are soft on palpation but may have an indurated quality and range in color from reddish blue to flesh colored to white.⁴ Primary mucinous carcinoma most commonly is found on the eyelid (38%) but may affect other sites on the face (20.3%), scalp (16%), and axilla (10%).⁵ Historically, it has been thought to be more common among men; however, a 2005 large case series by Kazakov et al⁵ found that women were twice as likely to be affected. Primary mucinous carcinoma most frequently is diagnosed in the fifth through seventh decades of life, with a median age at onset of 63 years.^6,7 Because of its rarity, PMC is most frequently confused clinically with basal cell carcinoma, keratoacanthoma, apocrine hidrocystoma, epidermoid cyst, Kaposi sarcoma, neuroma, lacrimal sac tumor, squamous cell carcinoma, granulomatous tumors, and metastatic adenocarcinoma.^1,8-10

Primary mucinous carcinoma is thought to be derived from sweat glands, and select features such as decapitation secretion are more suggestive of apocrine than eccrine differentiation.^5,8 On histopathology, PMC classically is described as nests of epithelial cells floating in lakes of extracellular mucin, primarily in the dermis and subcutis. The nests are composed of basaloid cells in solid to cribriform arrangements, usually with a low mitotic count and little nuclear atypia. These nests are suspended within periodic acid–Schiff positive mucinous pools partitioned by delicate fibrous septa. The mucin produced by PMC is sialomucin, and as such it is hyaluronidase resistant and sialidase labile.⁶ At least 1 report has been made of the presence of psammoma bodies in PMC.¹¹

The neoplasm is characterized by an indolent course with frequent recurrence but rare metastasis.^5,12 Treatment is primarily surgical, with Mohs micrographic surgery (MMS) offering improved tissue conservation and reduced recurrence rates.¹² The diagnostic challenge lies in distinguishing PMC from a variety of metastatic mucinous internal malignancies that portend a notably greater morbidity and mortality to the patient. We describe a case of PMC, discuss the differentiation of PMC from metastatic mucinous carcinoma, and review the literature regarding treatment of this rare neoplasm.

A 65-year-old white woman was referred to our tertiary-care dermatologic surgery clinic for treatment of an incompletely excised mucinous carcinoma of the right lateral canthus (Figure 1). The clinically evident scar measured 0.5×0.5 cm. Although difficult to appreciate in Figure 1, a slight textural change of the surrounding skin, including the upper and lower eyelid, was apparent. Prior to her arrival to our clinic, the referring physician had completed a thorough review of systems and physical examination, which did not suggest an underlying malignancy. Computed tomography of the head, neck, chest, abdomen, and pelvis revealed a mass in the thyroid that was removed and found to be benign. The patient’s cutaneous lesion was therefore considered to be a PMC of the skin.

Given the prior incomplete excision of the lesion and its periocular location, we treated the patient with MMS. After 6 surgical stages, we continued to see evidence of the neoplasm as it tracked medially along the orbicularis oculi muscle (Figure 2). Due to the patient’s physical and emotional exhaustion at this point, we discontinued MMS and referred her to a colleague in plastic surgery for further excision of the remaining focus of positivity as well as repair. The final Mohs defect measured 4.2×4.0 cm (Figure 3). Approximately 2.3×1.0 cm of tissue in the area of remaining tumor was excised by plastic surgery, and the defect was repaired with a cervicofacial advancement flap closure of the right cheek and lower eyelid and full-thickness skin graft of the left upper eyelid. Histopathologic investigation found the additional tissue resected to be free of residual tumor.

To diagnose a patient with PMC, one must first rule out cutaneous metastasis of various internal malignancies that may appear similar on histopathology. A full clinical investigation consisting of a thorough history, physical examination, and appropriate radiographic imaging is required. Cutaneous metastases most commonly arise from the breast or gastrointestinal tract (GIT) but also can originate from the prostate, lungs, ovaries, pancreas, and kidneys.⁵ Histologically, PMC may be identical to metastatic adenocarcinoma.¹³ Location on the body may be a clue to a lesion’s origin, as metastases from a mucinous adenocarcinoma of the breast typically occur on the chest, breast, or axilla,⁵ whereas PMC primarily is found on the head and neck.

Certain histopathologic features may be suggestive of either a primary or metastatic etiology. Lesions arising in the skin may reveal an in situ component representing ductal hyperplasia, atypical ductal hyperplasia, or ductal carcinoma in situ. Identification of an in situ component defines a cutaneous primary neoplasm, but its absence does not exclude PMC.⁵ Additionally, metastatic lesions from the GIT typically have greater pleomorphism and “dirty” necrosis defined as eosinophilic foci containing nuclear debris.⁵

The expression pattern of cytokeratins (CKs) also can be suggestive. Primary mucinous carcinoma and metastatic breast adenocarcinoma are both CK7⁺ and CK20⁻. By contrast, mucinous adenocarcinoma of the GIT stains CK20⁺ and CK7⁻.¹⁴ Another marker that stains PMC is CK5 and CK6, though infrequently present. Levy et al¹⁵ reported positive staining for CK5 and CK6 in only 1 of 5 PMC cases. Positive staining for CK5 and CK6 has not been reported in any metastatic mucinous carcinoma.

The role of p63 immunostaining in the setting of mucinous carcinoma is controversial.^16-18 Some practi-tioners have reported using p63 immunostaining to assist in establishing the diagnosis of PMC but only after performing a clinical workup to search for any primary sites of mucinous carcinoma in other organs.¹¹ Other studies, however, have found select metastatic lesions from the breast^17,18 and GIT¹⁸ to stain positively with p63. It is important to remember that these clinical and pathologic features are only suggestive of the primary etiology and are not replacement for a full clinical investigation.

