Addressing Benzodiazepines in Primary Care (Threlfall, 2/28/2024)

A recording of this presentation is available HERE.

***

Many thanks to local psychiatrist, Dr. Alex Threlfall for an excellent Grand Rounds presentation this week: Addressing Benzodiazepines in Primary Care. As we all know, benzodiazepines (heretofore BZD) play a major role in the national opioid epidemic, and despite lack of organized attention to the issue, addressing concomitant BZD use and misuse is an important public health and safety issue for all our patients.

My summary:

BZD were historically the number one prescribed medication in the world (in 1960s and 1970s). They are frequently involved in opiate overdose deaths. The combination is very dangerous. After opioids, BZD are the most commonly caused agent involved in intentional and unintentional overdoses. 

Let's start with six areas of risk (i.e. patients in which you should NOT start BZD).

History of:

• Mental health conditions associated with trauma
• Substance use disorder (SUD)
• Elderly (>65)
• Chronic pulmonary disease (e.g. COPD)
• Women of child-bearing age
• Chronic pain (with or without opiate use)

Trauma

Trauma is rampant in our patient population, upwards of 20-50% of the general US population report a history of childhood physical or sexual abuse. It's even higher -- 70%-- in populations with depression/anxiety, SUD, chronic pain and functional pain disorders like IBS. Sexual abuse directly influences development of SUD.

**THERE IS NO EVIDENCE SUPPORTING THE USE OF BZD in TRAUMA or PTSD (either acute trauma or long term treatment). In fact, there are  studies that suggest that adding benzos at the time of a traumatic event can increase PTSD, can lead to addiction, and that benzos can even reduce the efficacy of psychotherapy by blunting therapeutic effect.

Benzodiazepines are highly addictive. 

VA data from 2016 shows that 58-100% of patients prescribed BZD will become physically dependent, 50% of patients with a pre-existing SUD will develop a BZD use disorder, and 5-10% of patients newly on BZD will develop SUD. 

Benzodiazepines are particularly risky for older adults.

We have not paid attention to our patients as they have aged. People prescribed a BZD were often not offered a safer alternative. In a study of geriatric patients on BZD, <1% had been referred for psychotherapy, 10% were co-prescribed an opioid. The most common indication for BZD in these patients were insomnia and anxiety. Despite this evidence of harm, population studies show increasing rates of BZD rx in elderly patients and failure to discontinue, particularly in people >80 years old and Trend women>> men.

All adults on new or continuous BZD rx

The older you are, the more likely you are to be on a BZD. This is a problem. White people have "better access" to BZD than their non-white cohorts and higher rates of BZD misuse. 

Risks of BZD are real.

Falls, hip fractures, sedation, cognitive impairment, motor vehicle accidents. While an observational study from ~10 years ago found that being on BZD, people were more likely to develop dementia. It is not true that BZD causes dementia, but it does cause cognitive impairment. All BZD are on the Beer's list of medications not safe for elderly patients. Number needed to harm is 2: for every person you successfully treat with BZD, you will harm two. 

As in everything, prevention in the best strategy. In other words, avoid new starts of BZD.

Okay, so when are BZD actually indicated as first-line therapy?

• Acute crisis setting (e.g. patient floridly manic, psychotic, agitated patient => 2mg lorazepam)
• Bipolar mania
• Severe panic
• Alcohol withdrawal (though we are moving away from BZD use)
• Seizure disorders
• Procedures & planned events (e.g. for patients with intellectual disability who need sedation)
• Phobias (e.g. flying, but beware)

If prescribing BZD for these conditions, you should use the lowest effective dose, avoid alprazolam (aka xanax) whenever possible (super short, very fast acting, too much reinforcement) and restrict prescription for 2 weeks or less.

Of note, BZD are THIRD line treatment for anxiety

First line is pharmacotherapy -- SSRI, SNRI-- and/or evidence based psychotherapy (CBT, mindfulness). 

