Keith Loria

Pediatric patients in need of infusion therapy face the same access barriers as their adult counterparts. But they also deal with the added challenges of finding sites of care and infusion providers that are accustomed to caring for children.

Robert Dracker, MD, MHA, MBA, CPI, medical director of Summerwood Pediatrics & Infusacare Medical Services in Liverpool, New York, spoke on these issues at the 2022 National Infusion Center Association (NICA) Annual Conference, held June 17 and 18, 2022, in New Orleans, Louisiana.1

Dracker discussed considerations and developmentally appropriate interventions to promote high-quality, patient-centered pediatric infusion care. Dracker started with a question: “What is infusion medicine?” There is no standard definition, he said, so he crafted his own, drawing on his decades of experience in the field. Dracker defined this growing sector as, “the health care specialty involved with the clinical science and practice of administering parenteral fluids, medications, biologic, and cellular therapies to adults and children.”

Dracker also touched on the unique needs of children who will be receiving intravenous (IV) or parenteral medications. Compared with an emergency department visit or for a hospitalized child, outpatient infusion therapy is semielective and scheduled, allowing time to prepare the child for the therapy.

“This is a very difficult thing to do for a child in general,” he said. “A [provider] should explain the need for the therapy and obtain an informed consent in some cases, particularly with regards to some of the newer biologic therapies for diagnoses like eczema and psoriasis.”

He compared general therapy considerations with important concerns specific to dealing with pediatric patients, reminding health care professionals that children are not just tiny adults. Things to know and factor into the care include the age of the child and any developmental issues, how ill the child is (acute vs chronic), who is with the child at the visit, any preexisting conditions that could complicate the therapeutic intervention, and the child’s understanding of the treatment and informed consent.

Dracker spoke about the importance of establishing a relationship between a provider and the infusion site team; there must be open communication between both to address issues such as special needs of a particular patient, who will be monitoring the response and follow up labs, and how to partner medically with regards to new emerging therapies.

Dracker also noted that treating a child may raise the anxiety of the medical team, so a collaborative approach is helpful, especially at the start of the therapy. He stressed that an open, interactive relationship between the provider's practice and the therapy site is critical for care.

“There should obviously be communication between both regarding currently available and emerging therapies, the needs for prior authorization, follow-up lab work required, and the fact that the infusion center staff will commonly see the patient during treatment more often than the dermatologist, which means that the response to treatment, or lack thereof, should also be conveyed back to the referring office,” he said.

Providers can take steps to prepare children for treatment in this environment by familiarizing the child with the environment, explaining each step of the process, and surrounding them with love, affection, and even “bribes” such as cartoons, books, and snacks.

In general, parents are fairly motivated to ensure that their child receives their therapy on a scheduled basis. It is important to provide a reminder to parents for an upcoming appointment with regards to date and time. Dracker also urged infusion providers to make an attempt to accommodate a child’s needs, especially related to school and activities.

Dracker also touched on vascular access issues in pediatric patients, the importance of being honest with children, and steps the FDA is taking to ensure access to safe and effective medical products for children.

At the conclusion of his talk, Dracker shared his hope that attendees could walk away with the understanding that children have special needs when it comes to infusion therapy treatments and that in many ways, these providers are treating a whole family—an experience that can be positive or negative depending upon the efforts made and the accommodations provided.

This article was published by our sister publication Drug Topics.

1. Dracker R. Not just tiny adults: considerations for a pediatric population. Presented at: 2022 National Infusion Center Association Annual Conference; June 17-18, 2022; New Orleans, LA.

Articles

An expert offers considerations and developmentally appropriate interventions to promote high-quality, patient-centered pediatric infusion care.

Optimizing outcomes for pediatric infusion therapy

Pediatric patients in need of infusion therapy face the same access barriers as their adult counterparts. But, they also deal with the added challenges offinding sites of care and infusion providers that are accustomed to caring for children.

Robert Dracker, MD, MHA, MBA, CPI, medical director of Summerwood Pediatrics & Infusacare Medical Services in Liverpool, New York, spoke on these issues at the 2022 National Infusion Center Association (NICA) Annual Conference, held June 17 to 18, 2022 in New Orleans.