Primary mucinous carcinoma is considered an indolent tumor with the majority of patient morbidity attributable to local recurrence and regional metastasis. Although uncommon, regional and distant metastasis rates have been reported to be 11% and 3%, respectively.¹⁹ Direct lymphatic invasion has been reported and indicates a more aggressive tumor with shorter recurrence-free intervals and predicts nodal metastases. Paradela et al²⁰ recommended the use of D2-40, a monoclonal antibody and specific marker for lymphatic endothelium, to detect lymphatic invasion, particularly in node-negative primary tumors.

In one case of PMC on the jaw of a 39-year-old Japanese man, no recurrence or metastases were discovered until the 11th year of follow-up. At that time, he was found to have lung and bone metastases and died after 3 years.²¹ Other investigators report death occurring 4 to 24 months following diagnosis of distant metastases.^7,22 Direct extension of the tumor into skeletal muscle, periosteum, bone, and dura also has been documented.⁷

Treatment principally is surgical, with PMC known to be resistant to both chemotherapy and radiation therapy.^19,22 The recommended margins for simple excision range from 1 to 2 cm, but this method of treatment yields recurrence rates upward of 30% to 40%, especially for lesions located on the eyelid.^12,13 First utilized in PMC of the eyelid to conserve tissue, MMS is rapidly becoming the treatment of choice because of its notably improved recurrence rate. A case series of 4 PMCs of the eyelid treated via MMS or frozen section control found the recurrence rate to be 7%.²³ Another report of 2 cases of PMC treated by MMS reported no recurrence after 42 and 26 months.¹³ Ortiz et al⁷ reported an additional case of a patient treated by MMS that was recurrence free for 30 months at the time of publication. Further investigation is required to definitively recommend MMS on the basis of improved recurrence rate but should now be considered standard of care in recurrent, sizeable, or eyelid PMC.

Despite its ascension as treatment of choice in many cases of PMC, MMS is not without its risk of metastasis and recurrence. Tam et al²⁴ reported a case of PMC with multiple recurrences and metastases following 3 simple excisions and 2 excisions via MMS. Although the lesion’s previously recurrent nature increased the likelihood of failure of MMS, this case demonstrates that all patients should be followed periodically after the treatment of PMC.

We presented a case of PMC in which standard surgical margins would have been insufficient to clear the lesion. Mohs micrographic surgery was used to remove the majority of the tumor. As is common in PMC, the lesion was indolent and periocular in location. It also was incompletely excised due to notable subclinical extension, which is common for PMC. The distinction of PMC from metastatic mucinous carcinoma is paramount but sometimes difficult. Randomized controlled trials are lacking with regards to preferred method of treatment, but MMS has shown benefit and should be considered for recurrent lesions and lesions in cosmetically sensitive areas.

To the Editor:

Primary mucinous carcinoma (PMC) is an exceedingly rare adnexal tumor with an incidence of 0.07 cases per million individuals.^1,2 First described by Lennox et al³ in 1952, this entity often presents as slow-growing, solitary nodules that often are soft on palpation but may have an indurated quality and range in color from reddish blue to flesh colored to white.⁴ Primary mucinous carcinoma most commonly is found on the eyelid (38%) but may affect other sites on the face (20.3%), scalp (16%), and axilla (10%).⁵ Historically, it has been thought to be more common among men; however, a 2005 large case series by Kazakov et al⁵ found that women were twice as likely to be affected. Primary mucinous carcinoma most frequently is diagnosed in the fifth through seventh decades of life, with a median age at onset of 63 years.^6,7 Because of its rarity, PMC is most frequently confused clinically with basal cell carcinoma, keratoacanthoma, apocrine hidrocystoma, epidermoid cyst, Kaposi sarcoma, neuroma, lacrimal sac tumor, squamous cell carcinoma, granulomatous tumors, and metastatic adenocarcinoma.^1,8-10

Primary mucinous carcinoma is thought to be derived from sweat glands, and select features such as decapitation secretion are more suggestive of apocrine than eccrine differentiation.^5,8 On histopathology, PMC classically is described as nests of epithelial cells floating in lakes of extracellular mucin, primarily in the dermis and subcutis. The nests are composed of basaloid cells in solid to cribriform arrangements, usually with a low mitotic count and little nuclear atypia. These nests are suspended within periodic acid–Schiff positive mucinous pools partitioned by delicate fibrous septa. The mucin produced by PMC is sialomucin, and as such it is hyaluronidase resistant and sialidase labile.⁶ At least 1 report has been made of the presence of psammoma bodies in PMC.¹¹

The neoplasm is characterized by an indolent course with frequent recurrence but rare metastasis.^5,12 Treatment is primarily surgical, with Mohs micrographic surgery (MMS) offering improved tissue conservation and reduced recurrence rates.¹² The diagnostic challenge lies in distinguishing PMC from a variety of metastatic mucinous internal malignancies that portend a notably greater morbidity and mortality to the patient. We describe a case of PMC, discuss the differentiation of PMC from metastatic mucinous carcinoma, and review the literature regarding treatment of this rare neoplasm.

A 65-year-old white woman was referred to our tertiary-care dermatologic surgery clinic for treatment of an incompletely excised mucinous carcinoma of the right lateral canthus (Figure 1). The clinically evident scar measured 0.5×0.5 cm. Although difficult to appreciate in Figure 1, a slight textural change of the surrounding skin, including the upper and lower eyelid, was apparent. Prior to her arrival to our clinic, the referring physician had completed a thorough review of systems and physical examination, which did not suggest an underlying malignancy. Computed tomography of the head, neck, chest, abdomen, and pelvis revealed a mass in the thyroid that was removed and found to be benign. The patient’s cutaneous lesion was therefore considered to be a PMC of the skin.