• SSRIs/SNRIs are all effective for anxiety disorders. Dr. Threlfall's favorites are escitalopram and sertraline. Start super low (2.5 or 5mg escitalopram=> target 5-20mg 25 mg sertraline, target 100-200mg). People with anxiety will often respond sooner than with depression. If they tolerate, will respond within 1-2 weeks. 
• Part of efficacy is you! You need to reassure patients, check in (1 week to be sure started/tolerating), then see again in 2-4 weeks. You hold them psychically through the process
• Buspirone can be effective either standing or PRN. Literature doesn't do it justice. Titrate 10-30mg TID. Watch for serotonin syndrome. 
• uses for Vets for prn to cross the GG bridge, take prn
• It's not sedating, but still helps with anxiety.

Second line: gabapentin>>pregabalin>>propranolol>>clonidine

                    amitriptyline*>>nortriptyline

                    hydroxyzine*>> diphenhydramine

• clonidine for pts with extreme anxiety 0.1mg TID can be pretty effective (BP and adherence and rebound BP issues). Can use 0.1mg patch, can co-treat hypertension
• *DON'T use amitriptyline in EVER elderly b/c of anti-cholinergic effects
• *DON'T use hydroxyzine and diphenhydramine in older adults >65 (people with cognitive impairment). Effects wear off pretty quickly, get tolerant quickly

Another note, BZD is NOT FIRST LINE TX for INSOMNIA (per all professional societies)

First line

All recommend CBTi as first-line treatment for insomnia

VA has a free app, free to download, easy to use, is effective: "CBTi coach"

Free app from the VA

Second line options

• Melatonin: 1-2mg max (long acting)
• Prazosin (if nightmares/trauma or waking startled): 1mg to start, up by 1mg every 4 days, as much as tolerated, stop at 3mg to see again. VA says 12-18 is safe dose (Threlfall maxes out at 8mg). Side effects: orthostasis, sedation, congestion
• Trazodone, low dose, start at 25mg, rarely go about 150mg. Above 200mg getting into anti-depressant range. Can get serotonin syndrome if on SSRI. People don't like hangover effect
• Mirtazapine: more sedating at lower dose 7.5-15mg, above that lose the sedation effect (but keep increased appetite). People when starting mirtazapine can experience heavy sedation during the day for 3-5 days. Try it when that sedation is not going to be too bothersome. Restless legs
• Doxepin: recent study 3-6mg safe and effective in older adults. On Beer's list, anti-cholintergic but at low dose, better safety profile
• Amitriptyline/nortriptyline 

Third line

• Hydroxyzine, diphenhydramine
• Ambien (Zolpidem) or Temazepam if you really need to, particularly if concomitant bipolar disorder

How to initiate BZD:

1. Rare if you ever do

2. Short symptomatic relief 1-2 weeks

3. Get psychiatry consult

4. Make sure you have the conversation with the patient, "This is going to be short term"

5. Be very clear about the risks

6. Discuss exit strategies (taper, switch)

7. Be the only prescriber

8. Document failure of other trials of meds

9. CURES, urine drug screen

10. BZD treatment agreement if going more than 2 weeks

Use these only Total Daily Dose (TDD): Lorazepam 2mg TDD, Clonazepam 1.5 TDD, Diazepam (he doesn't like, people seek out the effect, 15mg TDD), Temazpeam 30mg TDD

There is evidence that if patients are informed of the risks of BZD, they want off them. In one study in which pharmacy sent patients taking BZD information on the risks of BZD: 62% self-started BZD taper, 21% were completely off at 6 month follow-up. Give people the opportunity to get off with education, encouragement and support. 

One final note, getting people off BZD is not not without risk. In fact, there is new evidence that there is real risk in discontinuing BZD. When people are taken off BZD, they die more, fall more, go to the hospital more, have more suicide attempts and non-fatal overdoses. We have to pay attention to the effects of long-term BZD on their neurophysiology. When people are really attached, get them on the lowest dose possible. And don't use them every day -- intermittent use is MUCH safer than chronic use. 

Tapering is a team-based approach. Use your nurse, your MA, a colleague, behavioral health. Don't start right away. Establish relationship. You ARE the medicine in the room. Establish that before making any changes. It goes a long way. 

Live long and Prosper: Longevity and Blue Zones (Perez, 2/21/2024)

 A recording of this presentation can be found HERE.

***

Many thanks to Dr. Jesse Perez for an excellent talk on longevity and "the blue zones". A recording is available above.

My notes:

Dan Buettner, a National Geographic explorer and journalist, coined a term called "the blue zones" in 2004  (and later wrote a book about them with the same name) after an exploratory visit to Okinawa to investigate longevity.