In his seminar, Dracker discussed considerations and developmentally appropriate interventions to promote high quality, patient-centered pediatric infusion care.

“What is infusion medicine?” he began. Dracker explained there’s no standard definition so he crafted his own, drawing on his decades of experience in the field. He defined this growing sector as, “the healthcare specialty involved with the clinical science and practice of administering parenteral fluids, medications, biologic, and cellular therapies to adults and children.”

He then delved into the essential attributes of an infusion facility, which include parking, facility access, patient privacy, a drug preparation space, a private consultation area, lab support, and emergency cart and room for allowing ease of emergency transportation.

Dracker moved his talk to the unique needs of children who will be receiving IV or parenteral medications. As compared to an emergency room visit or for a hospitalized child, outpatient infusion therapy is semi-elective and scheduled, allowing time to preparethe child for the therapy.

“This is a very difficult thing to do for a child in general, however,” he said. “A dermatologist should explain the need for the therapy and obtain an informed consent in some cases, particularly with regards to some of the newer biologic therapies for diagnoses like eczema and psoriasis.”

He compared general therapy considerations with important concerns specific to dealing with pediatric patients, reminding health care professionals that children are not just tiny adults. Things to know and factor into the care include the age of the child and any developmental issues, how ill the child is (acute vs chronic), who is with the child at the visit, any pre-existing conditions that could complicate the therapeutic intervention, and the child’s understanding of the treatment and informed consent.

Dracker spoke about the importance of establishing a relationship between a dermatologist and an infusion site’s team as there needs to be open communication between both for such issues like special needs of a particular patient, who will be monitoring the response and follow up labs if necessary, and how to partner medically with regards to new emerging therapies.

Furthermore, Dracker noted that treating a child may raise the medical team’s anxiety level, so a collaborative approach is helpful in discussing the start of a child’s therapy. He stressed the need for an open, interactive relationship between a dermatology practice and the administering therapy site is critical for care.

“There should obviously be communication between both regarding currently available and emerging therapies, the needs for prior authorization, follow up lab work required, and the fact that the infusion center staff will commonly see the patient during treatment more often than the dermatologist, which means that the response to treatment, or lack thereof should also be conveyed back to the referring office,” he said.

In his opinion, a sound treatment preparation would involve familiarizing a child with the environment, explaining what you are going to do (regardless of age), and surrounding them with love, affection and even “bribes” such as cartoons, books, and snacks.

Dracker mentioned that parents typically are fairly motivated to ensure that their child receives any thetherapy on a scheduled basis. It is important to provide a reminder to parents for an upcoming appointment with regards to date and time, he stated, and urged infusion providers to make an attempt to accommodate a child’s needs, especially related to school and activities.

Other key points of his talk included vascular access issues that one might face, the importance of being honest with children and he introduced some noteworthy clinical research, including what the FDA is doing to assure pediatric patients have access to safe and effective medical products.

At the conclusion of his talk, Dracker returned to his thesis of the unique needs of children and hoped attended walked away with the understanding that children have special needs in understanding their treatments and that in many ways, the dermatologists are treating the whole family, which can be negative or positive depending upon the efforts made and the accommodations provided.

Originally published on our sister brand, 

Dracker R. Not just tiny adults: considerations for a pediatric population. Presented at: 2022 National Infusion Center Association Annual Conference; June 17-18, 2022, New Orleans, Louisiana.

Robert Dracker, MD, MHA, MBA, CPI, discussed considerations and developmentally appropriate interventions to promote high quality, patient-centered pediatric infusion care in a presentation at the 2022 National Infusion Center Association Annual Conference.

Optimize outcomes for pediatric patients who need infusion therapy

Prescription of isotretinoin is a challenging endeavor, requiring collaboration between providers, office administrative staff, pharmacists, insurance companies, and laboratory facilities.

As the administrative burden is not well elucidated, a group of researchers conducted a study aiming to quantify the workload required to manage patients on isotretinoin compared to non-isotretinoin medications for acne treatment.

“We wanted to explore this topic because often in the clinic we encountered issues with processing isotretinoin prescriptions and many patients faced difficulty with the process,” said Anna Kirkorian, MD, chief of dermatology at Children’s National in Washington, D.C. “For example, our nurses would have to spend hours on the phone explaining the iPLEDGE process and how to complete the online questionnaires to patients, as well as speak with insurance companies regarding medication prior authorizations.”