Given the prior incomplete excision of the lesion and its periocular location, we treated the patient with MMS. After 6 surgical stages, we continued to see evidence of the neoplasm as it tracked medially along the orbicularis oculi muscle (Figure 2). Due to the patient’s physical and emotional exhaustion at this point, we discontinued MMS and referred her to a colleague in plastic surgery for further excision of the remaining focus of positivity as well as repair. The final Mohs defect measured 4.2×4.0 cm (Figure 3). Approximately 2.3×1.0 cm of tissue in the area of remaining tumor was excised by plastic surgery, and the defect was repaired with a cervicofacial advancement flap closure of the right cheek and lower eyelid and full-thickness skin graft of the left upper eyelid. Histopathologic investigation found the additional tissue resected to be free of residual tumor.

To diagnose a patient with PMC, one must first rule out cutaneous metastasis of various internal malignancies that may appear similar on histopathology. A full clinical investigation consisting of a thorough history, physical examination, and appropriate radiographic imaging is required. Cutaneous metastases most commonly arise from the breast or gastrointestinal tract (GIT) but also can originate from the prostate, lungs, ovaries, pancreas, and kidneys.⁵ Histologically, PMC may be identical to metastatic adenocarcinoma.¹³ Location on the body may be a clue to a lesion’s origin, as metastases from a mucinous adenocarcinoma of the breast typically occur on the chest, breast, or axilla,⁵ whereas PMC primarily is found on the head and neck.

Certain histopathologic features may be suggestive of either a primary or metastatic etiology. Lesions arising in the skin may reveal an in situ component representing ductal hyperplasia, atypical ductal hyperplasia, or ductal carcinoma in situ. Identification of an in situ component defines a cutaneous primary neoplasm, but its absence does not exclude PMC.⁵ Additionally, metastatic lesions from the GIT typically have greater pleomorphism and “dirty” necrosis defined as eosinophilic foci containing nuclear debris.⁵

The expression pattern of cytokeratins (CKs) also can be suggestive. Primary mucinous carcinoma and metastatic breast adenocarcinoma are both CK7⁺ and CK20⁻. By contrast, mucinous adenocarcinoma of the GIT stains CK20⁺ and CK7⁻.¹⁴ Another marker that stains PMC is CK5 and CK6, though infrequently present. Levy et al¹⁵ reported positive staining for CK5 and CK6 in only 1 of 5 PMC cases. Positive staining for CK5 and CK6 has not been reported in any metastatic mucinous carcinoma.

The role of p63 immunostaining in the setting of mucinous carcinoma is controversial.^16-18 Some practi-tioners have reported using p63 immunostaining to assist in establishing the diagnosis of PMC but only after performing a clinical workup to search for any primary sites of mucinous carcinoma in other organs.¹¹ Other studies, however, have found select metastatic lesions from the breast^17,18 and GIT¹⁸ to stain positively with p63. It is important to remember that these clinical and pathologic features are only suggestive of the primary etiology and are not replacement for a full clinical investigation.

Primary mucinous carcinoma is considered an indolent tumor with the majority of patient morbidity attributable to local recurrence and regional metastasis. Although uncommon, regional and distant metastasis rates have been reported to be 11% and 3%, respectively.¹⁹ Direct lymphatic invasion has been reported and indicates a more aggressive tumor with shorter recurrence-free intervals and predicts nodal metastases. Paradela et al²⁰ recommended the use of D2-40, a monoclonal antibody and specific marker for lymphatic endothelium, to detect lymphatic invasion, particularly in node-negative primary tumors.

In one case of PMC on the jaw of a 39-year-old Japanese man, no recurrence or metastases were discovered until the 11th year of follow-up. At that time, he was found to have lung and bone metastases and died after 3 years.²¹ Other investigators report death occurring 4 to 24 months following diagnosis of distant metastases.^7,22 Direct extension of the tumor into skeletal muscle, periosteum, bone, and dura also has been documented.⁷

Treatment principally is surgical, with PMC known to be resistant to both chemotherapy and radiation therapy.^19,22 The recommended margins for simple excision range from 1 to 2 cm, but this method of treatment yields recurrence rates upward of 30% to 40%, especially for lesions located on the eyelid.^12,13 First utilized in PMC of the eyelid to conserve tissue, MMS is rapidly becoming the treatment of choice because of its notably improved recurrence rate. A case series of 4 PMCs of the eyelid treated via MMS or frozen section control found the recurrence rate to be 7%.²³ Another report of 2 cases of PMC treated by MMS reported no recurrence after 42 and 26 months.¹³ Ortiz et al⁷ reported an additional case of a patient treated by MMS that was recurrence free for 30 months at the time of publication. Further investigation is required to definitively recommend MMS on the basis of improved recurrence rate but should now be considered standard of care in recurrent, sizeable, or eyelid PMC.

Despite its ascension as treatment of choice in many cases of PMC, MMS is not without its risk of metastasis and recurrence. Tam et al²⁴ reported a case of PMC with multiple recurrences and metastases following 3 simple excisions and 2 excisions via MMS. Although the lesion’s previously recurrent nature increased the likelihood of failure of MMS, this case demonstrates that all patients should be followed periodically after the treatment of PMC.

We presented a case of PMC in which standard surgical margins would have been insufficient to clear the lesion. Mohs micrographic surgery was used to remove the majority of the tumor. As is common in PMC, the lesion was indolent and periocular in location. It also was incompletely excised due to notable subclinical extension, which is common for PMC. The distinction of PMC from metastatic mucinous carcinoma is paramount but sometimes difficult. Randomized controlled trials are lacking with regards to preferred method of treatment, but MMS has shown benefit and should be considered for recurrent lesions and lesions in cosmetically sensitive areas.