Buettner called out these places

• Sardinia, Italy
• Nicoya, Costa Rica
• Ikaria, Greece
• Okinawa, Japan
• Loma Linda, CA USA

These are all geographic centers where, not only do people live longer, but they also have a good quality of life as they age. They all contain a disproportionate number of centenerians (people over 100 years old), who have been able to delay chronic disease by decades and who have also been able to stay physically active and mentally sharp. 

In terms of centenarians (per 100K population), Japan boasts 73/100K, the US 24/100K, Canada 33.5/100K, and China 4/100K.

Sardinia, Italy, a Mediterranean island, was one of the first blue zones to be "discovered". They eat a mostly plant-based diet (only 5% of the diet is meat/fish/poultry). They have steep hills that inhabitants must traverse -- going up and down many times per day -- and they have cultural norms that promote stress management. 

Loma Linda, CA is a Seventh Day Adventist community which deeply respects the sabbath as a day of rest, is grounded in gratitude and fellowship with community, follows a mostly plant-based diet with the biggest  meal earlier in the day. People there partake in regular exercise, and limited alcohol, tobacco and caffeine. 

What do these places have in common?

 Customs and norms that seem to be replicated in different places and promote longevity include:

• natural daily movement
• purpose (reason to get out of bed)
• stress management 
• 80% rule of Okinawa, in which people are taught to eat until they are "80% full"
• plant-based diets
• moderate alcohol use (except Loma Linda, which has none)
• community and religiosity
• keeping family close
• positive influences

There is much debate over the nature vs. nurture in life expectancy. Twin studies show that 50% is likely environmental and 20-30% is genetics. Interestingly people who live over 90 years old have an even stronger genetic influence.

A case study of a town of 9K people in 2009, in which some of the principles of the "blue zones" were implemented town-wide -- found that when you increase people's access to plant based food options, provide healthier options, have children provide no-candy based fundraisers, offer fruit as the default (instead of fries) and improve walking paths -- in just one year, life expectancy was extended by 3.2 years AND health care costs were decreased by 40%. 

What are the leading causes of death in the US?

Lifespan vs. Health span

There is also a concept in longevity medicine of considering Health span vs. Lifespan, that is what is the quality of your life not just the longevity of life. Health span takes into account cognitive health, physical health and emotional/mental health. 

We know that several lifestyle factors influence cognitive decline: exercise, moderate alcohol use (1/day), sleep, mental stimulation, and social connection.

UCSF has an E Prognosis calculator, which estimates your 6 month mortality based on a number of factors. Evidence shows that patients DO want to understand how much time they have left. Consider trying it out with some of your elders.

Final recs from Dr. Perez to live a long and health life:

• plant based diet
• movement
• stress management
• community
• have a purpose

Colorectal Cancer Screening (Toub, 2/14/2024)

 A recording of this presentation is available HERE

***

Many thanks to Dr. Danny Toub, who gave an excellent Grand Rounds presentation this week titled Colorectal Cancer Screening: What PCPs need to Know. A recording of the presentation is available above. 

My notes:

Colorectal cancer (CRC) is the #2 cause of US cancer deaths overall in men and women, the #1 cause of cancer death in men under 50. In 2024, we are predicted to have more CRC deaths than COVID deaths. In theory, CRC is an ideal disease to screen for because the slow development from polyp to cancer -- on average, 10 years -- means that early detection can actually lead to lives saved; if caught early, death can be prevented. 

Through many efforts, we have increased our CRC screening over the last two decades, and in doing so, we have  decreased CRC mortality. Unfortunately, it's not by a lot: a 2023 study published in JAMA, found that CRC screening only extends a person's life by an average of 110 days (see forest plot below). 

Despite what seems like a perfect set-up for screening success (a slow growing cancer with multiple tools for early detection), the reality is that CRC screening doesn't have great evidence for all-cause mortality benefit either. Sigh. Plus, the number needed to screen (NNS) to prevent one cancer death is not small -- 450 with q5 year flex sig and 900 with annual FOBT. Double sigh. 

It is important to note, however, that current quality measures do incentivize us to screen for CRC-- there are payments attached to our doing so, through both state and federal programs. So, to be clear, we screen to 1) decrease CRC mortality and 2) because our systems are set up to do so. 