In the paper, “The administrative burden of prescribing and treating with isotretinoin,” Kirkorian and the research team explored he effectiveness of the iPLEDGE program, and found it decreased patient access and increased administrative burden.

The study included all patients under the age of 21 years who received both isotretinoin and non-isotretinoin medications for acne treatment at Children’s National Hospital in the time period of 2012 to 2019.

“We performed a retrospective chart review of all patients who received isotretinoin for the treatment of acne,” said Nidhi Shah, MD, candidate at George Washington University’s school of medicine and health sciences, who served as lead author on the paper. “Patients generally have to fail other medications such as topicals and oral antibiotics prior to receiving isotretinoin, hence, we compared the administrative burden associated with prescribing these other acne medications (pre-isotretinoin) and for prescribing isotretinoin in the same patient cohort.”

From the chart review, the researchers extracted information such as patient demographics, patient treatment information, as well as the number and type of communications made by the nursing staff.

“We explored the nursing communications which provided insight on the number of calls that the staff had to make to different stakeholders, including patient, pharmacy, insurance company, physician, and iPLEDGE,” Shah said. “Additionally, we explored the reasons behind the communications which allowed us to understand the biggest hurdles to isotretinoin prescription.”

The data showed a total of 1,609 communications occurred during isotretinoin treatment; 40 percent were calls with patients, 24 percent were calls with pharmacies, 13 percent were calls with insurance, and 19 percent were messages with providers. And the majority of the calls involved prior authorization processing and patient education about iPLEDGE.

“By comparing the number of communications required for non-isotretinoin medication and isotretinoin within the same patient population, we were able to control for variables and appreciate the vast difference in workload required for isotretinoin vs. other medications,” Shah said.

One notable takeaway of the data is that despite having similar lengths of treatment of non-isotretinoin and isotretinoin, prescription of isotretinoin resulted in significant more workload for the administrative staff (10.70±8.54 communications/patient occurred during isotretinoin treatment versus 1.72±2.66 communications/patient during non-isotretinoin treatment (p<0.01)).

Another was that significantly more communications/patient occurred for patients with public insurance (2.24±1.72) than private insurance (1.58±1.28) (p=0.033) and when a prior authorization (PA) was required (2.38±1.34) than when no PA was required (1.64±0.97) (p<0.001).

“Although we were not surprised to learn that isotretinoin prescriptions required a greater workload than non-isotretinoin acne medications, we were definitely surprised by the significant difference in the amount of workload,” Kirkorian said. “Additionally, when translating the call lengths to the cost on the healthcare system, we were surprised that the cost to process one isotretinoin prescription could range from $6.15-$147.80 in non-reimbursable staff hours.”

One of the biggest challenges for the administrative staff that was gleaned during the study was the coordination of prior authorization process between the pharmacy and the insurance company, especially within the 7-day prescription window which added a time urgency.

The administrative staff served as an intermediary between stakeholders, investigating why the physician sign-off is not displaying on the pharmacy’s iPLEDGE portal or coordinating PAs between the pharmacy and insurance company.

After all, the iPLEDGE program requires involvement of all stakeholders (patient, physician, pharmacy, insurance company, laboratory facility, and iPLEDGE) to successfully prescribe isotretinoin.

“iPLEDGE requires each stakeholder to complete their own set of required tasks (ex. physician must sign off in iPLEDGE, patient must take an online questionnaire, pharmacy must verify patient’s iPLEDGE status),” Shah said. “The multiple steps required especially for childbearing patients poses a lot of obstacles for patients and the nursing staff is in the center to coordinate all of the items. The administrative staff is racing against time to ensure successful prescription of isotretinoin and this orchestration is burdensome, costly, and takes away time from other duties.”

Since the implementation of iPLEDGE, there has been a shift in isotretinoin prescription pattern from low-volume general providers to high-volume specialist providers, which may be secondary to the administrative burden associated with isotretinoin prescription.