To the Editor:

Primary mucinous carcinoma (PMC) is an exceedingly rare adnexal tumor with an incidence of 0.07 cases per million individuals.^1,2 First described by Lennox et al³ in 1952, this entity often presents as slow-growing, solitary nodules that often are soft on palpation but may have an indurated quality and range in color from reddish blue to flesh colored to white.⁴ Primary mucinous carcinoma most commonly is found on the eyelid (38%) but may affect other sites on the face (20.3%), scalp (16%), and axilla (10%).⁵ Historically, it has been thought to be more common among men; however, a 2005 large case series by Kazakov et al⁵ found that women were twice as likely to be affected. Primary mucinous carcinoma most frequently is diagnosed in the fifth through seventh decades of life, with a median age at onset of 63 years.^6,7 Because of its rarity, PMC is most frequently confused clinically with basal cell carcinoma, keratoacanthoma, apocrine hidrocystoma, epidermoid cyst, Kaposi sarcoma, neuroma, lacrimal sac tumor, squamous cell carcinoma, granulomatous tumors, and metastatic adenocarcinoma.^1,8-10

Primary mucinous carcinoma is thought to be derived from sweat glands, and select features such as decapitation secretion are more suggestive of apocrine than eccrine differentiation.^5,8 On histopathology, PMC classically is described as nests of epithelial cells floating in lakes of extracellular mucin, primarily in the dermis and subcutis. The nests are composed of basaloid cells in solid to cribriform arrangements, usually with a low mitotic count and little nuclear atypia. These nests are suspended within periodic acid–Schiff positive mucinous pools partitioned by delicate fibrous septa. The mucin produced by PMC is sialomucin, and as such it is hyaluronidase resistant and sialidase labile.⁶ At least 1 report has been made of the presence of psammoma bodies in PMC.¹¹

The neoplasm is characterized by an indolent course with frequent recurrence but rare metastasis.^5,12 Treatment is primarily surgical, with Mohs micrographic surgery (MMS) offering improved tissue conservation and reduced recurrence rates.¹² The diagnostic challenge lies in distinguishing PMC from a variety of metastatic mucinous internal malignancies that portend a notably greater morbidity and mortality to the patient. We describe a case of PMC, discuss the differentiation of PMC from metastatic mucinous carcinoma, and review the literature regarding treatment of this rare neoplasm.

A 65-year-old white woman was referred to our tertiary-care dermatologic surgery clinic for treatment of an incompletely excised mucinous carcinoma of the right lateral canthus (Figure 1). The clinically evident scar measured 0.5×0.5 cm. Although difficult to appreciate in Figure 1, a slight textural change of the surrounding skin, including the upper and lower eyelid, was apparent. Prior to her arrival to our clinic, the referring physician had completed a thorough review of systems and physical examination, which did not suggest an underlying malignancy. Computed tomography of the head, neck, chest, abdomen, and pelvis revealed a mass in the thyroid that was removed and found to be benign. The patient’s cutaneous lesion was therefore considered to be a PMC of the skin.

Given the prior incomplete excision of the lesion and its periocular location, we treated the patient with MMS. After 6 surgical stages, we continued to see evidence of the neoplasm as it tracked medially along the orbicularis oculi muscle (Figure 2). Due to the patient’s physical and emotional exhaustion at this point, we discontinued MMS and referred her to a colleague in plastic surgery for further excision of the remaining focus of positivity as well as repair. The final Mohs defect measured 4.2×4.0 cm (Figure 3). Approximately 2.3×1.0 cm of tissue in the area of remaining tumor was excised by plastic surgery, and the defect was repaired with a cervicofacial advancement flap closure of the right cheek and lower eyelid and full-thickness skin graft of the left upper eyelid. Histopathologic investigation found the additional tissue resected to be free of residual tumor.

To diagnose a patient with PMC, one must first rule out cutaneous metastasis of various internal malignancies that may appear similar on histopathology. A full clinical investigation consisting of a thorough history, physical examination, and appropriate radiographic imaging is required. Cutaneous metastases most commonly arise from the breast or gastrointestinal tract (GIT) but also can originate from the prostate, lungs, ovaries, pancreas, and kidneys.⁵ Histologically, PMC may be identical to metastatic adenocarcinoma.¹³ Location on the body may be a clue to a lesion’s origin, as metastases from a mucinous adenocarcinoma of the breast typically occur on the chest, breast, or axilla,⁵ whereas PMC primarily is found on the head and neck.

Certain histopathologic features may be suggestive of either a primary or metastatic etiology. Lesions arising in the skin may reveal an in situ component representing ductal hyperplasia, atypical ductal hyperplasia, or ductal carcinoma in situ. Identification of an in situ component defines a cutaneous primary neoplasm, but its absence does not exclude PMC.⁵ Additionally, metastatic lesions from the GIT typically have greater pleomorphism and “dirty” necrosis defined as eosinophilic foci containing nuclear debris.⁵

The expression pattern of cytokeratins (CKs) also can be suggestive. Primary mucinous carcinoma and metastatic breast adenocarcinoma are both CK7⁺ and CK20⁻. By contrast, mucinous adenocarcinoma of the GIT stains CK20⁺ and CK7⁻.¹⁴ Another marker that stains PMC is CK5 and CK6, though infrequently present. Levy et al¹⁵ reported positive staining for CK5 and CK6 in only 1 of 5 PMC cases. Positive staining for CK5 and CK6 has not been reported in any metastatic mucinous carcinoma.

The role of p63 immunostaining in the setting of mucinous carcinoma is controversial.^16-18 Some practi-tioners have reported using p63 immunostaining to assist in establishing the diagnosis of PMC but only after performing a clinical workup to search for any primary sites of mucinous carcinoma in other organs.¹¹ Other studies, however, have found select metastatic lesions from the breast^17,18 and GIT¹⁸ to stain positively with p63. It is important to remember that these clinical and pathologic features are only suggestive of the primary etiology and are not replacement for a full clinical investigation.

Primary mucinous carcinoma is considered an indolent tumor with the majority of patient morbidity attributable to local recurrence and regional metastasis. Although uncommon, regional and distant metastasis rates have been reported to be 11% and 3%, respectively.¹⁹ Direct lymphatic invasion has been reported and indicates a more aggressive tumor with shorter recurrence-free intervals and predicts nodal metastases. Paradela et al²⁰ recommended the use of D2-40, a monoclonal antibody and specific marker for lymphatic endothelium, to detect lymphatic invasion, particularly in node-negative primary tumors.