How do we screen? 

Many of us are well aware that there are several CRC screening modalities, which are essentially considered equivalent in terms of CRC detection rates:

1. q10 year colonoscopy (most invasive, most cumbersome, but benefit of being able to remove polyps during the procedure)
2. Annual FIT test (has replaced the older FOBT test)
3. stool DNA tests (frequency variable)
4. q5 year flexible sigmoidoscopy (screens less of the colon but does not require anesthesia and can be done by primary care clinicians, if trained)
5. Emerging serum tests (not yet FDA approved)

Current USPSTF Guidelines (2021) recommend shared decision-making to decide which modality to use; there are a number of studies showing many different screening tools decrease CRC mortality (with colonoscopy slightly better than the other modalities in detecting cases and averting death). More invasive screening tools have more serious potential harms, but all positive screening tests lead to colonoscopy, as the gold standard for diagnosis of CRC. Of note, there is a lack of screening colonoscopy access in our county, and most of the community health centers (and even Kaiser) default to non-invasive screening via FIT testing. As an aside, SRCH is moving to a pilot program screening with a more expensive DNA FIT testing q3 year (rather than q year) starting next month. 

Who to screen?

Current USPSTF Guidelines (2021) make a Grade A recommendation for all adults 50-75, with a Grade B recommendation for adults 45-49. They make a Grade C recommendation for people 76-85 with shared decision-making based on 10 year life expectancy (i.e. healthy 76+ year adults should  be selectively screened).

Whereas there is a statistical benefit to screening people 45-49, the AAFP actually disagrees with the USPSTF and recommends  screening starting age 50 with "insufficient evidence (I)" to recommend screening before then. The 2023 ACP guidelines are slightly less in terms of screening frequency, recommending a q2 year FIT test (vs 1 year per USPSTF).

What works?

Dr. Toub spent some time talking about how we get patients to get screened -- possible interventions include one on one conversations, client reminders, group education, provider prompts, navigation, EHR enhancements, but in the end, Dr. Toub advocates for all kinds of ways:

• give nudges
• use decision aids
• give options (not too many)
• tell stories
• make personal recommendations
• send serial text messages
  

There are lots of different ways to remove friction to opt-out defaults. This includes not requiring physician visits to be screened, giving people deadlines, and helping people to structure their choices. This also includes having PCP mention the importance during the visit -- once again, what we say matters.

For more ideas, see some of the images below. Also note that The Community Guide , supported by the same folks who sit on the USPSTF and gives evidence-based recommendations for what works to improve population health, recommends making sure that materials in given in the correct language, that transportation assistance be made available, and that dedicated patient navigation helps in low-resourced settings-- increaseing rates of colonoscopy and FIT by 13 and 12 percentage points respectively.

Dr. Toub ended his information-packed presentation with the suggestion that maybe we are thinking about CRC screening all wrong. In some well-resourced countries with national health plans, CRC screening is approached differently: rather than screening EVERYONE over a certain age, this BMJ guideline, published in 2019, suggests using a risk-based calculator to determine who should be screened. This suggests NOT screening anyone with <3% 15-year risk (based on this RISK CALCULATOR), which takes into account age, smoking and alcohol use, BMI, cancer hx among other things) to calculate that risk. IT also uses decision aids to help in determining screening decisons (see below):

What is the future of CRC screening?
It's AI, of course! Watch the last few minutes of Dr. Toub's talk for breaking news on AI in CRC screening. I think I'll leave it out of my summary today, as it is all still just starting and not really moved into standard practice.

For now,  remember these take home points: 1) CRC screening decreases deaths from CRC but does NOT improve all-cause mortality, 2) multiple screening modalities at multiple different intervals are considered equivalent, so use shared decision-making with your patients to meet their needs, and 3) use lots of different nudges to get your patients to follow-up with screening, but don't forget to mention the importance of CRC screening yourself!

Until next week. . .

Kink and BDSM for the Medical Provider (Grimley 2/7/2024)

A recording of this presentation can be found HERE.