“Given that many of the patient barriers to accessing isotretinoin—for example patient delays in insurance prior authorization or inability to find an iPLEDGE-participating pharmacy—align with the reasons for the significant administrative burden,” Kirkorian said. “Our study sheds light on the implications of iPLEDGE from a physician/administrative perspective, with hopes that future changes in the program both work towards decreasing the burden on both patients and providers to increase access and treatment adherence.”

While the study was limited to a single site and pediatric population, Shah noted that by comparing the workload for isotretinoin and non-isotretinoin in the same cohort, the research team could control for patient variables, contributing to workload differences.

Therefore, the findings underestimate the actual administrative burden as non-phone communications and time spent on documentation, iPLEDGE portal activities, and electronic PA submissions were not included.

“The administrative staff is racing against time to ensure successful prescription of isotretinoin, and this orchestration is burdensome, costly, and takes away time from other duties,” she said. “While iPLEDGE serves an important role in preventing fetal exposure to isotretinoin, streamlining the process of prescription could decrease both the administrative and patient workload, thereby reducing potential treatment barriers.”

Shah N, Smith E, Czermak J, Ferris M, Kirkorian A. The administrative burden of prescribing and treating with isotretinoin. Journal of the American Academy of Dermatology. April 23, 2021.

Isotretinoin prescriptions are a challenging endeavor, requiring collaboration between providers, office administrative staff, pharmacists, insurance companies, and laboratory facilities.

The challenges of prescribing isotretinoin

Femke de Vries, MD, department of dermatology, Radboud University Medical Center in Nijmegen, Netherlands, led a study designed to discover a better understanding of the association between quality of life (QoL) and physician-led acne care. The study, “The association between acne care provision and quality of life: A cross-sectional survey,” also investigated other factors that might influence QoL such as age, gender, and acne severity.

“Since acne is often considered a cosmetic and non-life-threatening skin condition, the consequences are frequently overlooked medically,”de Vries wrote in the study. “Acne can have a profound negative psychological impact with implications for patients’ health related quality of life. In order to improve the quality of the Dutch dermatological care provision, a more personalized patient-centered care is recommended.”

This more personalized approach includes facilitating the integration of measuring QoL into routine clinical practice, she continued. To do so, de Vriesnoted, it is important to gain a better understanding of the factors influencing the QoL in the acne population and which patients would benefit most from measuring QoL in routine clinical practice.

To gather data on the impact of acne vulgaris on health-related QoL, a cross-sectional, questionnaire-based study was conducted utilizing the Cardiff Acne Disability Index (CADI) and a global QoL scale to capture the QoL within the Dutch acne population. This data was then examined for QoL factors. The study was reported according to the recommendations in the Strengthening the Reporting of Observational Studies in Epidemiology checklist.

“Furthermore, to investigate the association of QoL with acne severity we used the Patient Reported Global Assessment (PGA)-severity scale,”de Vries said. “We analyzed several factors that derived from literature and could potentially influence the QoL, such as acne severity, the type of care provider providing treatment, age, and gender.”

The CADI contained 5 questions to gather QoL data points, including patients’ feelings when dealing with acne, social life, skin exposure, and overall acne severity assessment by the patient. The questionnaire also contained a global QoL scale, to give the patients the opportunity to report QoL improvement experienced before and after care.

In total, 371 CADI questionnaires were completed, though 9 were dismissed due to incomplete data. The study population consisted of 79% females and 21% males and was conducted between 2019 and 2020.

No statistically significant differences were found in QoL measured by CADI among patients visiting the 4 investigated acne caregivers (mean CADI score: dermatologist, 4.49; GPs, 4.42; dermal therapist, 4.07; beautician, 4.20, P = .24). However, the impact of the treatment on the QoL, which was measured by the level of global QoL improvement before and after care,demonstrated a statistically significant improvement when attending a dermatologist, compared to the care provided by beauticians.

“The study delineated factors that influence QoL in acne patients,” she said. “As the QoL was not associated with the type of caregiver, the greatest QoL improvement before and after care was achieved after medical treatment by the dermatologist.”

Discrepancies were found for the influence of age on QoL. de Vries noted previous studies have demonstrated a greater impact on QoL in older patients with acne than in younger patients; however, the study authors were not able to detect this difference, citing the small number of respondents over 40 years old involved.