In one case of PMC on the jaw of a 39-year-old Japanese man, no recurrence or metastases were discovered until the 11th year of follow-up. At that time, he was found to have lung and bone metastases and died after 3 years.²¹ Other investigators report death occurring 4 to 24 months following diagnosis of distant metastases.^7,22 Direct extension of the tumor into skeletal muscle, periosteum, bone, and dura also has been documented.⁷

Treatment principally is surgical, with PMC known to be resistant to both chemotherapy and radiation therapy.^19,22 The recommended margins for simple excision range from 1 to 2 cm, but this method of treatment yields recurrence rates upward of 30% to 40%, especially for lesions located on the eyelid.^12,13 First utilized in PMC of the eyelid to conserve tissue, MMS is rapidly becoming the treatment of choice because of its notably improved recurrence rate. A case series of 4 PMCs of the eyelid treated via MMS or frozen section control found the recurrence rate to be 7%.²³ Another report of 2 cases of PMC treated by MMS reported no recurrence after 42 and 26 months.¹³ Ortiz et al⁷ reported an additional case of a patient treated by MMS that was recurrence free for 30 months at the time of publication. Further investigation is required to definitively recommend MMS on the basis of improved recurrence rate but should now be considered standard of care in recurrent, sizeable, or eyelid PMC.

Despite its ascension as treatment of choice in many cases of PMC, MMS is not without its risk of metastasis and recurrence. Tam et al²⁴ reported a case of PMC with multiple recurrences and metastases following 3 simple excisions and 2 excisions via MMS. Although the lesion’s previously recurrent nature increased the likelihood of failure of MMS, this case demonstrates that all patients should be followed periodically after the treatment of PMC.

We presented a case of PMC in which standard surgical margins would have been insufficient to clear the lesion. Mohs micrographic surgery was used to remove the majority of the tumor. As is common in PMC, the lesion was indolent and periocular in location. It also was incompletely excised due to notable subclinical extension, which is common for PMC. The distinction of PMC from metastatic mucinous carcinoma is paramount but sometimes difficult. Randomized controlled trials are lacking with regards to preferred method of treatment, but MMS has shown benefit and should be considered for recurrent lesions and lesions in cosmetically sensitive areas.

NATIONAL HARBOR, MD. – Innovative tools for vaginal hysterectomy were in the spotlight during a surgical demonstration at the AAGL Global Congress.

“I think it’s really compelling that we use the technologies that the AAGL is known for investigating and teaching each other,” said Charles Rardin, MD, director of the robotic surgery program at Women & Infants Hospital, Providence, R.I. “It’s nice to see a renewed interest in some newer technologies and applying them to vaginal hysterectomy.”

The presentation of new tools comes as the number of vaginal hysterectomies have decreased and laparoscopic procedures are on the rise. The rate of vaginal hysterectomy in the United States has fallen from 24.8% in 1998 to 16.7% in 2010, according to the Nationwide Inpatient Sample from the Agency for Healthcare Research and Quality.

Surgeons demonstrated new tools with the intent of showing physicians that the benefits some associate with laparoscopic procedures, such as having easier access or a better sense of the uterus, can be associated with vaginal hysterectomy as well.

Advanced tools, such as a self-retaining retractor and 3-D camera systems, could make it easier to teach students by allowing more mobility and easier visual access, Dr. Rardin said in a video interview.

The tutorial ended with a demonstration of the natural orifice transluminal endoscopic surgery tool that allows laparoscopic tools to be introduced through the vaginal pathway.

All the tools exhibited at AAGL are currently available.

Dr. Rardin reported having no relevant financial disclosures.

[email protected]

On Twitter @eaztweets

NATIONAL HARBOR, MD. – Innovative tools for vaginal hysterectomy were in the spotlight during a surgical demonstration at the AAGL Global Congress.

“I think it’s really compelling that we use the technologies that the AAGL is known for investigating and teaching each other,” said Charles Rardin, MD, director of the robotic surgery program at Women & Infants Hospital, Providence, R.I. “It’s nice to see a renewed interest in some newer technologies and applying them to vaginal hysterectomy.”

The presentation of new tools comes as the number of vaginal hysterectomies have decreased and laparoscopic procedures are on the rise. The rate of vaginal hysterectomy in the United States has fallen from 24.8% in 1998 to 16.7% in 2010, according to the Nationwide Inpatient Sample from the Agency for Healthcare Research and Quality.

Surgeons demonstrated new tools with the intent of showing physicians that the benefits some associate with laparoscopic procedures, such as having easier access or a better sense of the uterus, can be associated with vaginal hysterectomy as well.

Advanced tools, such as a self-retaining retractor and 3-D camera systems, could make it easier to teach students by allowing more mobility and easier visual access, Dr. Rardin said in a video interview.

The tutorial ended with a demonstration of the natural orifice transluminal endoscopic surgery tool that allows laparoscopic tools to be introduced through the vaginal pathway.

All the tools exhibited at AAGL are currently available.

Dr. Rardin reported having no relevant financial disclosures.

[email protected]

On Twitter @eaztweets

NATIONAL HARBOR, MD. – Innovative tools for vaginal hysterectomy were in the spotlight during a surgical demonstration at the AAGL Global Congress.

“I think it’s really compelling that we use the technologies that the AAGL is known for investigating and teaching each other,” said Charles Rardin, MD, director of the robotic surgery program at Women & Infants Hospital, Providence, R.I. “It’s nice to see a renewed interest in some newer technologies and applying them to vaginal hysterectomy.”

The presentation of new tools comes as the number of vaginal hysterectomies have decreased and laparoscopic procedures are on the rise. The rate of vaginal hysterectomy in the United States has fallen from 24.8% in 1998 to 16.7% in 2010, according to the Nationwide Inpatient Sample from the Agency for Healthcare Research and Quality.