***

Many thanks to Dr. Grimley, who gave their senior Grand Rounds presentation on a topic that most of us would be nervous to touch with a ten-foot pole -- alternative sexual lifestyles -- also known as "kink" and "BDSM." Dr. Grimley shared the prevalence and range of alternative sexual practices in our country and showed data about how people's sexual behavior influence health, illness, and experiences of the healthcare system (no surprise). Many healthcare providers don't know enough about alternative sexual practices to even begin a conversation, much less to be able to provide comprehensive care. Ultimately, they encouraged us to not shy away from learning about all the kinds of sex our patients are having so that we can better serve them. 

First, let's define "kink" --the practice of unconventional or unusual sexual preferences or behaviors, fantasy or desires. These are behaviors that are influenced by political leanings, cultural upbringings, and religious beliefs and vary widely among individuals. What about "BDSM"? The BDSM Triskelion, created in the 1990s defines the three pillars of BDSM: Bondage and Discipline, Domination and Submission, Sadism and Masochism. But there are many other "kinks" outside these tenets. And not all of them are sex-related

Dr. Grimley started by laying out the dominant "sex hierarchy" -- reading a passage from a 1984 piece by Gayle Rubin's "Thinking Sex: Notes for Radical Theory of the Politics of Sexuality.

"Good" sex according to this hierarchy is heteronormative, monogamous, home-based sex. "Bad" sex is trans-sex, fetish-sex, public sex. If we, as healthcare providers,  are acculturated to accept these norms, then we tend to disregard a decent subset of the population that engages in alternative behaviors, and in so doing, miss opportunity to improve people's health. From this perspective, there may be an uninformed assumption that kink is happening without consent and is potentially harmful to participants. These are biases we carry. 

In fact, Dr. Grimley said, every interaction in the kink community must be grounded affirmative consent (i.e. "opt in"-- we talked about what was going to happen before it happened and I agreed that I want to participate), rather than the more traditional, heteronormative "opt out" (i.e. person pushes forward with sexual advances without aforementioned conversation, participant is supposed to say "no" if they  don't want to mid-act).

Core principles of kink:

• Consent: mutual, iterative, can be revoked at any time, enthusiastic yes!
• Pre-negotiation: talk about everything
• Community: strong cultural values (see above), historic roots, and social networks that guard against violence/abuse
• Knowledge
• Producing good experiences

Here are some important terms to learn in the kink community:

• safe word: a word agreed on before the sexual act beings that, when spoken, stops any sexual act/scene immediately
• scene: a kinky encounter or experience, often planned in advance, that can last for minutes, hours or days
• aftercare: time and attention given to partners after a scene is complete
• drop: a feeling of mental or physical exhaustion after a scene
• hard limits: what someone will absolutely not do, does not want to do
• soft limits: a behavior or action someone is hesitant to do but may try
• munch: a public meet up of people in the kink community
• roles: top/dominant/dom/domme/sadist + bottom/submissive/sub/masochist
• dungeon: a room or venue specifically for BDSM activities
• play: kinky activities or interactions
• toys: implements or tools
• edge play: greater risk, higher intensity, or considered more transgressive than common play

For providers who may be concerned about how to distinguish BDSM (which may involved marks on skin, restraining partners, etc.) from abuse/intimate partner violence, Dr. Grimley encouraged us to very simply "ask the patient". Any sexual act or harm done without consent is abuse.

After giving us a education on the vocabulary and core principles of kink, Dr. Grimely went on to share with us some of the statistics showing kinksters as an unrecognized sexual minority. But activities associated with kinking are perhaps less rare than you might suppose: 21% of participants in the 2015 Sexual Exploration in America Study had been "tied up", 15% had participated in "playful whipping", 31.9% reported "spanking" during sex, and 3.4% reported having gone to a BDSM party or dungeon. In another study, sponsored by Durex condoms, 10% of participants reported SM and 36% bondage in their sexual acts.

Kinks were surveyed in the 2016 Kink Health Survey, revealing some startling health disparities: 10x national average of HIV , 5x the national average (24%) had attempted suicide. Interestingly enough, 85% of kinky people said that their involvement in kink had But many people are not "out" with their healthcare providers -- there is a lot of anticipated stigma, only 38% were out as kinky, and in another study 58% had not disclosed to their provider (even lower for BIPOC patients). Perhaps unsurprisingly, many report previous disappointing experiences with the system.

How should providers talk to patients about kink? Invite your kink patients to "come out". First, know the language. Second, do not be afraid to ask the question: "Is there anything else you want me to know about your sexuality?