What it did learn was patients dealing with more severe acne experienced a more effected QoL. de Vries said it is recommended to take these factors into account in acne management to optimize professional treatment in line with patient needs.

“I think that the most important message coming from this research is that several identified factors (females and more severe acne) influence the QoL and professional treatment can improve the QoL in acne patients,” de Vries said.

The study authors recommend taking these factors into account in acne management and guidance of patients to optimize professional treatment in line with patient needs.

“We were not surprised finding results that underlined the fact that dealing with acne influence the QoL; however, from a certain degree of acne severity (i.e. corresponding to moderately to severe acne), the level of QoL impairment did not increase, indicating that the magnitude of acne on a person’s QoL was almost equal among more severe types of acne.”

Buidling on this knowledge shows that identifying the possible value of caregivers on the acne related QoLmay contribute to adopting QoL measures as an integral part of acne management, the study authors found. This identification may improve acne care in clinical practice and create treatment more in line with the patients' needs.

Based on these conclusions, the study authors are encouraged to use QoL measurements in clinical practice as a substantial indicator in delivering high quality of care, that is more in line with the patients’ needs.

“Due to the high impact of acne on patients’ lives, adopting a QoL measure as an integral part of acne management is highly recommended and benefits both the patient and the care (provider) in many ways,” de Vries said.

This article was originally posted by sister publication Dermatology Times.

de Vries F, Driessen R, Tjin E, Westenberg A, Vehof H, van de Kerkhof P. The association between acne care provision and quality of life: A cross-sectional survey. 

There is a theory that professional acne care can be effective not only in decreasing clinical signs but also in improving quality of life.

Improve quality of life with acne care

The sexual and gender minority population, which includes transgender and gender diverse people, have long been underserved in the field of dermatology.

While the unique dermatologic needs for adult patients in this group have been documented, the knowledge surrounding pediatric and adolescent transgender/gender diverse patients can be grown.

In the study, “Dermatologic considerations for transgender and gender diverse youth,” published by 

 researchers found that although the dermatologic conditions faced by transgender and gender diverse youth are also seen in cisgender patients. The cause of their conditions and treatments may differ between the groups which may require special considerations.

“LGBTQ+ patients are an underserved population and I believe that as physicians, we are obligated to provide support to vulnerable groups in order to close the gaps on inequities in healthcare,” said lead author Shane M. Swink, DO, MS, chief resident of dermatology at the Lehigh Valley Health Network in Allentown, Pennsylvania. “On a personal note, as a gay physician, I feel compelled to use my status and privilege as a physician to advocate for all members of the LGBTQ+ community including transgender and gender diverse people.”

With his interests in pediatric dermatology, Swink was particularly interested to review the literature and see what has been done in the treatment of transgender and gender diverse youth. He aimed to summarize the unique dermatologic needs of these patients, with a key focus on the data available in younger groups.

“Many sexual and gender minority individuals are underserved and vulnerable in all fields of medicine, not just dermatology,” Swink said. “This is due to several reasons including discrimination, stigma, and denial of basic civil and human rights for this group. This can lead to things like lack of insurance, lack of access to care, and simply not seeking care from providers due to embarrassment or fear of discrimination.”

Sexual and gender minority individuals are an underrepresented population of patients, and many are speaking up to raise awareness through research and advocacy. This study highlights the role pediatric dermatologists can play in the care of transgender and gender diverse youth including but not limited to treating acne and hair pattern changes as well as being knowledgeable about surgical and other cosmetic options for gender affirmation.

It also dives into the areas for potential future research including dermatologic effects of hormone therapy in children as well as treatments for those side effects.

“The biggest takeaway from this literature review research was that while more research is being done regarding dermatologic care of transgender and gender diverse adult patients, there is ample opportunity for much research and work to be done regarding younger populations,” Swink said.

Pediatric dermatologists increase the quality of care for these patients, the study suggests, by treating dermatologic side effects of hormonal therapy. This help can include providing guidance relevant to dermatologic treatments related to surgical procedures patients may undergo, providing minimally invasive procedures for gender affirmation, and serving as an advocate and educator for patients and their families. There are challenges.