Surgeons demonstrated new tools with the intent of showing physicians that the benefits some associate with laparoscopic procedures, such as having easier access or a better sense of the uterus, can be associated with vaginal hysterectomy as well.

Advanced tools, such as a self-retaining retractor and 3-D camera systems, could make it easier to teach students by allowing more mobility and easier visual access, Dr. Rardin said in a video interview.

The tutorial ended with a demonstration of the natural orifice transluminal endoscopic surgery tool that allows laparoscopic tools to be introduced through the vaginal pathway.

All the tools exhibited at AAGL are currently available.

Dr. Rardin reported having no relevant financial disclosures.

[email protected]

On Twitter @eaztweets

To the Editor:

Acrodermatitis enteropathica (AE) is an inherited defect in zinc absorption that leads to hypozincemia. Its clinical presentation can vary based on serum zinc level and ranges from periorificial erosive dermatitis to psoriasiform dermatitis.¹ Recognition of the cutaneous manifestations of zinc deficiency can lead to early intervention with zinc supplementation and prevention of long-term morbidity and even mortality. In our case, the coexistence of a bullous acral dermatosis with the additional feature of extensor digital dermatitis with fissuring suggests a diagnosis of AE and can alert the astute clinician to the need for testing of serum zinc levels and/or treatment with zinc supplementation. Causes of acquired zinc deficiency that have been reported in the literature include eating disorders such as anorexia nervosa and bulimia nervosa, Crohn disease, food allergy, intestinal parasitic infestations, and an inborn error of metabolism known as nonketotic hyperglycemia (Table).^2-4

RELATED ARTICLE: Acquired Acrodermatitis Enteropathica Secondary to Alcoholism

A 42-year-old woman with a medical history of rheumatoid arthritis and short bowel syndrome due to multiple small bowel obstructions with subsequent bowel resections who was on chronic total parenteral nutrition (TPN) presented with bullae on the hands, shins, and feet. The patient initially noticed small erythematous macules on the hands and feet months prior to presentation. Three weeks prior to presentation, bullae started to form on the hands, mostly between the web spaces; dorsal aspects of the feet; and anterior aspects of the shins. The patient denied any oral ulcers. One day prior to presentation the patient was seen at an outside hospital and was started on prednisone 5 mg daily, oral clindamycin, mupirocin ointment, and nystatin-triamcinolone cream. These medications failed to improve her condition. On review of systems, the patient denied any fever, chills, eye pain, or dysuria.

Upon initial presentation the patient appeared weak and fatigued, though vital signs were normal. Physical examination revealed multiple flaccid bullae in the web spaces of the hands and shallow erosions with hemorrhagic crusts on the bilateral wrists. She also had violaceous patches in the extensor creases of the metacarpophalangeal, proximal interphalangeal, and distal interphalangeal joints, which were strikingly symmetric (Figure 1). Prominent flaccid bullae and shallow erosions with hemorrhagic crusts also were present on the bilateral shins and dorsal aspects of the feet (Figure 2). No oral ulcers were present. A punch biopsy from the dorsal aspect of the left foot revealed psoriasiform hyperplasia of the epidermis with prominent ballooning degeneration and hyperkeratosis/parakeratosis (Figure 3); a periodic acid–Schiff stain was negative for fungal organisms.

Given the biopsy results and clinical presentation, a nutritional deficiency was suspected and serum levels of zinc, vitamin B₁, vitamin B₂, and vitamin B₃ were assessed. Vitamins B₁, B₂, and B₃ all were within reference range, but the patient’s serum zinc level was found to be low at 11 μg/mL (reference range, 55–150 μg/mL). The alkaline phosphatase level also was measured to be low at 22 U/L (reference range, 31–103 U/L). Additionally, a hepatitis panel was drawn and glucagon levels were checked, which were found to be within reference range. These findings were consistent with a diagnosis of acquired AE. Prednisone and clindamycin were stopped and the patient was started on zinc supplementation in her TPN therapy. Mupirocin ointment was continued on the existing bullae. Upon discharge 10 days later, there were no new bullae and the existing bullae had sloughed off, revealing healthy skin underneath.

Zinc is an essential trace element and can be found in high concentration in foods such as shellfish, green vegetables, legumes, nuts, and whole grains.⁶ The majority of zinc is absorbed in the jejunum; as such, many cases of acquired zinc deficiency leading to AE are dueto disorders that affect the small intestine.² Conditions that may lead to poor gastrointestinal zinc absorption include alcoholism, eating disorders, TPN, burns, surgery, and malignancies.^2,7

Diagnosis typically is made based on characteristic clinical features, biopsy results, and a measurement of the serum zinc concentration. Although a low serum zinc level supports the diagnosis, serum zinc concentration is not a reliable indicator of body zinc stores and a normal serum zinc concentration does not rule out AE. The gold standard for diagnosis is the resolution of lesions after zinc supplementation.¹ Notably, because the production of alkaline phosphatase is dependent on zinc, levels of this enzyme also may be low in cases of AE,⁶ as in our patient.

The clinical manifestations of AE can vary greatly; patients may initially present with eczematous pink scaly plaques, which may subsequently become vesicular, bullous, pustular, or desquamative. The lesions may develop over the arms and legs as well as the anogenital and periorificial areas.⁵ Other notable manifestations that may present early in the course of AE include angular cheilitis followed by paronychia. In patients who are not promptly treated, long-term zinc deficiency may lead to growth delay, mental slowing, poor wound healing, anemia, and anorexia.⁵ Of note, deficiencies of branched-chain amino acids and essential fatty acids may appear clinically similar to AE.²

Zinc replacement is the treatment of choice for patients with AE due to dietary deficiency, and replacement therapy should begin with 0.5 to 1 mg/kg daily of elemental zinc.⁵ Response to acquired AE with zinc supplementation often is rapid. Lesions tend to resolve within days to weeks depending on the degree of deficiency.²

Although AE is an uncommon dermatosis in the United States, it is an important diagnosis to make because its clinical features are fairly specific and early zinc supplementation allows for full resolution of the disease without permanent sequelae. The diagnosis of AE should be strongly considered when features of an acral bullous dermatosis are combined with a fissured dermatitis of extensor joints of the hands or elbows. It is particularly important to recognize that alcoholics, burn victims, postsurgical patients, and those with malignancies and eating disorders are at an increased risk for developing this nutritional deficiency.