Also, ask about mark on people's bodies. "Where these marks consensual?" and "I noticed bruises on your buttocks. Can you tell me how you got them?" are two simple ways to enter into that conversation.

Dr. Grimley ended their presentation by running through some specific unintentional injuries that can happen in kink and BDSM play, and some suggestions on how to help mitigate risks. These include but are not limited to:

• Risks in bondage: rope constriction, strangulation and circulatory issues. Risk mitigation includes bondage release methods, soft rope, breaks to allow for circulation (after 20-30 minutes), never leaving someone unattended when bound, stopping immediately in the case of loss of sensation, and even using a squeaky toy to stop/drop as a non-verbal communication
• Risks in impact play of unintentional injuries. Risk mitigation includes hitting fleshy and fatty areas (buttocks, thighs, calves); avoiding and watching out for "wrapping", and cleaning and dressing any broken skin
• Risks of wax play: skin issues, burns, and unintentional fire. Risk mitigation includes moisturizing skin and/or shaving hair prior to wax play; not wearing flammable perfumes or colognes, avoiding skin that is already problematic (eczema, psoriasis), choosing the correct materials (bees wax burns the skin, shea butter or soy is safer and lower heat)
• Risks of electricity play: not suitable for those with heart disease or electronic device or implant, not suitable with water/fluids, avoid mucous membranes, care with metal piercings
• Risks of breath play: very high risk and potentially deadly. Risk mitigation: avoid using anything other than a hand, never put pressure on the windpipe, never do breath play alone.

Resources:

• Fetlife: https://fetlife.com/

• National Coalition for Sexual Freedom’s “Kink and Polyamory Aware Professionals (KAP) Directory” http://www.ncsfreedom.org/key-programs/kink-aware-professionals-59776

• The Network / La Red - http://tnlr.org/en/

• National Coalition for Sexual Freedom (NCSF) http://www.ncsfreedom.org

• The Alternative Sexualities Health Research Alliance (TASHRA) https://www.tashra.org

• Community-Academic Consortium for Research on Alternative Sexualities (CARAS) https://www.carasresearch.org

• Kink Knowledgeable http://training.kinkknowledgeable.com

• Diverse Sexualities Research and Education Institute https://dsrei.org

• Multiplicity of the Erotic Conference (MOTE) https://www.mote-con.org

Neonatal and Infant Eruptions (Sugarman, 1/31/2024)

 A recording of this presentation can be found HERE

***

Many thanks to Dr. Jeff Sugarman, local dermatologist, who gave an excellent Grand Rounds presentation this week on Neonatal and Infant Eruptions: when to worry and when to reassure. See above for the link to watch it.

My notes this week come in the form of a dermatologic photo quiz for your testing pleasure. Answers are below the final photo and question.

1. What is this neonatal rash categorized by fragile pustules that erode very quickly, leaving behind brown collarettes with post-inflammatory hyperpigmentation. It can be present at birth and last days to weeks. More common in neonates with more pigment. Benign.

Source: DermNetNZ.org

2. What is this benign newborn rash often caused by over-bundling newborns? Erythematous papules and pustules often occurring on covered portions of the skin. Totally benign.

Source: DermNetNZ.org

3. What is this benign rash of the neonatal period, often during the first 1-3 weeks of life, acneiform, often occurring on the face and upper chest and scalp. It has NO comedones and is the result of an inflammatory reaction. Treatment is either topical antifungal or  topical 1% hydrocortisone, though no treatment is also acceptable. Often mistaken for neonatal acne, which presents later AND always has comedones.

Source: DermNetNZ.org

4. What is this benign self-resolving rash that is notable for pustules on feet and hands that can last for months, come and go in crops, and look a lot like scabies (but when scraped show no mites). This will resolve on its own but can take months to do so?

Source: DermNetNZ.org

5. What is this papule on the scalp, often solitary with a unique yellow color? The papule is self-resolving but can last years (5-10) before it disappears. Can sometimes occur in multiples and have extra-cutaneous involvement, with particular issues in the eye. In rare cases, multiple of these are associated with leukemia. 

Source: DermNetNZ.org

6) What is this condition characterized by peau d'orange (texture). Often missed by primary care clinicians. Usually solitary but can be present in sheets. Featured by pockets of histamine -- Darier's sign when stimulated (scratched or rubbed), can be accentuated. Can last for years. Activated by cold/hot/rubbing. 