“The skin conditions faced by transgender and gender diverse youth are not specifically distinct from cisgender skin conditions, but they may necessitate a unique or sensitive approach to care,” Swink said. “For example, until December 2021, the iPLEDGE system mandated when treating patients with isotretinoin for acne used gendered (male and female) categories and language. This required a gentle and more gender-neutral counseling regarding iPLEDGE counseling."

, gender neutral categories—patients who can get pregnant and patients who cannot get pregnant—were adopted, making a more inclusive experience for patients, but these changes were made without consultation. 

There are still other issues that dermatologists have with the program.

“This example highlights the importance of always going into encounters with transgender and gender diverse patients and maintaining a gender-neutral vocabulary,” Swink said. “Some things that occur during encounters can make patients feel uncomfortable or labeled in a way that does not align with their gender identity.”

These can include inadvertently using gendered titles of hair loss patterns, gendered language in educational pamphlets in doctor offices, examination of sensitive areas, and lacking an understanding that a patient's gender may not align with traditionally masculine or feminine features.

“It’s our job as physicians to identify a patient's goals and work toward them in a way that makes them the most comfortable,” Swink said.

The report examined testosterone therapy for this patient population and pointed to its side effects, including the development, or worsening of acne. The treatment for acne follows the same algorithm for acne treatment of patients not taking testosterone therapy for the most part, according to the study.

“A few important differences include the potential need for liver function monitoring in patients being given tetracyclines for acne while taking testosterone therapy due to theoretical risk of hepatotoxicity,” Swink said. “Additionally, antiandrogens, such as spironolactone, that are traditionally used in cisgender females to treat acne cannot be given to transmasculine patients taking testosterone as they directly interfere with the desired masculinization.”

Another potential side effect of testosterone therapy is androgenetic alopecia (AGA).

“Dermatologists can provide numerous treatment options individualized to a patient based on their goals and other treatments they may be undergoing,” Swink said. “These treatments include topical minoxidil and low-level laser light therapy which are FDA approved therapies for AGA, as well as finasteride, platelet rich plasma injections, and oral minoxidil which are not FDA approved for AGA.”

There are many important aspects in the dermatologic care of transgender and gender diverse youth that must be considered, however, one of the greatest tools dermatologists have in their toolkit is education.

“Being able to adequately educate patients and families is so important,” Swink said. “This helps to ensure that patients are receiving high quality care from appropriate professionals and avoiding treatments that may be harmful to their health. Being a supportive provider in the care of transgender and gender diverse youth is critical in helping patients to live their lives as they truly are meant to be, and really has the potential to save a life.”

This article was originally published by sister publication Dermatology Times.

Swink SM, Castelo-Soccio L. Dermatologic considerations for transgender and gender diverse youth. 

 2021;38 Suppl 2:58-64. doi:10.1111/pde.14685

The education on how to treat patients with skin issues in this group needs to be expanded so physicians can better treat their patients. 

A look at the special dermatology needs of transgender and gender diverse youth 

This week, the American Academy of Dermatology (AAD) Association’s annual meeting returned live and in-person in Boston, Massachusetts. In the scientific seminar, “Practical Management of Birthmarks and Hamartomas,” 5 presenters discussed the practical management of a variety of birthmarks and birthmark-like conditions including hemangiomas, vascular stains, melanocytic nevi, nevus sebaceous, and hamartomas using a check-list approach.

The experts discussed diseases and clinical presentations posing questions such as “What should I be thinking about?” “Are any tests needed?” “Is there a window of opportunity to intervene?” “Do I (or others) need to see patients back in a follow-up appointments and if so what should we be looking for?” and “How do I counsel parents about what to expect over time?”

Renee M. Howard, MD, FAAD, pediatric dermatologist at University of California, San Francisco (UCSF) Benioff Children’s Hospital Oakland, California, presented a section entitled, “Developmental Hotspots: When to Worry, What to Do,” offering a checklist approach for diagnosis and treatment and outlining differential diagnosis of developmental anomalies with skin findings at 4 hotspots—the nose, brow, scalp, and lower back.

“My talk [covers] how to evaluate skin lesions that result from developmental mistakes that present in the skin, though some of them have deep connections. So you can’t approach them only as a dermatologist,” Howard says. “You have to think about what to image, where to refer, and specifically, I’m focusing on anomalies in high-risk locations.”