To the Editor:

Acrodermatitis enteropathica (AE) is an inherited defect in zinc absorption that leads to hypozincemia. Its clinical presentation can vary based on serum zinc level and ranges from periorificial erosive dermatitis to psoriasiform dermatitis.¹ Recognition of the cutaneous manifestations of zinc deficiency can lead to early intervention with zinc supplementation and prevention of long-term morbidity and even mortality. In our case, the coexistence of a bullous acral dermatosis with the additional feature of extensor digital dermatitis with fissuring suggests a diagnosis of AE and can alert the astute clinician to the need for testing of serum zinc levels and/or treatment with zinc supplementation. Causes of acquired zinc deficiency that have been reported in the literature include eating disorders such as anorexia nervosa and bulimia nervosa, Crohn disease, food allergy, intestinal parasitic infestations, and an inborn error of metabolism known as nonketotic hyperglycemia (Table).^2-4

RELATED ARTICLE: Acquired Acrodermatitis Enteropathica Secondary to Alcoholism

A 42-year-old woman with a medical history of rheumatoid arthritis and short bowel syndrome due to multiple small bowel obstructions with subsequent bowel resections who was on chronic total parenteral nutrition (TPN) presented with bullae on the hands, shins, and feet. The patient initially noticed small erythematous macules on the hands and feet months prior to presentation. Three weeks prior to presentation, bullae started to form on the hands, mostly between the web spaces; dorsal aspects of the feet; and anterior aspects of the shins. The patient denied any oral ulcers. One day prior to presentation the patient was seen at an outside hospital and was started on prednisone 5 mg daily, oral clindamycin, mupirocin ointment, and nystatin-triamcinolone cream. These medications failed to improve her condition. On review of systems, the patient denied any fever, chills, eye pain, or dysuria.

Upon initial presentation the patient appeared weak and fatigued, though vital signs were normal. Physical examination revealed multiple flaccid bullae in the web spaces of the hands and shallow erosions with hemorrhagic crusts on the bilateral wrists. She also had violaceous patches in the extensor creases of the metacarpophalangeal, proximal interphalangeal, and distal interphalangeal joints, which were strikingly symmetric (Figure 1). Prominent flaccid bullae and shallow erosions with hemorrhagic crusts also were present on the bilateral shins and dorsal aspects of the feet (Figure 2). No oral ulcers were present. A punch biopsy from the dorsal aspect of the left foot revealed psoriasiform hyperplasia of the epidermis with prominent ballooning degeneration and hyperkeratosis/parakeratosis (Figure 3); a periodic acid–Schiff stain was negative for fungal organisms.

Given the biopsy results and clinical presentation, a nutritional deficiency was suspected and serum levels of zinc, vitamin B₁, vitamin B₂, and vitamin B₃ were assessed. Vitamins B₁, B₂, and B₃ all were within reference range, but the patient’s serum zinc level was found to be low at 11 μg/mL (reference range, 55–150 μg/mL). The alkaline phosphatase level also was measured to be low at 22 U/L (reference range, 31–103 U/L). Additionally, a hepatitis panel was drawn and glucagon levels were checked, which were found to be within reference range. These findings were consistent with a diagnosis of acquired AE. Prednisone and clindamycin were stopped and the patient was started on zinc supplementation in her TPN therapy. Mupirocin ointment was continued on the existing bullae. Upon discharge 10 days later, there were no new bullae and the existing bullae had sloughed off, revealing healthy skin underneath.

Zinc is an essential trace element and can be found in high concentration in foods such as shellfish, green vegetables, legumes, nuts, and whole grains.⁶ The majority of zinc is absorbed in the jejunum; as such, many cases of acquired zinc deficiency leading to AE are dueto disorders that affect the small intestine.² Conditions that may lead to poor gastrointestinal zinc absorption include alcoholism, eating disorders, TPN, burns, surgery, and malignancies.^2,7

Diagnosis typically is made based on characteristic clinical features, biopsy results, and a measurement of the serum zinc concentration. Although a low serum zinc level supports the diagnosis, serum zinc concentration is not a reliable indicator of body zinc stores and a normal serum zinc concentration does not rule out AE. The gold standard for diagnosis is the resolution of lesions after zinc supplementation.¹ Notably, because the production of alkaline phosphatase is dependent on zinc, levels of this enzyme also may be low in cases of AE,⁶ as in our patient.

The clinical manifestations of AE can vary greatly; patients may initially present with eczematous pink scaly plaques, which may subsequently become vesicular, bullous, pustular, or desquamative. The lesions may develop over the arms and legs as well as the anogenital and periorificial areas.⁵ Other notable manifestations that may present early in the course of AE include angular cheilitis followed by paronychia. In patients who are not promptly treated, long-term zinc deficiency may lead to growth delay, mental slowing, poor wound healing, anemia, and anorexia.⁵ Of note, deficiencies of branched-chain amino acids and essential fatty acids may appear clinically similar to AE.²

Zinc replacement is the treatment of choice for patients with AE due to dietary deficiency, and replacement therapy should begin with 0.5 to 1 mg/kg daily of elemental zinc.⁵ Response to acquired AE with zinc supplementation often is rapid. Lesions tend to resolve within days to weeks depending on the degree of deficiency.²

Although AE is an uncommon dermatosis in the United States, it is an important diagnosis to make because its clinical features are fairly specific and early zinc supplementation allows for full resolution of the disease without permanent sequelae. The diagnosis of AE should be strongly considered when features of an acral bullous dermatosis are combined with a fissured dermatitis of extensor joints of the hands or elbows. It is particularly important to recognize that alcoholics, burn victims, postsurgical patients, and those with malignancies and eating disorders are at an increased risk for developing this nutritional deficiency.