Source: DermNetNZ.org

7) There are four variants of #6: 1) solitary (most common) 2) urticaria pigmentosa with peak onset first year of life 3) diffuse cutaneous mastocytosis and 4) most rarely mast cell leukemia. This is one of those variants, which features flushing, hives, pruritis, blisters and sometimes diarrhea (due to mast cells in the GI system). Treatment involves: antihistamines, oral cromolyn for GI symptoms, consider epi pen. If a high burden of disease, consider monitoring serial serum triptase.

Source: DermNetNZ.org

Of note, activators of mast cells include: alcohol, aspirin, narcotics (including codeine), some contrast agents, and hot/cold/sunlight. Consider limiting these triggers in patients with mastocytosis.

8) What is this eyebrow bump, often seen on the lateral brow, caused by a sequestration of ectodermal tissue during embryogenesis? It is usually present at birth but not noticed. It is rubbery,  not compressible, not tender. It is also not a true cyst. 

Source: DermNetNZ.org

Of note, you need to worry when the location suggests a CNS connection, which is always in the midline (25% of midline dermoids have a CNS connection). If a dermoid cyst is leaking clear fluid, this is likely spinal fluid and is BAD. It is good to remember that the neural tube closes in a series of discontinuous zippers, and there is no CNS connection in any other place than the "hot spots" where the zippers close. 

9) Do you have to worried about a CNS connection in this infant with a dermoid cyst?

Source: https://webeye.ophth.uiowa.edu/eyeforum/cases/115-dermoid-cyst.htm

Of note, dermoid cysts on the lateral brow are generally removed after a child's second birthday. They should be referred to pediatric plastics for the excision for best cosmetic effect. 

10) What vitamin deficiency -- often due to inadequate intake and appearing around the time a baby is weaning -- leads to  this psoriaform rash (well demarcated) on the face and in the diaper area? Hint: the name of the rash is acrodermatitis enteropathica.

Source: MDEdge

11) What is this uniquely pediatric form of this autoimmune disorder, often characterized by raccoon eyes (periorbital accentuation of erythema) and diagnosed by maternal serologies? Hint: photosensitive, 50% of cases have congenital heart block. Rare (10%) have hepatobiliary disease and even rarer (1%) thrombocytopenia.

Source: DermNetNZ

Source: DermNetNZ

12) Cool pearl!! Alternate treatment for umbilical granuloma. Aside from silver nitrate, a study showed that a single application of THIS common household item, covered with bandage tape can resolve this problem. What is the common household item?

13) What is this scary looking fixed erythematous rash (lasting 24+ hours) that often appears on the face, arms and legs. Child tends to be non-toxic appearing, and the trunk is spared. It resolves on its own. It is in the HSP spectrum, but has no cutaneous findings.

Source: DermNetNZ

14) Last but not least, what is this rash in a 6 week old baby that did not respond to topical steroid, topical anti-fungal, systemic antihistamines, or antibiotics?

And the bonus final: how do you treat it? Treatment of infantile scabies, even in children under 2 months and <15kg is the same as for children >2 months and >15kg (though the drug labeling does not promote this). Per Dr. Sugarman, you can confidently treat with either oral ivermectin OR topical permethrin safely and effectively in neonates.

********************
No cheating!!! Don't look down here until you have really thought about each of the photo questions above!!

Answers:

1) Transient Neonatal Pustular Melanosis

2) Miliaria Rubra

3) Neonatal cephalic pustulosis

4) Acropustulosis of infancy

5) Juvenile Xanthogranuloma

6) Mastocytosis

7) Urticaria pigmentosa

8) Dermoid Cyst

9) YES! This baby needs imaging (CT or MRI) to rule out encephalocele or mucocele prior to excision).

10) Zinc Deficiency: Treatment is rx zinc 3mg/kg/day

11) Neonatal Lupus Erythematosus

12) Table Salt!! A study of 17 infants with umbilical hernia found 100% resolution  (17/17) with a single application of table salt, occluded with medical bandage x 24 hours.

13) Acute hemorrhagic edema of infancy

14) Infant scabies, characterized by vesicles and pustules on the palms and the soles