When Howard was a resident in dermatology in the early 1990s, she saw a patient with a midline cervical cleft, a rare anomaly on the anterior neck, and she couldn’t find much information about the condition. That led her to becoming interested in developmental birthmarks.

“It turns out that not just the front of the neck, but the front of the ear, nose, scalp, back, and also around the umbilicus are really high-risk areas for these skin findings,” Howard said.

She said it’s important to educate dermatologists about developmental birthmarks because they need to be managed differently and sometimes present later in adulthood.

“If you see a skin lesion that has a deep extension because it has a developmental origin—for example a cyst that’s on the middle of the lower back—that deep component can extend into the spinal cord,” Howard said. “As a dermatologist, you shouldn’t be managing that the way you would a regular cyst in the skin. It’s important to be knowledgeable about what lesions are high-risk.”

During her 25-minute presentation, Howard showed photos of patients that she and her colleagues have seen in their practices, with pits, nodular growths, and aplasia cutis congenita—the absence of skin in a focal area).

“I…talk about how to manage lesions in each of the 4 locations—nose, brow, scalp, and lower back—what causes them, how you image them, and when you need to refer,” she said.

For example, high risk and cutaneous signs for lumbosacral (LS) spine include midline mass, atypical dimple, thick tuft of terminal hair, membranous aplasia cutis congenita (ACC), tail or tag, and segmental hemangioma.

Other important signs to be discussed include a ring of dark hair, midline defects, and other high-risk features to look out for.

“I hope people will feel confident that they can recognize any skin lesions that may be overlying a developmental anomaly with deep extension and feel confident on how to manage that,” Howard said.

During another part of the program, pediatric dermatologist Ilona J. Frieden, MD, FAAD, director of UCSF Birthmarks & Vascular Anomalies Center, discusses the features of vascular birthmarks, particularly those which may have extra-cutaneous associations.

She discussed nevus simplex complex, and possible associations; what are considered regular port-wine stains, with potential concerns and approach; 3 types of special vascular stains; and other conditions in the differential diagnosis.

While previously, all vascular stains were lumped under port-wine stain or capillary malformation, further delineation can help guide management, she explained

Concerning nevus simplex, Frieden pointed out that it is the most common vascular birthmark present at birth, between 20% to 60%. While it occurs in all races and skin types, it may be easier to appreciate in those with lighter skin tones.

“Most cases are not associated with extra-cutaneous dz [disease],” she says. “However, there are someextracutaneous associations including megancephaly-capillary malformation (M-Cap), beckwith weidemann syndrome, a nevus-simplex like stain overlying developmental anomaly (eg, a lump, lipoma, hypertrichosis, aplasia cutis); Roberts SC [pseudo-thalidomide syndrome], and nova syndrome.”

Highlighing what are considered regular port-wine stains, Frieden explained how they virtually always present at birth and discussed those that may have added risks. For example, the risk of Sturge-Weber syndrome should be considered if the port-wine stains involved the forehead and size is an additional determinant or risk. In a recent study of 96 infants with forehead port-wine stains, Sturge-Weber syndrome was present in 22%, nearly all in those with larger port-wine stains.

The presentation also included exam essentials for major differential diagnoses, widespread blotchy stains with overgrowth and worrisome and not-so worrisome features, and a trio of special vascular stains—Cutis marmorata telangiectatica congenita (CMTC), geographic stains, and fast flow stains.

Frieden provided a checklist as to what to look out for, the care involved, and associated risks. She concluded her talk by discussing the differential diagnosis of vascular stains including infantile hemangioma precursors and tufted angiomas.

Howard noted in her session that if a case has a vascular stain that also has another skin finding in a high-risk area, such as extra hair on the scalp, it is high risk for having a deep extension.

Frieden, IJ, Howard RM, Hawryluk EB, Oza VS, Shah SD. Practical management of birthmarks and hamartomas. Presented at: American Academy Dermatology Association 2022 Annual Meeting; March 25-29, 2022; Boston, MA.

In a presentation at the 2022 American Academy of Dermatology annual meeting, experts discussed differential diagnoses and clinical presentations in birthmarks and hamartomas.