To the Editor:

Acrodermatitis enteropathica (AE) is an inherited defect in zinc absorption that leads to hypozincemia. Its clinical presentation can vary based on serum zinc level and ranges from periorificial erosive dermatitis to psoriasiform dermatitis.¹ Recognition of the cutaneous manifestations of zinc deficiency can lead to early intervention with zinc supplementation and prevention of long-term morbidity and even mortality. In our case, the coexistence of a bullous acral dermatosis with the additional feature of extensor digital dermatitis with fissuring suggests a diagnosis of AE and can alert the astute clinician to the need for testing of serum zinc levels and/or treatment with zinc supplementation. Causes of acquired zinc deficiency that have been reported in the literature include eating disorders such as anorexia nervosa and bulimia nervosa, Crohn disease, food allergy, intestinal parasitic infestations, and an inborn error of metabolism known as nonketotic hyperglycemia (Table).^2-4

RELATED ARTICLE: Acquired Acrodermatitis Enteropathica Secondary to Alcoholism

A 42-year-old woman with a medical history of rheumatoid arthritis and short bowel syndrome due to multiple small bowel obstructions with subsequent bowel resections who was on chronic total parenteral nutrition (TPN) presented with bullae on the hands, shins, and feet. The patient initially noticed small erythematous macules on the hands and feet months prior to presentation. Three weeks prior to presentation, bullae started to form on the hands, mostly between the web spaces; dorsal aspects of the feet; and anterior aspects of the shins. The patient denied any oral ulcers. One day prior to presentation the patient was seen at an outside hospital and was started on prednisone 5 mg daily, oral clindamycin, mupirocin ointment, and nystatin-triamcinolone cream. These medications failed to improve her condition. On review of systems, the patient denied any fever, chills, eye pain, or dysuria.

Upon initial presentation the patient appeared weak and fatigued, though vital signs were normal. Physical examination revealed multiple flaccid bullae in the web spaces of the hands and shallow erosions with hemorrhagic crusts on the bilateral wrists. She also had violaceous patches in the extensor creases of the metacarpophalangeal, proximal interphalangeal, and distal interphalangeal joints, which were strikingly symmetric (Figure 1). Prominent flaccid bullae and shallow erosions with hemorrhagic crusts also were present on the bilateral shins and dorsal aspects of the feet (Figure 2). No oral ulcers were present. A punch biopsy from the dorsal aspect of the left foot revealed psoriasiform hyperplasia of the epidermis with prominent ballooning degeneration and hyperkeratosis/parakeratosis (Figure 3); a periodic acid–Schiff stain was negative for fungal organisms.

Given the biopsy results and clinical presentation, a nutritional deficiency was suspected and serum levels of zinc, vitamin B₁, vitamin B₂, and vitamin B₃ were assessed. Vitamins B₁, B₂, and B₃ all were within reference range, but the patient’s serum zinc level was found to be low at 11 μg/mL (reference range, 55–150 μg/mL). The alkaline phosphatase level also was measured to be low at 22 U/L (reference range, 31–103 U/L). Additionally, a hepatitis panel was drawn and glucagon levels were checked, which were found to be within reference range. These findings were consistent with a diagnosis of acquired AE. Prednisone and clindamycin were stopped and the patient was started on zinc supplementation in her TPN therapy. Mupirocin ointment was continued on the existing bullae. Upon discharge 10 days later, there were no new bullae and the existing bullae had sloughed off, revealing healthy skin underneath.

Zinc is an essential trace element and can be found in high concentration in foods such as shellfish, green vegetables, legumes, nuts, and whole grains.⁶ The majority of zinc is absorbed in the jejunum; as such, many cases of acquired zinc deficiency leading to AE are dueto disorders that affect the small intestine.² Conditions that may lead to poor gastrointestinal zinc absorption include alcoholism, eating disorders, TPN, burns, surgery, and malignancies.^2,7

Diagnosis typically is made based on characteristic clinical features, biopsy results, and a measurement of the serum zinc concentration. Although a low serum zinc level supports the diagnosis, serum zinc concentration is not a reliable indicator of body zinc stores and a normal serum zinc concentration does not rule out AE. The gold standard for diagnosis is the resolution of lesions after zinc supplementation.¹ Notably, because the production of alkaline phosphatase is dependent on zinc, levels of this enzyme also may be low in cases of AE,⁶ as in our patient.

The clinical manifestations of AE can vary greatly; patients may initially present with eczematous pink scaly plaques, which may subsequently become vesicular, bullous, pustular, or desquamative. The lesions may develop over the arms and legs as well as the anogenital and periorificial areas.⁵ Other notable manifestations that may present early in the course of AE include angular cheilitis followed by paronychia. In patients who are not promptly treated, long-term zinc deficiency may lead to growth delay, mental slowing, poor wound healing, anemia, and anorexia.⁵ Of note, deficiencies of branched-chain amino acids and essential fatty acids may appear clinically similar to AE.²

Zinc replacement is the treatment of choice for patients with AE due to dietary deficiency, and replacement therapy should begin with 0.5 to 1 mg/kg daily of elemental zinc.⁵ Response to acquired AE with zinc supplementation often is rapid. Lesions tend to resolve within days to weeks depending on the degree of deficiency.²

Although AE is an uncommon dermatosis in the United States, it is an important diagnosis to make because its clinical features are fairly specific and early zinc supplementation allows for full resolution of the disease without permanent sequelae. The diagnosis of AE should be strongly considered when features of an acral bullous dermatosis are combined with a fissured dermatitis of extensor joints of the hands or elbows. It is particularly important to recognize that alcoholics, burn victims, postsurgical patients, and those with malignancies and eating disorders are at an increased risk for developing this nutritional deficiency.